Professore Ordinario di Cardiologia –Dipartimento di ... · le Linee Guida della Società Europea...

Professoressa Silvia G. PRIORI Curriculum Vitae

Professoressa Silvia G. Priori Professore Ordinario di Cardiologia –Dipartimento di Medicina Molecolare- Università degli Studi di Pavia - Italia Direttore Scientifico Centrale e Direttore delle UO di Cardiologia Riabilitativa e Molecolare - IRCCS Istituti Clinici Scientifici Maugeri Spa e SB - Pavia - Italia Direttore del Programma di Genetica Cardiovscolare - Centro National de Investigaciones Cardiovasculares Carlos III (CNIC) Madrid - Spagna

Dipartimento di Medicina Molecolare UNiversità degli Studi di Pavia-ITALIA

IRCCS Istituti Clinici Scientifici Maugeri SpA-SB Pavia - ITALY

Centro National de Investigaciones Cardiovasculares Carlos III (CNIC) Madrid - SPAIN


“Noi pensavamo che il nostro destino fosse nelle stelle. Ma ora sappiamo che in larga misura il nostro destino è nei nostri geni" - James Watson


CURRICULUM VITAE

FOTO E BREVE BIOGRAFIA

La Prof.ssa Silvia G. Priori è nata a Torino il 17 Ottobre 1960. Si è Laureata in Medicina e Chirurgia nel

1986 presso l’Università degli Studi di Milano “cum Laude” e si è Specializzata in Cardiologia, nella stessa

Università, con il massimo dei voti e la lode, nel 1990. La Prof.ssa Priori ha poi conseguito il Dottorato in

Fisiopatologia Cardiaca nel 1995. Al momento, la Prof.ssa è Professore Ordinario di Cardiologia presso

l’Università degli Studi di Pavia, Direttore Scientifico Centrale degli Istituti Clinici Scientifici Maugeri

Spa & SB - IRCCS, Direttore del Reparto di Cardiologia Riabilitaiva e del Servizio di Cardiologia

Molecolare presso gli stessi ICS Maugeri. Attualmente la Professoressa Priori è Direttore del Programma

di Genetica Cardiovascolare Genetica Cardiovscolare presso il Centro National de Investigaciones

Cardiovasculares Carlos III (CNIC) Madrid – Spagna.

La Prof.ssa Priori è stata Professor of Medicine e Direttore del Programma di Genetica presso la Leon H.

Charney Division of Cardiology of the New York University, New York, USA dal 2008 a Gennaio 2016.

L’Unità di Cardiologia Molecolare degli ICS Maugeri, diretta dalla Prof.ssa Priori, si occupa delle cure

dei pazienti affetti da patologie aritmogene ereditarie ed è il centro di riferimento nazionale ed

internazionale per i pazienti affetti da Sindrome del QT Lungo, del QT corto, Sindrome di Brugada,

Tachicardia Ventricolare Polimorfa Catecolaminergica, Cardiomiopatia Dilatativa, Cardiomiopatia

Ipertrofica e Cardiomiopatia del Ventricolo Destro. Inoltre fanno riferimento alla Cardiologia Molecolare

anche Atleti e giovani pazienti affetti da tachicardia ventricolari idiopatica.

Il personale dei Laboratori Diagnostici della Cardiologia Molecolare, accreditati dal Sistema sanitaria

Nazionale (SSN), lavora in stretta collaborazione con lo staff clinico, eseguendo lo scerrening genetico

necessario per scoprire le mutazioni correlate alle patologie aritmogene eraditarie.

Nell’ultimo ventennio la Prof.ssa Priori si è occupata degli studi dei meccanismi legati alle aritmie

cardiache su base genetica, contribuendo all’analisi della relazione tra il sistema nervoso autonomo e la

triggered activity cardiaca. La Prof.ssa ha partecipato attivamente alla costituzione ed implementazione del

Registro “Unexplained Cardiac Arrest Registry of Europe (UCARE)” che ha raccolto la più ampia casistica

di pazienti a cuore sano, sopravvissuti ad un episodio di fibrillazione ventricolare. Dal 1995 la Prof.ssa

Priori si è dedicata allo studio dei meccanismi genetici della sindromi aritmogene ereditarie. Quest’attività

l’ha portata ad ottenere grandi risultati nella evidenziare l’esistenza della correlazione genotipo-fenotipo


sia negli studi “in vivo” che “in vitro” ed alla definizione delle conseguenze elettrofisiologiche delle

mutazioni presenti nei canali ionici.

Tra i maggiori risultati conseguiti dalla Prof.ssa Priori nel corso dei sui studi delle basi genetiche delle

aritmie cardiache ci sono: l’identificazione del primo gene (RyR2) la cui alterazione è causa di una forma

grave di aritmia cardiaca maligna che provoca morte improvvisa, chiamata Tachicardia Ventricolare

Polimorfa Catecolaminergica, la scoperta delle mutazioni che inducono la perdita di funzione del gene

KCNJ2, che causa la Sindrome del QT corto di tipo 3 e le pubblicazioni sul New England Journal of

Medicine e sul JAMA del primo algoritmo di stratificazione del rischio per i pazienti affetti da Sindrome

del QT lungo. Nel 2012 inoltre la Prof.ssa Priori ha pubblicato i risultati dello studio PRELUDE, uno studio

multicentrico, che ha contribuito a definire l’algoritmo di stratificazione del rischio per la Sindrome di

Brugada.

I Laboratori di Ricerca di Base diretti dalla Prof.ssa Priori presso la Fondazione Salvatore Maugeri si sono

dedicati negli anni alla creazione di modelli murini transgenici, che hanno contribuito alla caratterizzazione

degli aspetti fondamentali della fisiopatologia della CPVT (2005-2011).

Recentemente è stata attivata una nuova Unità di Terapia Genica presso i Laboratori di Cardiologia

Molecolare e nel 2012 uno dei modelli murini della CPVT è stato usato per sviluppare la prima strategia

di trasferimento genico mediato da vettori virali, che si è dimostrata in grado di eliminare tutte le

manifestazioni cliniche della malattia.

La Prof.ssa Priori ha attivamente collaborato con le più importanti riviste scientifiche cardiologiche e di

elettrofisiologia, assumendo ruoli di Leadership ed ha ricevuto moltissimi riconoscimenti scientifici ed

onorificenze dalle più importanti società scientifiche del settore.

Dal 2008 al Gennaio 2016 la Prof.ssa Priori dirige il Programma di Ricerca sulle aritmie cardiache

ereditarie presso la New York University ed è Membro del Consiglio e Chairman della Commissione per

le Linee Guida della Società Europea di Cardiologia (ESC).

La Prof.ssa Priori è ed è stata Membro del Consiglio Direttivo di molte Società Scientifiche tra la quali la

Heart Rhythm Società Statunitense, la Società Europea di Cardiologia, la Società Italiana di Cardiologia

e nel 2015 è diventata Membro dello Scientific Panel for Health della Comunità Europea, organismo di

consultazione per l’identificazione delle aree di ricerca e di applicazione delle scoperte scientifiche nella

pratica clinica.

La Prof.ssa Priori è stata Presidentessa e Past- President della European Heart Rhythm Association,

l’Associazione Aritmologica Europea, afferente alla Società Europea di Cardiologia.

La Prof.ssa Priori è inoltre Associate Editor di Circulation Research e Membro del Board di numerose

riviste scientifiche quali: Circulation, Circulation Research, European Heart Journal, Journal of

Cardiovascular Electrophysiology, Pacing and Clinical Electrophysiology, Journal of Cardiovascular

Pharmacology and Therapeutics.


La Prof.ssa Priori ha pubblicato oltre 425 articoli nelle riviste internazionali più importanti del settore e ad

oggi ha un H-Index of 123, che ha contribuito nel 2014 ad insignirla del certificato di ''Highly Cited

Researchers'', della ISI Thomson-Reuters (cf. http://highlycited.com) (confermato anche per il 2015, 2016

e per quest’anno 2017 (Certificate “Highly Cited Researcher in the Field of Clinical Medicine” of the

Clarivate Analytics, Boston, USA) e proprio quest’anno ha raggiunto un Total Citation Index di 109.056.

Nel corso degli ultimi 14 anni di carriera gli studi della Prof.ssa Priori sono stati costantemente finanziati

con i fondi di ricerca dei maggiori Enti Pubblici e Privati, Nazionali ed Internazionali, quali ad esempio: il

Ministero Italiano della Ricerca e quello della Salute, i Fondi della Comunità Europea (Progetti EU: FP6,

FP7, ERC), l’NIH, le Fondazioni Telethon Italia, Leducq e Cariplo per un totale di più di 8.600.000 €.

La Prof.ssa Priori ha inoltre al suo attivo 3 Brevetti: US 7.741.529B1 (22th June, 2010) "Transgenic

animal model for catecholaminergic polymorphic ventricular tachicardia (CPVT) and use thereof"; EU

06763551.6 (28th May, 2010) "Mutations associated with the long QT syndrome and diagnostic use

thereof”; US 8.859.517 B2 (14th Oct, 2014) “A method of gene transfer for treatment of recessive

catecholaminergic polymorphic ventricular tachycardia (CPVT)”


Silvia G. Priori, M.D., Ph.D DATI PERSONALI Cittadinanza Italiana

Data di Nascita 17 Ottobre 1960

Luogo di Nascita Torino, Italia

Stato di Famiglia Separata

2 figli

Indirizzo Ufficio Direttore Scientifico Centrale

IRCCS – Istituti Clinici scientifici Maugeri Spa SB

Via Salvatore Maugeri n.4,

27100 Pavia – ITALIA

Recapiti Telefonici +39 0382 592774 (Diretto)

+39 0382 592040 (Executive Assistant)

+39 0382 592649 (Segreteria di Direzione Scientifica Centrale)

Cellulare Aziendale +39 333 8166845 (ITALIA)

Fax +39 0382 592059

Email [email protected];

Email secretaries [email protected]; [email protected]

Languages Italiano, Inglese e Francese

Researcher unique identifier(s): Research ID: A-6116-2014

CARRIERA SCOLASTICA 1974-1979 Liceo Scientifico “L. Cairoli”- Vigevano, Italy

1979-1985 Corso di Laurea in Medicina e Chirurgia, Università degli Studi di Milano, Italia

1985-1989 Specializzazione in Cardiologia, Università degli Studi di Milano, Italia

1993-1995 Dottorato in Fisiopatologia Cardiovascolare, Università degli Studi di Milano, Italia

TRAINING Internati

1980-1982 Istituto di Anatomia Umana, Università degli Studi di Milano, Italia

1983-1985 Istituto Clinica Medica IV, Università degli Studi di Milano, Italia

1985-1988 Specializzando in Cardiologia, presso l’Istituto di Clinica Medica Generale e Terapia

del Policlinico di Milano - Università degli Studi di Milano, Italia

1986 Borsista di Ricerca in elettrofisiologia sperimentale presso la Cardiovascular

Division Washington University St Louis, Missouri, USA

1988-1990 Fellow dell’American Heart Association, Washington University St Louis, Missouri,

USA

1992-1994 Tutor di Medicina Interna, Università degli Studi di Milano, Italia

1990-1995 Borsista dell’Istituto di Clinica Medica Generale e Terapia Medica, Università degli

Studi di Milano, Italia

1996-1997 Stage di ricerca in Biologia Molecolare presso l’Istituto TIGEM-Telethon Institute

for Genetic and Medicine- di Milano, Italia


DIPLOMI 1985

1989

1995

Laurea in Medicina e Chirurgia - Università degli Studi di Milano, Italia

Diploma di Specilizzazione in Cardiologia - Università degli Studi di Milano,

Italia

Dottorato in Fisiopatologia Cardiovascolare - Università degli Studi di Milano,

Italia

RUOLI OSPEDALIERI 1997-2000 Assistente Cardiologo, Fondazione Salvatore Maugeri Clinica del Lavoro e della

Ribilitazione IRCCS, Pavia, Italia

Dal 2000 Direttore del Servizio di Cardiologia Molecolare, degli Istituti Clinici Scientifici Maugeri

SpA SB- IRCCS già Fondazione Salvatore Maugeri Clinica del Lavoro e della Ribilitazione

IRCCS, Pavia, Italy

Dal 2008 Direttore Scientifico dell’Istituto Scientifico di Pavia, degli Istituti Clinici Scientifici

Maugeri SpA SB- IRCCS già Fondazione Salvatore Maugeri Clinica del Lavoro e della

Ribilitazione IRCCS, Pavia, Italy

Dal 2010 Direttore della UO di Cardiologia Riabilitativa, degli Istituti Clinici Scientifici Maugeri SpA

SB- IRCCS già Fondazione Salvatore Maugeri Clinica del Lavoro e della Ribilitazione

IRCCS, Pavia, Italy

Dal 2013 Direttore Scientifico Centrale degli Istituti Clinici Scientifici Maugeri SpA SB- IRCCS già

Fondazione Salvatore Maugeri Clinica del Lavoro e della Ribilitazione IRCCS, Pavia, Italy

PRINCIPALI FUNZIONI OSPEDALIERE

Cardiologia Riabilitativa Direttore delle attività cliniche e coordinatore delle attività terapeutiche Molecular Cardiology Unit Direttore delle attività di diagnostica molecolare, Direttore delle attività di Ricerca per le

patologie aritmiche su base genetica e per le cardiomiopatie, preparazione e scrittura di Grant di Ricerca e di articoli scientifici

Direzione Scientifica Coordinamento delle attività di Ricerca Scientifica della Rete degli Istituti Clinici Scientifici Maugeri SpA SB- IRCCS già Fondazione Salvatore Maugeri Clinica del Lavoro e della Ribilitazione IRCCS, Pavia, Italy

CARRIERA ACCADEMICA 1985 Laurea in Medicina e Chirurgia - Università degli Studi di Milano, Italia

1989 Specilizzazione in Cardiologia - Università degli Studi di Milano, Italia

1995 Dottorato in Fisiopatologia Cardiovascolare - Università degli Studi di Milano, Italia

1997-2000 Professore a Contratto in Cardiologia, Università degli Studi di Pavia, Italia

2000-2014 Professore Associato di Cardiologia presso il Dipartimento di Scienze Ematologiche,

Pneumologice Cardiovascolari Mediche e Chirurgiche; e da Novembre 2011 del

Departimento di Medicina Moleculare.

2004-2006 Adjunct Professor di Farmacologia alla SUNY Upstate Medical University Syracuse NY,

USA

2008-2016 Professorore di Medicina e Directtore del “Cardiovascular Genetics Program”, presso la

Leon H. Charney Divisione di Cardiologia della New York University, New York, USA

Since 2014 Professor Ordinario di Cardiologia press oil Departimento di Medicina Molecolare

dell’Università degli Studi di Pavia, Pavia, Italia

Since 2016 Direttore del Programma di Genetica Cardiovascolare presso il Centro National de

Investigaciones Cardiovasculares Carlos III (CNIC), Madrid, Spagna


PRINCIPALI RUOLI ACCADEMICI

Managment Membro della Giunta del Dipartimento di Medicina Molecolare dell’Università degli Studi

di Pavia, Pavia, Italia

Membro della Commissione Strategica del Dipartimento di Medicina Molecolare


Insegnamento Corso di Laurea in Medicina in Lingua Italiana "Golgi" dell’Università degli Studi di Pavia,

Pavia, Italia

Corso di Laurea in Medicina in Lingua Inglese "Harvey " dell’Università degli Studi di

Pavia, Pavia, Italia

Membro del Collegio dei Docenti del Corso di Dottorato in BIOINGEGNERIA E

BIOINFORMATICA (ciclo XXXII) dell’Università degli Studi di Pavia, Pavia, Italia

Membro del Collegio Docenti del Corso di Dottorato in Scienze Biomediche dell’Università

degli Studi di Pavia, nel Ruolo di Coordinatore dell’Area Medica

ALTRE ESPERIENZE PROFESSIONALI

ITALIA

2001-2005 Comitato di Amministrazione del Centro Interdisciplinare di Biologia e

Medicina dello Sport dell’Università degli Studi di Pavia, Pavia, Italia

2001-2005 Membro del Consiglio del Centro per le malattie cardiovascolari professionali,


2013-2014 Membro del Consiglio Direttivo del Dipartimento di Medicina Molecolare dell’Università

degli Studi di Pavia

2013-2017 Membro del Board del Dottorato di Bioingegneria dell’Unievrsità degli Studi di Pavia

2015-2016 Membro del Consiglio di Amministrazione dell’Ospedale Terranova- Bracciolini - San

Giovanni in Valdarno, Italia

Dal 2014 Membro della Commissione Strategica del Dipartimento di Medicina Molecolare

dell’Università degli Studi di Pavia

Dal 2017 Membro del Corpo Docente del Dottorato in Sceinze Biomediche dell’Università degli Studi

di Pavia Ruolo: Coordinatore Area Medica

Dal 2017 Membro dell’Accademia Olimpica di Vicenza (Italia) con il ruolo di Accademico Olimpico

Corrispondente – Classe Scienza e Tecnica

Dal 2017 Membro del Comitato Scientifico della Fondazione Regionale per la Ricerca Biomedica -

Milano

ESTERO

2002-2006 Chairman della Commissione for Practice Guidelines della European Society of Cardiology

2005-2007 Presidente della European Heart Rhythm Society (EHRA) della European Society of

Cardiology (ESC)

2007-2009 Past-President e membro del Direttivo della European Heart Rhythm Society (EHRA) della

European Society of Cardiology (ESC)

2010-2013 Membro del Board of Trustees della Heart Rhythm Society, USA

Dal 2014 Membro del Consiglio Direttivo di Cardiogen Sciences, Inc


2015-2017 Membro della Sottocommissione Awards per la Heart Rhythm Society, USA

Dal 2015 Membro dello Scientific Panel for Health dell’Unione Europea

http://ec.europa.eu/research/health/pdf/mandate-of-the-sph-18062014_en.pdf

COMMITTEE SERVICE

1997 Coordinatore del Corso ESC “Ventricular Tachyarrhythmias in the young: from molecular

biology to the clinic”, Sophia Antipolis (France), 20 CME.

1997 Membro della Task Force della Società Europea di Cardiologia (ESC) sulla Prevenzione

delle malattie cardiovascolari

1999-2001 Chairperson della Task Force dell’ESC sulla “Morte Cardiaca Improvvisa”.

1999-2003 Membro del Board Scientifico della Società Italiana di Cardiologia

2002 Chairman dell’ESC-ERC Policy Conference per le raccomandazioni dell’uso dei

Defibrillatori Esterni Automatici (AEDs) in Europa

2002-2006 Membro del Board della Società Europea di Cardiologia (ESC)

2002 Membro del Board della Sudden Arrhythmia Death Syndromes (SADS) Foundation.

2003 Membro della ACC/AHA/ESC Commissione per lo sviluppo delle Linee Guida per la

gestione dei pazienti con Aritmie Ventricolari e la prevenzione della Morte Cardiaca

Improvvisa.

2004-2006 Membro della Educational Committee della Heart Rhythm Society (NASPE).

2004-2006 Membro del Board della Società Italiana di Cardiologia e Coordinatore del Gruppo di Studio

sulla Ricerca Scientifica

2004 Coordinatore della Women’s Cardiovascular Health Project della Società Europea di

Cardiologia

2005 Membro della Task Force della Società Europea di Cardiologia per le Linee Guida sulla

Gestione delle Malattie Cardiovascolari nei Diabetici

2005 Membro, per conto della Società Italiana di Cardiologia, della Commissione della

Federazione Italiana per le Linee Guida di Cardiologia

2005 Membro della Commissione per la stesura dei Documenti Clinici e Scientifici della Heart

Rhythm Society (NASPE).

2008 Membro della Nuova Press Committee della Società Europea di Cardiologia (ESC) e

Portavoce della stessa nelle conferenze stampa

Since 2008 Membre del Board Scientifico della Fondazione Centro National de Investigaciones

Cardiovasculares (CNIC), Calos III, Madrid, Spagna

2013-2015 Chairman delle Linee Guida sulle Aritmie Ventricolari della Società Europea di Cardiologia

(ESC)

2014 Membro del Working Group “Cardiovascular Domain Working Group of the Clinical

Genome Resource (ClinGen)” del National Institute of Health (USA)

PREMI/RICONOSCIMENTI

1990 Premio Giovane Ricercatore – Società Italiana di Cardiologia, Roma, Italia

1994 Cardiovascular Research Award Bristol-Myers Squibb, USA

1994 Borsista della European Society of Cardiology, Sophia Antipolis, France

1995 Premio Giovane Ricercatore – Società Italiana di Cardiologia, Roma, Italia


2001 Oustanding Research Award in Paediatric Cardiology della American Heart Association,

USA

2002 Nomination come “Scholar in Cardiology” - Società Italiana di Cardiologia, Roma, Italia

2003 Sterling Visiting Professorship: SUNY University of Syracuse, NY, USA

2003 The Harry & Ellen Steinbaum Visiting Professorship in Cardiac Electrophysiology Cedar

Sinai Hospital and University Los Angeles California USA

2004 “The Michel Mirowski Lectureship”, John Hopkins University, Baltimore, USA.

2005 “Paul Wood Lecture” e Medaglia della British Cardiac Society, Manchester UK

2006 “Thomas W. Smith Memorial Lecture” della American Heart Association, USA

2007 “Douglas Zipes Lectureship Award” - Heart Rhythm Society, Denver, USA

2007 “Henry H Neufeld” Memorial Lecture della Società Israeliana di Cardiologia, Israele

2007 “Douglas and Joan Zipes visiting professorhip” Krannert Research Center, Indiana

University- Indianapolis USA

2007 Honorary Professor of Huazhong University of Science and Technology (HUST) at Wuhan,

China

2009 10^th Paul Zoll Memorial Lecture della Beth Israel Deaconess Medical Center at Boston,

USA

2009 “Frank Wilson” Lecture - University of Michigan, USA

2010 Leonardo Career Award – Società Italiana di Cardiologia, Roma, Italia

2011 “The Michel Mirowski” Award, San Francisco, USA

2011 “Leonard N. Horowitz” Memorial Lecture, Philadelphia, USA

2012 Morgagnana Lecture "Geni Aritmici" e Medaglia, Padova, Italy

2012 The Michael Potter & Family Cardiovascular Genetics Endowed Lectureship, Ottawa,

Canada

2012 Sixth Annual NHLI/RBHT Paul Wood - Cardiovascular Lecture Imperial College, London

2013 ESC Rene Laennec Lecture – The European Society of Cardiology-Amsterdam, The

Netherlands

2013 Premio Internazionale Madre Teresa di Calcutta “Inno alla Vita”, Milan, Italy

2014 Heart Rhythm Society Distinguished Scientist Award, The Heart Rhythm Society, San

Francisco, USA

2014 Certificate “Highly Cited Researcher in the Filed of Clinical Medicine” of the Thomson

Reuters.

2015 RT Hall Lecture, “Inherited arrhythmias: from genes to genetic therapy”, CSANZ 2015,

Melbourne, Australia

2015 2015 “Hans Hect” Lecture - University of Chicago

2016 32nd Annual Esward Massie Lecture, “From drug repositioning to gene therapy: taking

channellopaties beyond beta-blockers”, Washington University, St Louis, (USA).

2016 Top Italian Woman Scientist Award of the ONDA (osservatorio Nazionale sulla Salute della

Donna), Milan (Italy).

2016 Onorificenza del Comune di Pavia – Medaglia San Siro


2017 Commendatore al Merito dell’Onorificienza della Repubblica Italiana – Conferimento da

parte del Presidente della Repubblica Italiana Sergio Mattarella e del Primo Ministro Paolo

Gentiloni

2017 Premio Internazionale “Angiola Gili e Cataldo Agostinelli” 2017-Accademia delle

Scienze di Torino – Torino

GRANTS 1995-2004 National Institute of Health (USA) P50 HL52338-07 SCOR in Sudden Cardiac Death.

1996-2000 CEE -PL 96-0028- From molecular biology to clinical management: a novel approach for

the long QT syndrome

1998-1999 TELETHON #1058- Caratterizzazione Genetica e Funzionale delle mutazioni nella

Sindrome del QT Lungo.

1998-2000 CEE - BMH4-CT98-3872- Unexplained Cardiac Arrest Registry of Europe (UCARE).

1998-2003 National Institute of Health (USA) RO1 HL33843- Long QT syndrome: population, genetic

& cardiac studies

1998-2003 National Institute of Health (USA)- R01 HL51618- Long QT Syndrome: Genetic Aspects

1999-2001 Ministero Italiano della Salute: Prevenzione della Morte Cardiac Improvvisa

2000-2002 Ministero Italiano della Salute: La Sindrome della Morte Cardiaca Improvvisa Infantile

(SIDS): meccanismi e prevenzione

2000-2003 LEDUCQ Foundation: Molecular epidemiology of hereditary arrhythmogenic disorders

2001-2003 Ministero Italiano dell’Università e della Ricerca: COFIN 2001- 2001067817_003

Patogenesi e nuove strategie per la cura della morte cardiac improvvisa e le malattie

cardiovascolari.

2001-2003 Ministero della Salute Italiano: Caratterizzazione Clinica, Patologica e Molecolare della

Morte Giovanile Cardioaca ereditaria

2001-2004 TELETHON GP0227Y01: Catarretizzazione Clinica, Sperimentale e Molecolare della

Tachicardia Ventricolare Polimorfa Catecolaminergica (Catecolaminergic Polymorphic

Ventricular Tachycardia -CPVT).

2002-2004 FONDAZIONE CARIPLO: MOrte Improvvisa: Caratterizzazione clinica, genetica e

fisiopatologica della Tachicardia Ventricolare Polimorfa Catecolaminergica -

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT).

2001-2004 Ministero Italiano dell’Università e della Ricerca: Canalopatie genetiche: identificazione dei

markers e delle loro funzioni FIRB 2001 - RBNE01XMP4_006

2002-2004 Ministero Italiano della Salute: Valutazione funizionale e Molecolare delle disfunzioni

genetiche cardiache.

2003-2005 CV THERAPEUTICS, INC: Effetti della Ranolazina sul gene SCN5A dei canali ionici

mutati: Implicazioni per la Sindrome del QT Lungo di Tipo 3.

2003-2005 Ministero Italiano della Salute: Malattie aritmogene eraditarie e Morte Improvvisa: Nuovi

modelli: dalla epidemiologia alla genomica funzionale

2004-2007 TELETHON GGP04066: Patogenesi Molecoalre della Tachicardia Ventricolare Polimorfa

Catecolaminergica correlata alle mutazioni della calsequestrina 2 (CASQ2).


2005-2010 Ministero dell’Istruzione, dell’Università e della Ricerca: FIRB 2003: RBLA035A4X_002

“Terapie biologiche innovative per l’infarto del miocardio”

2004-2006 Ministero Italiano della Salute: Nuove strategie per la diagnosi e il trattamento delle

Patologie Cardiovascolari ad alto rischio.

2006-2009 European Com. - Sixth Framework programme priority 1- Life Sciences, Genomics and

Biotechnology for health: Control of intracellular calcium and arrhythmias. CONTICA

2006-2009 TELETHON GGP06007: Caratterizzazione di un modello murino know-how con la

mutazione di pazienti affetti da Tachicardia Ventricolare Polimorfa Catecolaminergica

(CPVT)

2009-2013 European Com. Potenziale aritmogeno dei farmaci ARITMO

2008-2013 FONDATION LEDUCQ: Alliance for Calmodulin Kinase II Signaling in Heart Disease

2010-2012 Fondation Veronesi: Cellular Models and experimental therapy for the cardiac defects

related with inherited arrhythmogenic diseases

2011-2013 Ministero Italiano della Salute: Bando Giovane Ricercatore 2009: Mouse and human

induced pluripotent stem cells for myocardial diseases.

2011-2014 TELETHON GGP11141: Mutations of Cardiac Calsequestrin and cardiac arrhythmias:

Novel Insights on pathogenesis and therapy

2011-2015 European Com. Guiding Patients Anytime Everywhere - Project Acronym 2 MobiGuide

Sixth Framework programme Grant agreement for: Collaborative project n. 287811

2012-2015 ATEL LQTS study- NIH Grant n. 1R01HL114944-01A1

2014-2017 Fondazione CARIPLO: Development of a corrective strategy for the treatment of Brugada

Syndrome through normalization of L-Type Calcium Channel

20114-2017 MIUR RF-2011-02348444 Inherited arrhythmias: clinical characterization, genetic

geography and experimental studies in the Calabria Region isolate

2014-2018 Gilead research grant: Long QT Syndrome Type 3 Epidemiological profile, risk

stratification and personalized management

2015-2016 Gilead research grant: Role of Calmodulin Kinase II inhibitors in a Catecholaminergic

Polymorphic ventricular tachycardia in Knock-in animal models.

2014-2017 MIUR - GR-2011-02346878 Blocking reciprocal down-regulation of sphingosine-1-

phosphate receptor -1 and B1-adrenergic receptor as a new therapeutic strategy for

improving post-ischemic left ventricular remodeling and dysfunction

2015-2020 European Research Council Grant “EU-rhythmy” ERC-ADG-2014-ID:669387

2017-2020 Eranet Project – FAT4HEART

2017-2020 FARE – MIUR Italia

Negli ultimi 15 anni I Laboratori diretti dalla Prof.ssa Priori hanno ricevuto finanziamenti costanti dalle maggiori istituzioni pubbliche e private, Nazionali ed Internazionali dedite alla Ricerca quali per esempio il Ministero Italiano della Salute, l’Unione Europea (FP6, FP7, ERC), NIH, la Fondazione Telethon Italiana, la Fondazione Leducq. L’ammontare totale dei maggiori grants finanziati è di € 8.600.000.

ATTIVITA’ EDITORIALI E DI REVISIONE Editore Associato Circulation Research

Deputy Editor Journal of Cardiac Electrophysiology


Consulting Editor Journal of the American College of Cardiology

Consulting Editor Cardiovascular Research

Editore Associato

Internazionale

Journal of Arrhythmia of the Japanese Society of Cardiac Pacing and Electrophysiology

Advisory Board Nature Reviews Cardiology

Editorial Board Member International Journal of Heart Rhythm Circulation

European Heart Journal Heart Rhythm Journal of Cardiovascular Pharmacology and Therapeutics Pacing and Clinical Electrophysiology

American Journal of Cardiovascular Drugs

Revisore per: (peer review journal)

Cardiovascular Research

Circulation

Circulation Research

European Heart Journal

Europace

Heart Rhythm

Human Molecular Genetics

Journal of Cardiovascular Electrophysiology

Journal of Clinical Investigation

Journal of the American College of Cardiology

Journal of the American Medical Association

Pacing and Clinical Electrophysiology

Nature Medicine

The Lancet

Then New England Journal of Medicine

Review Activities (Congressi Scientifici)

American Heart Association

Annual Scientific Sessions Congress

European Society of Cardiology

Annual Scientific ESC Congress

European Heart Rhythm Association EUROPACE Congress

Heart Rhythm

Annual Congress of the Heart Rhythm

BREVETTI

Granted

US 7.741.529B1 (22th June, 2010)

"Transgenic animal model for catecholaminergic polymorphic ventricular tachicardia

(CPVT) and use thereof"

EU 06763551.6 (28th May, 2010)

"Mutations associated with the long QT syndrome and diagnostic use thereof”

US 8.859.517 B2 (14th Oct, 2014)

“A method of gene transfer for treatment of recessive catecholaminergic polymorphic

ventricular tachycardia (CPVT)”


ATTIVITA’ EDUCAZIONALI E DI INSEGNAMENTO 1983-1989 Corso di Lettura ECG per gli Studenti di Medicina, Università degli Studi di Milano, Italia

1985-1997 Letture e Seminari per gli studenti di Medicina, Università degli Studi di Milano, Italia

Dal 1997 Letture e Seminari per gli studenti di Medicina, Università degli Studi di Pavia, Italia

Dal 1985 Presentazioni orali a congress Scientifici Nazionali ed Internazionali

Dal 1997 Letture e Seminari per gli Studenti della Scuola di Specializzazione in Cardiologia

dell’Università degli Studi di Pavia, Italia

Dal 2014 Letture e Seminari per gli Studenti della Scuola di Specializzazione in Medicina del Lavoro

dell’Università degli Studi di Pavia, Italia

Dal 2013 Letture e Seminari per gli Studenti di Bioingegneria, dell’Università degli Studi di Pavia,

Italia

Attività come Mentore

Dal 2000 Tesi di Laurea in Medicina per 1 o 2 Studenti l’anno – Università degli Studi di Pavia, Italia

Dal 2000 Specializzazione in Cardiologia – 1 studente l’anno

Dal 2008 Tutor per il Programma “Exchange Students” della New York University (NYU) 4 studenti

all’anno

Dal 2010 Tesi di Laurea in Biologia e Molecular Biology and Genetics 1 – 2 studenti all’anno

Since 2010 Tutor del Programma “Exchange Brazilian Postdoc” degli specialist in cardiologia (San

Paolo- Brazil) 4 studenti all’anno

La Prof.ssa Priori ospita per un training formative circa 5-6 studenti stranieri all’anno presso il Servizio di Cardiologia

Molecolare della Fondazione Salvatore Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS:

Attività didattica:

La professoressa Silvia G Priori ha sempre nutrito un grande interesse per l’attività didattica ed ha iniziato a

svolgere attività didattica sin dai primi anni della sua carriera.

Dal 2000, anno di nomina come Professore Associato in Cardiologia presso l’Università degli Studi di Pavia, l’attività

didattica della Prof.ssa Priori si è rivolta sia alla formazione degli studenti iscritti al secondo triennio della Facoltà di

Medicina, che alla cura dell’organizzazione della scuola di Specializzazione in Cardiologia dell’Università degli Studi

di Pavia.

Per gli studenti che frequentano il corso di Laurea in Medicina e partecipano alle Lezioni del Corso di Malattie

dell’Apparato Cardiovascolare, la Prof.ssa Priori svolge lezioni teoriche sia di aritmologia clinica che relative alla

descrizione delle basi genetiche e molecolari, che predispongono allo sviluppo di disfunzioni elettrofisiologiche e

strutturali (cardiomiopatie). Durate le lezioni “Teorico-pratiche” gli studenti hanno modo di partecipare all’attività

ambulatoriale ove possono acquisire quelle nozioni di base relative alla registrazione e all’interpretazione

dell’elettrocardiogramma, dei monitoraggi Holter, test da sforzo, ecografia e tilt test.

Agli studenti viene poi offerta la possibilità di svolgere sia l’internato semplice che l’internato di Laurea presso

l’Ambulatorio di Cardiologia Molecolare diretto dalla Prof.ssa Priori ed in stretta collaborazione con lo staff (15 fra

medici e biologi molecolari) degli ambulatori e dei laboratori.

L’obiettivo dell’internato di Laurea è quello di svolgere un percorso formativo clinico e di ricerca che offra ai

tesisti l’acquisizione della capacità di affiancare operativamente i medici nel lavoro clinico quotidiano e che pone


un’enfasi particolare alla acquisizione di fondamentali tecniche di comunicazione medico-paziente. Ogni tesista viene

formato alla ricerca partecipando attivamente a tutte le fasi di un progetto di ricerca, dalla stesura del protocollo, alla

raccolta dei dati, all’analisi statistica ed alla produzione di una comunicazione scientifica. Ogni tesista viene incoraggiato

a completare un piccolo protocollo originale che permetta la presentazione ad un congresso scientifico.

Il successo di questo percorso formativo è dimostrato dai riconoscimenti ricevuti dai tesisti della Prof Priori

(Raffaella Bloise Premio Giovane ricercatore Società Italiana di Cardiologia Pediatrica 2000; Marina Cerrone Premio

del Giovane ricercatore 2000; Massimilano Grillo: Premio Giovane ricercatore Società Italiana di Cardiologia 2001;

Jannì Nastoli Premio per il miglior abstract del Congresso Nazionale AIAC 2001; Giovanna Faggiano Premio “Federico

Marsico” per il giovane ricercatore Società Italiana di Cardiologia Pediatrica 2001- Alessandro Vicentini selezionato

come una delle dieci migliori presentazione scientifiche congresso ANMCO 2002, Chiara Tulipani finalista Premio

“Federico Marsico” per il giovane ricercatore Società Italiana di Cardiologia Pediatrica 2003; Cinzia Moncalvo finalista

Premio “Federico Marsico” per il giovane ricercatore Società Italiana di Cardiologia Pediatrica 2003, Marina Cerrone

borsa di Studio della Società Italiana di Cardiologia per uno stage formativo presso il Dipartimento di Pharmacology

della SUNY University di Syracuse in cui è stato caratterizzato, con modelli di mappaggio, il topo knock-in per difetto

sul gene RyR2 sviluppato dalla Cardiologia Molecolare diretta dalla Prof Priori, Giuseppe Luca Celano Premio

“Federico Marsico” per il giovane ricercatore Società Italiana di Cardiologia Pediatrica 2005; Emilia R. Buono Premio

per il miglior abstract del Congresso Nazionale AIAC 2006.

La formazione per gli Specializzandi in Cardiologia viene svolta mediante lezioni teorico pratiche di aritmologia

e di genetica in cardiologia.

L’attività didattica della Prof.ssa Priori si esplica anche negli impegni relativi alla Scuola di Specializzazione in

Cardiologia: la professoressa vi partecipa nella stesura dei programmi didattici, nella preparazione del materiale

didattico, nell’organizzazione e valutazione degli esami di profitto degli specializzandi, nonché di quelli di Diploma,

oltre all’organizzazione delle Lezioni teoriche e pratiche sui temi di competenza specifica. Agli specializzandi viene

offerta la possibilità di svolgere una parte della formazione presso gli ambulatori di Cardiologia Molecolare e, per chi

abbia sviluppato un interesse specifico, vi è l’opportunità di svolgere la tesi di specializzazione presso la struttura diretta

dalla Prof.ssa Priori.

La Professoressa Priori dal 2004 è Tutor per l’Istituto Universitario di Studi Superiori – Scuola Universitaria

Superiore di Pavia, diretta dal Prof. Franco Rositi; quest’attività la vede coinvolta quotidianamente nel training

formativo degli allievi a lei assegnati, che svolgono attività di ricerca presso i Laboratori di Cardiologia Molecolare.

Nel 2004 inoltre è stato conferito alla Professoressa Priori l’incarico di insegnamento (come docente e

responsabile del Modulo I “Meccanismi delle aritmie – Fondamenti di Elettrofisiologia” e come docente del Modulo 7

“Condizioni particolari- Sincope”) per il MASTER biennale di II livello in “Elettrofisiologia ed Elettrostimolazione”

organizzato dall’Università degli Studi dell’Insubria (Varese), congiuntamente all’Università degli Studi di Brescia,

rivolto a medici in possesso della Laurea/Laurea Specialistica in Medicina e Chirurgia e Diploma di Specializzazione

in Cardiologia.

L’obiettivo dell’istituzione di questo Master in Elettrofisiologia e della Elettrostimolazione Cardiaca prevede non solo

di sviluppare tutti gli aspetti clinici atti alla gestione delle aritmie e di delineare i meccanismi che stanno alla base di tali

affezioni ma anche di fornire significativi dati conoscitivi che consentano agli iscritti di meglio affrontare la


problematica anche sul piano scientifico. La Scuola di Specializzazione in Cardiologia fornisce oggi una preparazione

adeguata in ambito specialistico cardiologico avvicinando sempre di più lo specialista italiano a quello europeo. In

alcuni settori ultraspecialistici quale quello dell’Elettrofisiologia e della Elettrostimolazione Cardiaca, l’evoluzione delle

conoscenze dei meccanismi fisiopatologici e terapeutici oltre al rapido sviluppo tecnologico, rendono assolutamente

necessario prevedere, come già avviene regolarmente negli Stati Uniti d’America da parte del H.R.S. (Heart Rhythm

Society) ex N.A.S.P.E. (North American Society of Pacing and Electrophysiology), una adeguata preparazione post-

specialistica nel settore specifico, che è l’obiettivo del Master di II livello in oggetto.

Dal Novembre del 2008 a Gennaio 2016 è stata nominata Professor of Medicine and Director of Cardiovascular Genetic

Department The Leon H. Charney Division of Cardiology New York University School of Medicine USA ed anche in

questa struttura è incaricata di svolgere attività didattico-educativa con seminari, Grand Round, lezioni magistrali, ecc.

Dal Febbraio 2016 è stata nominata Professor of Medicine and Director of Cardiovascular Genetic Department presso

il Centro National de Investigaciones Cardiovasculares Carlos III (CNIC) Madrid – Spagna ed anche in questa struttura

è incaricata di svolgere attività didattico-educativa con seminari, Grand Round, lezioni magistrali, ecc oltre che attività

di ricerca.

Attività Clinica:

1991 al 1997 la Prof. Priori ha gestito l’attività clinica di diagnosi e cura dei pazienti con aritmie cardiache,

sotto la guida del Professor Peter J. Schwartz. Durante questi anni, immediatamente successivi al conseguimento della

Specializzazione in Cardiologia, la Prof Priori ha anche svolto attività del reparto di degenza presso la Clinica Medica

IV dell’Università di Milano presso il Policlinico di Milano acquisendo esperienza nella gestione del paziente iperteso

(Clinica dell’Ipertensione diretta dal Prof Zanchetti) e dei pazienti con Sindromi Coronariche Acute (UTC diretta dal

Prof Lotto). In particolare la Prof Priori ha acquisito competenze specifiche circa la gestioni di sperimentazioni

farmacologiche (farmaci antiaritmici) in pazienti con aritmie ventricolari su substrato di varia eziologia.

Dal 1997 al 2000 è stata responsabile della clinica sulle malattie ereditarie aritmogene seguendo i pazienti

provenienti da Centri Italiani ed Esteri in tutto il loro iter diagnostico comprendende la valutazione clinica cardiologica

non invasiva. Durante questo periodo la Prof Priori ha acquisito esperienza nella gestione in prima persone e nel

coordinamento delle attività ambulatoriali cardiologiche (ECG, Holter, Ecocardiogramma, Test da Sforzo) presso la

Clinica Medica Generale e Terapia Medica dell’Università di Milano.

Dal 2000 ad oggi è Direttore del Servizio “Laboratori di Cardiologia Molecolare”, comprensivo oltre che dei

laboratori di Biologia Molecolare e di Elettrofisiologia Cellulare, di una Unità Operativa che svolge attività diagnostica

(ECG Holter Ecocardiogramma Test da Sforzo Tilt Test e impianto di Reveal Loop recorder sottocutaneo) a cui

afferiscono pazienti affetti da malattie aritmogene erediatrie quali la Sindrome di Brugada, la Tachicardia Ventricolare

Polimorfa Catecolaminergica, la Sindrome di Timothy, la displasia aritmogena del ventricolo destro, la cardiomiopatia

ipertrofica. Tali pazienti sono riferiti da medici operanti su tutto il territorio nazionale. Presso la Fondazione Maugeri la

Cardiologia Molecolare dispone di letti per la degenza nell’ambito della Divisione di Cardiologia e la gestione di tali

ricoveri viene svolta sotto la diretta gestione dello Staff di Cardiologia Molecolare diretto dalla Prof Priori.

Nell’anno 2004 lo staff clinico del Servizio di Cardiologia Molecolare che afferisce alla Prof Priori è composto da 2

medici specialisti in Cardiologia, 1 medico specialista in Genetica, 4 Specializzandi in Cardiologia.


Attività di Ricerca Scientifica:

La Prof.ssa Priori ha iniziato l’attività di ricerca nel 1983 presso i Laboratori di Aritmologia Sperimentale, diretti

dal Professor Peter J. Schwartz, presso la Clinica Medica IV dell’Università di Milano, diretta dal Prof Zanchetti. In

questo periodo ha partecipato allo svolgimento di ricerche sperimentali su modelli di aritmie in corso di ischemia e

riperfusione e ha investigato il ruolo del sistema nervoso autonomo nella genesi delle aritmie. Queste ricerche si sono

sviluppate con l’ausilio di sperimentazioni esegute su gatti, che hanno permesso alla Prof.sa Priori di iniziare ad

acquisire le corrette tecniche di manipolazione e cura degli animali, nonché di imparare a redigere protocolli

sperimentali di ricerca su animali, nel rispetto delle leggi applicate all’utilizzo di animali in laboratorio. Ha inoltre

partecipato a studi clinici nell’ambito della sperimentazione di nuovi farmaci antiartimici (tocainide, flecainide,

CM7857-Pentisomide).

Nel 1986 ha frequentato per un periodo di training formativo, i Laboratori di Elettrofisiologia Cellulare presso

il Dipartimento di Cardiologia della Washington University, a St. Louis (Missuri), diretto del Professor Peter B. Corr

affiancando il team nel mappaggio intraoperatorio delle tachiaritmie ventricolari e nel mappaggio di aritmie indotte

nel cane, in corso di ischemia.

In questo primo training formativo alla Washington University, a St. Louis, la Prof.sa Priori ha imparato a gestire le

procedure clinico-sperimentali necessarie per operare su cani, acquisendo le tecniche di manipolazione dell’animale,

nonché quelle di medicazione e cura dello stesso, gestendo protocolli di sperimentazione e procedure di stabulazione,

rigorosamente applicate dalla Washington University, compilando la scheda dell’animale, indicando la provenienza,

il tipo di esperimento eseguito (con la redazione di un accurato protocollo di sperimentazione: sostanza testata,

differeniazione del dosaggio, effetto a breve e lungo tempo, analisi dei parametri vitali, etc.).

La Prof.ssa Priori è ritornata presso i Laboratori di Elettrofisiologia Cellulare presso il Dipartimento di

Cardiologia della Washington University, a St. Louis (Missuri), diretto del Professor Peter B. Corr per apprendere le

fondamentali metodiche di elettrofisiologia cellulare, current clamp e voltage clamp.

Dal 1996 al 1997 ha svolto uno stage di ricerca in Biologia Molecolare presso il Telethon Institute for Genetics

and Medicine (TIGEM) a Milano, diretto dal Prof. Andrea Ballabio, approfondendo le tecniche fondamentali di biologia

molecolare, clonaggio e site directed mutagenesi.

Dal 1997 la Professoressa Priori ha iniziato lo sviluppo di una linea di ricerca autonoma (interamente finanziata

da fondi ricevuti tramite grants competitivi) nei laboratori da Lei diretti. Fondendo l’attività clinica sulle malattie

artimogene alle competenze di biologia molecolare ed elettrofisiologia cellulare ha creato il primo centro in Italia,

ed uno dei primi al mondo, di Cardiologia Molecolare. Ha organizzato tre banche dati computerizzate che raccolgono

oggi uno dei più grandi data base al mondo di pazienti affetti da Sindrome del QT lungo, uno dei più grandi data

base al mondo di pazienti affetti da Sindrome di Brugada e il più grande data base al mondo di pazienti affetti

Tachicardie Polimorfe Catecolaminergiche.

L’analisi dei dati derivanti da questi registri ha grandemente contribuito alla definizione dei protocolli per la diagnosi

e la cura dei pazienti affetti da tali malattie.


E’ di rilievo notare che la gran parte dei pazienti arruolati in tali registri è stata seguita clinicamente e/o tipizzata

geneticamente dal gruppo clinico e dal gruppo di diagnostica molecolare operante presso la Cardiologia Molecolare

della Fondazione Maugeri di Pavia.

I risultati fondamentali del gruppo sperimentale diretto dalla professoressa Priori includono la prima registrazione di

depolarizzazioni diastoliche in vivo comprovanti l’esistenza di questo meccanismo aritmogeno in vivo (pubblicata

su Circulation), la scoperta del gene delle Tachicardie Polimorfiche Catecolaminergiche (pubblicata su Circulation)

e la scoperta del gene della Sindrome del QT lungo con sindattilia (pubblicata su Cell).

I risultati più prestigiosi del gruppo clinico diretto dalla professoressa Priori includono: la definizione del primo

algoritmo di stratificazione del rischio gene-specifico nella Sindrome del QT lungo (pubblicata sul New England of

Medicine), la predizione della risposta alla terapia beta bloccante nella sindrome del QT lungo (pubblicata su JAMA),

la identificazione della Sindrome di Brugada come causa di arresto cardiaco in età pediatrica (pubblicata su The

Lancet), la definizione della Storia Naturale e lo sviluppo di un algoritmo di stratificazione del rischio nella Sindrome

di Brugada (due articoli pubblicati su Circulation).

Un’interessante area di lavoro della Prof.ssa Priori è rivestita dalla responsabilità assunta dal 2002 ad operare

come Chairman delle Linee Guida della Società Europea di Cardiologia. In questo ruolo la Prof. Priori ha lavorato

attivamente non soltanto sugli aspetti organizzativi, ma anche e soprattutto è stata coinvolta nella discussione della

metodologia di sviluppo delle linee guida e degli aspetti correlati all’interfaccia fra le linee guide e i criteri per

definire la rimborsabilità delle procedure diagnostiche e dei presidi terapeutici.

Il contributo scientifico e l’esperienza clinica della Prof.ssa Priori sono ampiamente riconosciuti dalla

Comunità Scientifica Internazionale come dimostrato dalle numerose pubblicazioni, dai premi e dagli inviti a

contribuire a libri di grande prestigio in cardiologia ed aritmologia, quali il testo CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE, edito dal Professor D.P. Zipes e dal Professor J. Jalife per

la WB Saunders, che è considerato uno dei più prestigiosi volumi di aritmologia a livello internazionale. La

Professoressa Priori, per la sua competenza, viene invitata dagli Editors, ad ogni nuova edizione, a preparare uno o

più capitoli; per l’ultima edizione del testo ha preparato ben 3 capitoli.

La Professoressa Priori nel 2001 ha inoltre collaborato alla stesura della seconda edizione del libro CARDIAC

ARRHYTHMIA. MECHANISMS, DIAGNOSIS & MANAGEMENT, edito dal Professor PJ Podrid e dal Professor

PR Kowey per Lippincott Williams & Wilkins

Nel 2004 inoltre la Professoressa Priori è stata invitata dal Professor D.P. Zipes a collaborare alla 7° edizione del prestigioso

testo di cardiologia: HEART DISEASE. A TEXTBOOK OF CARDIOVASCULAR MEDICINE, Edito dai Professori D.P.

Zipes, P. Libby, R.O. Bonow, E. Braunwald- Elsevier.

Infine la competenza nel campo della prevenzione della Morte Improvvisa e l’autorevolezza riconosciutale dalla Comunità

Scientifica è documentata dal ruolo svolto nella stesura di Linee Guida: 1) 2001 Chairman del Committee per la Prevenzione

della Morte Improvvisa della Società Europea di Cardiologia; 2) 2003 Chairman per l’aggiornamento delle linee guida la

Prevenzione della Morte Improvvisa della Società Europea di Cardiologia; 3) 2003 Chairman per conto della Società

Europea di Cardiologia dello sviluppo delle raccomandazioni congiunte ESC – European Resucitation Council sull’uso del


defibrillatore Automatico Esterno; 4) 2003 Membro della Task Force congiunta ACC/AHA/ESC per lo sviluppo di linee

guida sulla gestione delle aritmie ventricolari.

ATTIVITA’ SCIENTIFICHE Prinicipali aree di ricerca Cardiologia Clinica

Aritmie Genetiche

Genetics

Gene Therapy

Pubblicazioni Editore di Libri 4

Articles su riviste peer reviewed 425

Capitoli di Libri 71

H-Index - Web of Science 94

H-Index Scholar 132

H-Index Scopus 114

Citazioni 106.997

i10-index 305

ARTICOLI

1 Schwartz PJ, Facchini M, Bonazzi O, Cuspidi C, Priori SG, Varisco T and Zuanetti G: Valutazione

della flecainide nella terapia delle aritmie ventricolari croniche con il carico orale acuto. G It Cardiol

15:273-282; 1985 – Luigi Pozzi, Roma.

2 Zuanetti G, Vanoli E, Zaza A, Priori SG, Stramba-Badiale M and Schwartz PJ: Lack of correlation

between occlusion and reperfusion arrhythmias. Am Heart J 109:932-936; 1985- Mosby, St Louis-

MO- USA

3 Priori SG, Facchini M, Varisco T, Zuanetti G, Songa V, Bonazzi O and Schwartz PJ: Acute oral drug

testing in the evaluation of a new antiarrhythmic agent: Penticainide (CM 7857) New Trends Arrh

1:381-384; 1985. -John Libbey CIC, Rome, Italy

4 Schwartz PJ, Priori SG, Vanoli E, Zaza A and Zuanetti G: Efficacy of diltiazem in two experimental

feline models of sudden cardiac death. J Am Coll Cardio l:661-668; 1986-Elsevier Science Publishing,

New York, NY, USA

5 Zuanetti G, De Ferrari GM, Priori SG and Schwartz PJ: Protective effect of vagal stimulation on

reperfusion arrhythmias in cats. Circ Res 61:429-435; 1987 American Heart Association Scientific

Publishing, Dallas, TX,USA

6 Priori SG, Bonazzi O, Facchini M, Varisco T and Schwartz PJ: Antiarrhythmic efficacy of

Penticainide and comparison with Disopyramide, Flecainide, Propafenone and Mexiletine by acute

oral drug testing. Am J Cardiol 60:1068-1072; 1987 Excerpta Medica, New York, NY, USA


7 Priori SG, Zuanetti G and Schwartz PJ: Ventricular fibrillation induced by the interaction between

acute myocardial ischemia and sympathetic hyperactivity: Effect of Nifedipine. Am Heart J 116:37-

45; 1988 Mosby, St Louis-Mo- USA

8 Priori SG, Mantica M and Schwartz PJ: Delayed afterdepolarizations elicited in vivo by left stellate

ganglion stimulation. Circulation 78:178-185; 1988 American Heart Association Scientific

Publishing, Dallas, TX,USA

9 Schwartz PJ and Priori SG: Progress in the evaluation of a new class I antiarrhythmic agent:

Penticainide. New Trends in Arrhythmias III:181-184; 1988 John Libbey CIC, Rome, Italy

10 Priori SG, Mantica M and Schwartz PJ: Monophasic action potential recording: A technique for the

detection of delayed afterdepolarizations in vivo. New Trends in Arrhythmias III:271-274; 1988 John

Libbey CIC, Rome, Italy.

11 Facchini M, Bonazzi O, Priori SG, Varisco T, Zuanetti G And Schwartz PJ: Multiple comparison of

several antiarrhythmic agents by acute oral drug testing in patients with chronic ventricular

arrhythmias. Eur Heart J 3:462-470; 1988 Academic Press, London United Kingdom

12 Schwartz PJ and Priori SG: Effetti dei calcio antagonisti nella protezione antiaritmica: studi

sperimentali. Cardiologia 33:3-10; 1988 CEPI-Roma

13 Facchini M, Varisco T, Bonazzi O, Priori SG, Songa V and Schwartz PJ: Efficacy e safety of

Flecainide in Low-Risk patients with chronic ventricular arrhythmias. A two-year follow up. Am

Heart J 117:1258-1264; 1989 Mosby, St Louis-Mo- USA.

14 Priori SG, Mantica M, Napolitano C and Schwartz PJ: Early afterdepolarizations induced in vivo by

reperfusion of the ischemic myocardium. A possible mechanism for reperfusion arrhythmias.

Circulation 81:1911-1920;1990-American Heart Association Scientific Publishing, Dallas, TX,USA

15 Priori SG, Corr PB: Mechanisms underlying early and delayed afterdepolarizations induced by

catecholamines. Am J Physiol 258 (Heart and Circ Physiol 27): H1796-H1805; 1990 American

Physiological Society Bethesda, MD, USA.

16 Priori SG, Corr PB: The importance of α-adrenergic stimulation of cardiac tissue and its contribution

to arrhythmogenesis during ischemia. J Cardiovasc Electrophysiol 1: 529-542; 1990 Futura Publishing

Company, Armonk NY, USA

17 Prior i SG, Yamada KA, Corr PB: Influence of hypoxia on adrenergic modulation of triggered activity

in isolated adult canine myocytes. Circulation 83:248-259, 1991 - American Heart Association

Scientific Publishing, Dallas, TX,USA

18 Priori SG, Napolitano C, Schwartz PJ: Electrophysiologic mechanisms involved in the development

of torsade de pointes. Cardiovascular Drugs and Therapy 5:203-212; 1991 - Kluwer Academic,

Boston, MA, USA

19 Priori SG, Corr PB: Variazioni della risposta aritmogena alle catecolamine in corso di ischemia

miocardica acuta. Cardiologia 36(3), 229-235; 1991 - CEPI-Roma

20 Yamada KA, Dobmeyer DJ, Kanter EM, Priori SG, Corr PB: Delineation of the influence of

propionylcarnitine on the accumulation of long-chain acylcarnitines and electrophysiologic


derangements evoked by hypoxia in canine myocardium. Cardiovascular Drugs and Therapy 5:67-76;

1991- Kluwer Academic, Boston, MA, USA

21 Priori SG, Borggrefe M, Camm AJ, Hauer NW, Klein H, Kuck KH, Schwartz PJ, Touboul P, Wellens

HJJ: Unexplained cardiac arrest. The need for a prospective registry. Eur Heart J 13;1445-1446; 1992

Academic Press, London United Kingdom

22 Priori SG, Napolitano C, Schwartz PJ: Cardiac receptors activation and arrhythmogenesis. Eur Heart

J 14 (Suppl. E):20-26;1993 Academic Press, London United Kingdom

23 Priori SG, Napolitano C, Diehl L, Schwartz PJ: Dispersion of repolarization: a marker to identify

high risk patients with long QT syndrome. New Trends in Arrhythmias IX:919-925; 1993 John Libbey

CIC, Rome, Italy

24 Napolitano C, Priori SG, Schwartz PJ: Torsade de pointes. Mechanisms and Management. Drugs

47:51-65; 1994 Adis, Auckland, New Zealand

25 Priori SG, Napolitano C, Diehl L, Schwartz PJ: Dispersion of the QT interval. A marker of therapeutic

efficacy in the idiopathic long QT syndrome. Circulation 89:1681-1689; 1994 American Heart

Association Scientific Publishing, Dallas, TX,USA

26 Priori SG, Paganini V, Boccalatte L, Schwartz PJ: Fibrillazione ventricolare idiopatica: da una

raccolta di casi clinici alla valutazione prospettica. G Ital Cardiol 25:149-158; 1995 Piccin Editore,

Padova

27 The role of the basic arrhythmia research. The continued need for experiments in the intact heart and

organism. Study group on experimental research of the working group on arrhythmias of the european

society of cardiology. European Heart Journal 1995;16:1469-1475. WB Saunders, London, United

Kingdom

28 Vanoli E, Priori SG, Nakagawa H, Hirao K, Napolitano C, Diehl L, Lazzara R, Schwartz PJ:

Sympathetic activation, ventricular repolarization and IKr blockade: Implications for the

antifibrillatory efficacy of K+ channel blocking agents. J Am Coll Cardiol 25:1609-1614; 1995

Elsevier Science Publishing, New York, NY, USA

29 Wang Q, Shen J, Li Z, Timothy K, Vincent GM, Priori SG, Schwartz PJ, Keating MT: Cardiac sodium

channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia. Hum Mol

Genet 4:1603-1607; 1995 Oxford University Press,Oxford, UK

30 Schwartz PJ, Priori SG, Locati EH, Napolitano C, Cantu' F, Jeffrey A, Towbin AJ, Keating MT,

Hammoude H, Brown AM, Chen LK, Colatsky TJ: Long QT syndrome patients with mutations on the

SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in

heart rate. Implications for gene-specific therapy. Circulation 1995; 92:3381-3386 American Heart

Association Scientific Publishing, Dallas, TX,USA

31 De Ferrari GM, Locati EHL, Priori SG, Schwartz PJ: Left Cardiac Sympathetic Denervation in Long

QT syndrome patients: J Interven Cardiol 1995; 8 (Suppl):776-781. Futura Publishing Company Inc.

Armonk, NY, USA


32 Priori SG, Paganini V on behalf of the Steering Committee of UCARE: Diagnosis and management

of patients with Idiopathic Ventricular Fibrillation. New Trends Arr. XI:63-67; 1995 CIC, Rome, Italy

33 Priori SG, Napolitano C, Cantu' F, Brown AM, Schwartz PJ: Differential response to Na+ channel

blockade, ß-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and

HERG defects present in the long QT syndrome. Circulation Research 1996; 78:1009-1015 American

Heart Association Scientific Publishing, Dallas, TX,USA

34 Priori SG, Mortara DW, Diehl L, Napolitano C, Paganini V, Cantù F, Fumagalli M, Schwartz PJ.

Quantification of ventricular repolarization: from dispersion to complexity. New Trends Arr. XI:95-

100; 1996 CIC, Rome, Italy

35 Schwartz PJ, Vanoli E, Priori SG: Aspetti elettrofisiologici della modulazione dei canali del potassio

ATP-dipendenti. Cardiologia 40 (Suppl 1 al n 12):83-86; 1995 CEPI-Roma

36 Priori SG, Cantù F, Schwartz PJ: The long QT syndrome: new diagnostic and therapeutic approach

in the era of molecular biology. Schweizerische Medizinische Wochenschrift 126:1727-1731;1996

Verlag Schwabe &Co, AG, Basel, Switzerland

37 Priori SG, Napolitano C, Schwartz PJ: A molecular basis for the therapy of the long QT syndrome.

Arch Mal Coeur 89:1185-1187;1996 Editions JB Balliere, Paris, France

38 Priori SG: Is Long QT syndrome entering the era of molecular diagnosis? Heart (Editorial) 77:5-

6,1997 BMJ Publishing Group, London, United Kingdom

39 Survivors of out-of-hospital cardiac arrest with apparently normal heart: need for definition and

standardized clinical evaluation. Consensus statement of the Joint Steering Committees of

Unexpleined Cardiac Arrest Registry of Europe and of Idiophatic Ventricular Fibrillation Registry of

the United States. Circulation 1997; 95:265-272 American Heart Association Scientific Publishing,

Dallas, TX,USA

40 Priori SG, Paganini V: Idiopathic Ventricular Fibrillation: Epidemiology, Pathophysiology, Primary

Prevention, Immediate Evaluation and Management, Long-Term Evaluation and Management,

Experimental and Theoretical Developments. Cardiac Electrophysiology Review 1997; 1:244-247

Kluwer Academic, Boston, MA

41 Priori SG, Napolitano C, Paganini V, Cantù F, Schwartz PJ: Molecular Biology of The Long QT

Syndrome: Impact On Management. Pacing and Clinical Electrophysiology 1997; 20/8:2052-2057

Futura Publishing Company, Armonk , NY, USA.

42 Priori SG, Mortara DW, Napolitano C, Paganini V, Diehl L, Cantù F, Cantù G, Schwartz PJ:

Evaluation of spatial aspects of T-wave complexity in the Long QT syndrome. Circulation 1997;

96:3006-3012 American Heart Association Scientific Publishing, Dallas, TX,USA

43 Schwartz PJ, Priori SG: Dal genotipo al fenotipo e dalla biologia molecolare al management clinico.

L’esempio della Sindrome del QT Lungo. Cardiologia 42(Suppl 3):261-268, 1997 CEPI-Roma

44 Schwartz PJ, Priori SG: The long QT syndrome. Annals of Noninvasive Electrocardiology 1998;

3(1): 63-73 Futura Publishing Company- Armonk New York


45 Priori SG, Schwartz PJ, Napolitano C, Bianchi L, Dennis A, De Fusco M, Brown AM, Casari G. A

Recessive Variant of the Romano-Ward Long QT Syndrome. Circulation 1998;97:2420-2425

Williams and Wilkins, Baltimore, MD, USA

46 Priori SG: Identificazione delle mutazioni intrageniche: Cardiologia 1998; 43 (2):1013-1016 CEPI-

Roma

47 Priori SG: L’analisi genetica della ripolarizzazione potrà guidare lo sviluppo della terapia

antiaritmica? Cardiologia 1998;43 (6):589-596 CEPI-Roma

48 Schwartz PJ, Priori SG: A molecular approach to the treatment of cardiac arrhythmias. The last

chance for poetry. Giornale Italiano di Cardiologia 1998;28(1):124-127 Scientific Press, Firenze

49 Priori SG, Napolitano C: Molecular diagnosis of inherited cardiac diseases: Impact on clinical

practice. Giornale Italiano di Cardiologia 1998;28(1):273-278 Scientific Press, Firenze

50 Li H,Chen Q, Moss A, Robinson J, Goytia V,Perry JT,Vincent M, Priori SG, Lehmann MH, Denfield

S, Duff D, Kaine S, Shimizu W, Schwartz PJ, Wang Q, Towbin JA. New mutations in the KVLQT1

potassium channel that cause long QT Syndrome. Circulation 1998;97:1264-1269 Williams and

Wilkins, Baltimore, MD, USA

51 Locati EH, Zareba W, Moss AJ, Schwartz PJ, Vincent GM, Lehmann MH, Towbin JA, Priori SG,

Napolitano C, Robinson JL, Andrews M, Timothy K, Hall WJ : Age and sex-related differences in the

clinical manifestations of congenital long QT syndrome: findings from the International Prospective

LQTS Registry Circulation 1998;97:2237-2244 Williams and Wilkins, Baltimore, MD, USA.

52 Zareba W, Moss AJ, Schwartz PJ, Vincent M, Robinson JL, Priori SG, Benhorin J, Locati H E,

Towbin JA, Keating MT, Lehmann MH, Hall J: Influence of the genotype on the clinical course of

the Long QT Syndrome. N Engl J Med 1998, 339:960-965 Massachusetts Medical Society, Waltham,

MA, USA

53 Priori SG: Exploring the hidden danger of noncardiac drugs: Journal of Cardiovascular

Electrophysiology 1998;9:1-3 (Editorial) Futura Publishing Company, Armonk, New York, USA

54 Priori SG: Gene specific therapy for cardiac disease: the case of long QT Sydnrome Rev Port Cardiol

1998;17:27-38 Sociedade Portuguesa de Cardiologia, Lisboa, Portugal

55 Priori SG, Napolitano C. Impact of molecular biology on diagnosis and management of

arrhythmogenic disordes. Rev Port Cardiol 1998;17:323-327 Sociedade Portuguesa de Cardiologia,

Lisboa, Portugal

56 Prevention of Coronary artery disease in the clinical practice. Recommendations of the Second Joint

Task Force of European and other Societies on Coronary Prevention. Eur Heart J 1998, 19:1434-1503

WB Saunders, London United Kingdom

57 Priori SG, Napolitano C, Schwartz PJ. Low penetrance in the Long QT Syndrome. Clinical Impact.

Circulation 1999;99(4):529-33-Lippincott Williams and Wilkins, Baltimore, MD, USA

58 Priori SG, Barhanin J, Hauer RN, Haverkamp W, Jongsma HJ, Kleber AG, McKenna WJ, Roden

DM, Rudy Y, Schwartz K, Schwartz PJ, Towbin JA, Wilde AM. Genetic and molecular basis of


cardiac arrhythmias: Impact on clinical management. Eur Heart J 1999;20(3):174-95 WB Saunders,

London United Kingdom



cardiac arrhythmias: impact on clinical management. Parts I and II. Circulation 1999;99(4):518-28

Lippincott Williams and Wilkins, Baltimore, MD, USA



cardiac arrhythmias: impact on clinical management. Part III. Circulation 1999;99(5):674-81

Lippincott Williams and Wilkins, Baltimore, MD, USA

61 Chen Q, Zhang D, Gingell RL, Moss AJ, Napolitano C, Priori SG, Schwartz PJ, Kehoe E, Robinson

JL, Schulze-Bahr E, Wang Q, Towbin JA. Homozygous Deletion in KVLQT1 Associated With Jervell

and Lange-Nielsen Syndrome. Circulation 1999; 99(10):1344-1347 Lippincott Williams and Wilkins,

Baltimore, MD, USA

62 Di Paolo M,Crescini S, Buzzi F, Priori S., Napolitano C, Schwartz PJ. Sindrome del QT lungo:

considerazioni da un caso di osservazione medico-legale. Gennaio-Dicembre 1998

63 Bianchi L, Priori SG, Shen Z, Dennis AT, Napolitano C, Ronchetti E, Bryskin R, Schwartz PJ, Brown

MA. Cellular dysfunction of LQT5-mink mutants: abnormalities of Iks, Ikr and trafficking in Long

QT Syndrome. Human Molecular Genetics 1999;8:1499-1507

64 Barbey JT, Anderson M, Ciprandi G, Frew AJ, Morad M, Priori SG, Ongini E, Affrime MB.

Cardiovascular safety of second-generation anthistamines. Am J Rhinol 1999;13:235-243

65 Priori SG, Cerrone M. Ion Channel Diseases and Sudden Cardiac Death. G It Cardiol 1999;29:430-

433

66 Priori SG, Cerrone M. Molecular Bases of Juvenile Sudden Cardiac Death. G It Cardiol 1999;29:49-

52

67 Napolitano C, Bloise R, Ronchetti E, Memmi M, Schwartz PJ, Priori SG. Low penetrance mutations

and compound heterozygosity in LQTS: phenotypic consequences and implications for the clinical

presentation of the disease. G It Cardiol 1999; 29:42-44

68 Moss AJ, Robinson JL, Gessman L, Gillespie R, Zareba W, Schwartz PJ, Vincent GM, Benhorin J,

Heilbron EL, Towbin JA, Priori SG, Napolitano C, Zhang L, Medina A, Andrews ML, Timothy K.

Comparison of clinical and genetic variables of cardiac events associated with luod noise versus

swimming among subjects with the long QT Syndrome. Am J Cardiol 1999; 84:876-879

69 Priori SG, Crotti L. Idiopathic Ventricular Fibrillation. Cardiac Electrophysiology Review 1999;

3:198-201

70 Priori SG, Napolitano C. La Sindrome di Brugada. Aspetti clinici, epidemiologici ed

elettrofisiologici. Cardiologia 1999; 44:225-228.

71 Priori SG, Napolitano C. Diagnostica Molecolare in Cardiologia. Cardiologia 1999; 44 :225-228


72 Moss AJ, Zareba W, Hall J, Schwartz PJ, Crampton RS, Benhorin J, Vincent GM, Locati EH, Priori

SG, Napolitano C, Medina A, Zhang L, Robinson JL, Timothy K, Towbin JA, Andrews ML.

Effectiveness and limitations of beta-blocker therapy in congenital Long QT Syndrome. Circulation

2000;101:616-623

73 Ali RH, Zareba W, Moss AJ, Schwartz PJ, Benhorin J, Vincent GM, Locati EH, Priori SG,

Napolitano C, Towbin JA, Hall JW, Robinson JL, Andrews ML, Zhang L, Timothy K, Medina A.

Clinical and genetic variables associated with acute arousal and non-arousal-related cardiac events

among subjects with the Long QT Syndrome. Am J Cardiol 2000; 85:457-461

74 Priori SG, Napolitano C, Giordano U, Collisani G, Memmi M. Brugada Syndrome and Sudden

Cardiac Death in Children. The Lancet 2000; 355:808-809

75 Napolitano C, Priori SG, Schwartz PJ. Significance of QT Dispersion in the Long QT Syndrome.

Progress in Cardiovascular Diseases 2000; 42:345-350

76 Napolitano C, Priori SG. Genetic and molecular basis of arrhythmias. Journal fur kardiologie 2000;

6:252-254

77 Napolitano C, Schwartz PJ, Brown AM, Ronchetti E, Bianchi L, Pinnavaia A, Acquaro G and Priori

SG. Evidence for a cardiac ion-channel mutation underlying drug-induced QT prolongation and Life-

Threatening Arrhythmias. J Cardiovasc Electrophysiol 2000; 691-696

78 Schwartz PJ, Priori SG, Dumaine R, Napolitano C, Antzelevitch C, Stramba-Badiale M, Richard TA,

Berti MR, Bloise R. A Molecular Link between the Sudden Infant Death Syndrome and the Long-QT

Syndrome. N Engl J Med 2000; 343:262-267

79 Stramba-Badiale M, Priori SG, Napolitano C, Locati EH, Vinolas X, Haverkamp W, Schulze-Bahr

E, Goulene K, Schwartz PJ. Gene-specific differences in the circadian variation of ventricular

repolarization in the long QT syndrome: a key to sudden death during sleep? Ital Heart J 2000; 1:323-

328.

80 Priori SG, Napolitano C, Schwartz PJ, Bloise R, Crotti L, Ronchetti E. The Elusive Link between

LQT3 and Brugada Syndrome: The role of Flecainide challenge. Circulation 2000; 102:945-947

81 Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Brignole M, Giordano U,

Giovannini T, Menozzi C, Bloise R, Crotti L, Terreni L, Schwartz PJ. Clinical and genetic

heterogeneity of the right bundle branch block and ST segment elevation syndrome. A prospective

evaluation of 52 families. Circulation 2000; 102:2509-2515

82 Ficker E, Thomas D, Viswanathan PC, Dennis AT, Priori SG, Napolitano C, Memmi M, Wible BA,

Kaufman ES, Iyengar S, Schwartz PJ, Rudy Y, Brown AM. Novel characteristics of a misprocessed

mutant HERG channel linked to hereditary long QT syndrome. Am J Physiol Heart Circ Physiol 2000;

279:H1748-H1756

83 Sesti F, Abbot GW, Wei J, Murray KT, Saksena S, Schwartz PJ, Priori SG, Roden DM, George AL

Jr, Goldstein SA. A common polymorphism associated with antibiotic-induced cardiac arrhythmia.

Proc Natl Acad Sci 2000; 97:10613-10618


84 Priori SG, Napolitano C. From catheters to vectors: the dawn of molecular electrophysiology. Nature

Medicine 2000; 6:1316-1318

85 Splawski I, Shen J, Timothy KW, Lehmann MH, Priori SG, Robinson JL, Moss AJ, Schwartz PJ,

Towbin JA, Vincent GM, Keating MT. Spectrum of mutations in long QT syndrome genes. KVLQT1,

HERG, SCN5A, KCNE1 and KCNE2. Circulation 2000; 102:1178-1185

86 Wilde AA, Priori SG. Brugada Syndrome and sudden death. Eur Heart J (Letters to Editor) 2000;

21:1483

87 Priori SG. Long QT and Brugada Syndromes: From genetics to clinical management. J Cardiovasc

Electrophysiol 2000; 11:1174-1178

88 Priori SG, Ronchetti E, Memmi M. Gene specific therapy for arrhythmogenic disorders. Ital Heart J

2000; 1:S52-S54

89 Zhang L, Timothy KW, Vincent GM, Lehmann MH, Fox J, Giuli LC, Shen J, Splawski I, Priori SG,

Compton SJ, Yanowitz F, Benhorin J, Moss AJ, Schwartz PJ, Robinson JL, Wang Q, Zareba W,

Keating MT, Towbing JA, Napolitano C, Medina A. Spectrum of ST-T-Wave Patterns and

Repolarization Parameters in Congenital Long-QT Syndrome. ECG Findings Identify Genotypes.

Circulation 2000; 102:2849-2855

90 Bianchi L, Priori SG, Napolitano C, Surewicz KA, Dennis AT, Memmi M, Schwartz PJ, Brown AM.

Mechanisms of I(Ks) suppression in LQT1 mutants. Am J Physiol Heart Circ Physiol. 2000;

279:H3003-H3011

91 Priori SG, Napolitano C, Tiso N, Memmi M, Vignati G, Bloise R,. Sorrentino V, Danieli GA.

Mutations in the cardiac ryanodine receptor gene ( hRyR2) underlite catecholaminergic polymorphic

ventricular tachycardia. Circulation. 2000.2001;103(2):196-200

92 Priori SG, Bloise R. Basi genetiche della morte cardiaca improvvisa. Recenti Progressi in Medicina

2000; 91;643-646.

93 Gasparini M, Mantica M, Galimberti P, Coltorti F, Ceriotti C, Priori SG. Inferior Vena Cava Loop of

the Implantable Cardioverter Defibrillator Endiocardial Lead: a possible solution to the growth

problem in pediatric implantation. PACE 2000; 23:2108-2112

94 Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, Denjoy I, Guicheney P,

Breithardt G, Keating MT, Towbin JA, Beggs AH, Brink P, Wilde AA, Toivonen L, Zareba W,

Robinson JL, Timothy KW, Corfield V, Wattanasirichaigoon D, Corbett C, Haverkamp W, Schulze-

Bahr E, Lehmann MH, Schwartz K, Coumel P, Bloise R. Genotype-Phenotype correlation in the Long

QT Syndrome. Gene-specific triggers for life-threatening arrhythmias. Circulation 2001; 103:89-95

95 Priori SG, Napolitano C, Tiso N, Memmi M, Vignati G, Bloise R, Sorrentino V, Danieli GA.

Mutations in the Cardiac Ryanodine Receptor Gene (hRyR2) Underlie Catecholaminergic

Polymorphic Ventricular Tachycardia. Circulation 2001; 103:196-200.

96 Tanabe Y, Masashi I, Kurita T, Nagaya N., Taguchi A, Suyama K, Aihara N, Kamakura S, Sunagawa

K, Nakamura K, Ohe T, Towbin JA, Priori SG, Shimizu W. Sympathetic Stimulation Produces a


Greater Increase in Both Transmural and Spatial Dispersion of Repolarization in LQT1 than LQT2

Forms of Congenital Long QT Syndrome. J Am Coll Cardiol 2001; 37:911-919

97 Priori SG, Bloise R, Crotti L. The Long QT Syndrome. Europace 2001; 3:16-27

98 Gandini C, Castoldi AF, Candura SM, Locatelli C, Butera R, Priori SG, Manzo L. Carbon Monoxide

Cariotoxicity. Clinical Toxicology 2001; 39:35-44

99 Gandini C, Castoldi AF, Candura SM, Priori SG, Locatelli C, Butera R, Bellet C, Manzo L. Cardiac

Damage in Pediatric Carbon Monoxide Poisoning. Clinical Toxicology 2001; 39:45-51

100 Abriel H, Cabo C, Wehrens XHT, Rivolta I, Motoike HK, Memmi M, Napolitano C, Priori SG, Kass

RS. Novel Arrhythmogenic mechanism revealed by Long-QT Syndrome mutation in the Cardiac NA+

Channel. Circ Res 2001; 88:740-745

101 Cerrone M, Crotti L, Faggiano G, De Michelis V, Napolitano C, Schwartz PJ, Priori SG. Sindrome

del QT lungo e Sindrome di Brugada: due aspetti della stessa malattia? Ital Heart J 2001; 2:253-257

102 Kaufman ES, Priori SG, Napolitano C, Schwartz PJ, Iyengar S, Elston RC, Schnell AH, Gorodeski

EZ, Rammohan G, Bahhur NO, Connuck D, Verrilli L, Rosenbaum DS, Brown AM.

Electrocardiographic prediction of abnormal genotype in congenital long QT syndrome: experience

in 101 related family members. J Cardiovasc Electrophysiol 2001; 12:455-461

103 Spooner PM, Priori SG, Myerburg RJ (Editors). Spotlight on sudden cardiac death. Cardiovasc Res

2001; 50:173-176

104 Priori SG, Napolitano C, Grillo M. Concealed arrhythmogenic syndromes: the hidden substrate of

idiopathic ventricular fibrillation? Cardiovasc Res 2001; 50:218-223

105 Priori SG. Foretelling the future in Brugada Syndrome: do we have the crystal ball? J Cardiovasc

Electrophysiol 2001; 12(9):1008-1009

106 Priori SG, Aliot E, Blomstrom-Lundqvist C, Bossaert L, Breithardt G, Brugada P, Camm AJ, Cappato

R, Cobbe SM, Di Mario C, Maron BJ, McKenna WJ, Pedersen AK, Ravens U, Schwartz PJ, Trusz-

Gluza M, Vardas P, Wellens HJ, Zipes DP. Task Force on Sudden Cardiac Death of the European

Society of Cardiology Eur Heart J. 2001; 22:1374-1450

107 Rivolta I, Abriel H, Tateyama M, Liu H, Memmi M, Vardas P, Napolitano C, Priori SG, Kass RS.

Inherited brugada and LQT-3 syndrome mutations of a single residue of the cardiac sodium channel

confer distinct channel and clinical phenotypes. J Biol Chem 2001; 276:30623-30630

108 Priori SG, Bloise R, Lucca E. Le patologie dei canali ionici cardiaci. Pathologica 2001; 93:451-452

109 Schwartz PJ, Priori SG, Bloise R, Napolitano C, Ronchetti E, Piccinini A, Goj A, Breithardt G,

Schulze-Bahr E, Wedekind H, Nastoli J: Molecular diagnosis a child with sudden infant death

syndrome. Medical and legal implications. Lancet 2001; 358:1342-1343

110 Kimbrough J, Moss AJ, Zareba W, Robinson JL, Hall WJ, Benhorin J, Locati EH, Medina A,

Napolitano C, Priori SG, Schwartz PJ, Timothy K, Towbin JA, Vincent GM, Zhang L. Clinical

Implications for affected parents and siblings of probands with Long QT Syndrome. Circulation 2001;

104:557-562


111 Priori SG. Evidenza che il miocita cardiaco umano rigenera dopo infarto miocardico. Editoriale. Ital

Heart J 2001; 2:1248-1249



Gluza M, Vardas P, Wellens HJ, Zipes DP. Task Force on Sudden Cardiac Death, European Society

of Cardiology. Summary of Recommendations. Europace 2002; 4:3-18

113 Marks AR, Priori SG, Memmi M, Kontula K, Laitinen PJ. Involvement of the Cardiac Ryanodine

Receptor/Calcium release channel in Catecholaminergic Polymorphic Ventricular Tachycardia. J.

Cell. Physiol. 2002; 190:1-6

114 Moss AJ, Zareba W, Kaufman ES, Gartman E, Peterson DR, Benhorin J, Towbin JA, Keating MT,

Priori SG, Schwartz PJ, Vincent GM, Robinson JL, Andrews ML, Feng C, Hall W J, Medina A,

Zhang L, Wang Z. Increased risk of arrhythmic events in Long-QT Syndrome with mutations in the

Pore Region of the human Ether-a-go-go-Related gene potassium channel. Circulation 2002; 105:794-

799. (on line 28 Jan. r7-r12).

115 Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, Bloise R, Giustetto C,

De Nardis R, Grillo M, Ronchetti E, Faggiano G, Nastoli J. Natural History of Brugada Syndrome:

Insights for risk stratification and management. Circulation 2002; 105:1342-1347

116 Auguadro C, Mortara A, Priori SG, Specchia G. GPIIb/IIIa polymorphism in patients with myocardial

infarction. Acta Cardiol 2002;57:32-33.

117 Napolitano C, Priori SG. Genetics of ventricular tachycardia. Curr Opin Cardiol 2002; 17:222-228

118 Priori SG, Napolitano C, Memmi M, Colombi B, Drago F, Gasparini M, De Simone L, Coltorti F,

Bloise R, Keegan R, Cruz Filho FES, Vignati G, Benatar A, Delogu A. Clinical and Molecular

Characterization of Patients with Catecholominergic Polymorphic Ventricular Tachycardia.

Circulation 2002; 106:69-74

119 Shimizu W, Tanabe Y, Aiba T, Inagaki M, Kurita T, Suyama K, Nagaya N, Taguchi A, Aihara N,

Sunagawa K, Nakamura K, Ohe T, Towbin JA, Priori SG, Kamakura S. Differential Effects of β-

Bolckade on dispersion of repolarization in the Absence and presence of Sympathetic stimulation

between the LQT1and LQT2 forms of congenital Long QT Syndrome. J Am Coll Cardiol 2002;

39:1984-1991

120 Noda T, Takaki H, Kurita T, Suyama K, Nagaya N, Taguchi A, Aihara N, Kamakura S, Sunagawa K,

Nakamura K, Ohe T, Horie M, Napolitano C, Towbin JA, Priori SG, Shimizu W. Gene-specific

response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3

forms of congenital long QT syndrome. Eur Heart J 2002; 23:975-983

121 Benhorin J, Moss AJ, Bak M, Zareba W, Kaufman ES, Kerem B, Towbin JA, Priori SG, Kass RS,

Attali B, Brown AM, Ficker E. Variable expression of long QT syndrome among gene carriers from

families with five different HERG mutations. Ann Noninvasive Electrocardiol 2002; 7:40-46.

122 Priori SG, Napolitano C. Genetic defects of cardiac ion channels. The hidden substrate for torsades

de pointes. Cardiovasc Drugs Ther 2002;16:89-92


123 Bloise R, Napolitano C, Priori SG. Romano-Ward and other congenital long QT syndromes.

Cardiovasc Drugs Ther 2002; 16:19-23

124 Rivolta I, Clancy CE, Tateyama M, Liu H, Priori SG, Kass RS. A novel SCN5A mutation associated

with long QT-3: altered inactivation kinetics and channel dysfunction. Physiol Genomics 2002;

10:191-197

125 Gasparini M, Priori SG, Mantica M, Coltorti F, Napolitano C, Galimberti P, Bloise R, Ceriotti C.

Programmed Electrical Stimulation in Brugada Syndrome: How reproducible are the results? J

Cardiovasc Electrophysiol 2002; 13:880-887

126 Wilde AAM, Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, Hauer

R.NW, Kass R.S, Nademanee K, Priori SG, Towbin JA. Proposed Diagnostic Criteria for the Brugada

Syndrome. Eur Heart J 2002; 23:1648-1654

127 Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada R, Brugada P, Corrado D, Hauer RN,

Kass RS, Nademanee K, Priori SG, Towbin JA; Study Group on the Molecular Basis of Arrhythmias

of the European Society of Cardiology. Proposed diagnostic criteria for the Brugada syndrome:

consensus report. Circulation 2002; 106:2514-2519

128 Bosi G, Cappato R, Priori SG, Stramba-Badiale M. Complex electrocardiographic findings in a

neonate with Long QT syndrome. Ital Heart J 2002; 3:605-607.

129 Grillo M, Napolitano C, Bloise R, Priori SG. La sindrome di Brugada: epidemiologia, stratificazione

del rischio e management clinico. Ital Heart J 2002; 3:919-927

130 Priori SG, Grillo M. Genetica ed elettrocardiografia: nuovi dilemmi per il cardiologo. GIAC 2002;

5:122-123

131 Grant AO, Carboni MP, Neplioueva V, Starmer CF, Memmi M, Napolitano C, Priori SG. Long QT,

Brugada syndrome and conduction system disease are linked to a single sodium channel mutation. J.

Clin. Invest. 2002; 110:1201-1209



Gluza M, Vardas P, Wellens HJ, Zipes DP. Linee Guida. Task Force sulla Morte Cardiaca Improvvisa,

Società Europea di Cardiologia. Riassunto delle raccomandazioni. Ital Heart J 2002; 3:1051-1065



Gluza M, Vardas P, Wellens HJ, Zipes DP. Update of the guidelines on Sudden Cardiac Death of the

European Task Force of the ESC. European Heart Journal 2003; 24:13–15

134 Shimizu W, Noda T, Takaki H, Kurita T, Nagaya N, Satomi K, Suyama K, Aihara N, Kamakura S,

Sunagawa K, Echigo S, Nakamura K, Ohe T, Towbin JA, Napolitano C, Priori SG. Epinephrine

unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome. J Am Coll Cardiol

2003; 41:633-642

135 Gasparini M, Priori SG, Mantica M, Napolitano C, Galimberti P, Ceriotti C, Simonini S. Flecainide

test in Brugada Syndrome: a reproducible but risky tool. PACE 2003; 26:338-341


136 Priori SG. The Brugada Syndrome: the path toward complexity. Hellenic J Cardiol 2003; 44:95-101

137 Priori SG, Schwartz PJ, Napolitano C, Bloise R, Grillo M, Vicentini A, Spazzolini C, Ronchetti E,

Cappelletti D, Nastoli J, Bottelli G, Folli R. Risk stratification in the Long-QT Syndrome. N Engl J

Med 2003; 348:1866-1874

138 Mok N-S, Priori SG, Napolitano C, Chan N-Y, Chahine M, Boroudi G. A newly charactarized

SCN5A mutation underlying Brugada Syndrome unmasked by Hyperthermia. J Cardiovascu

Electrophysiol 2003; 14:407-711

139 Napolitano C, Rivolta I, Priori SG. Cardiac Sodium Channel Diseases. Clin Chem Lab Med 2003;

41:439-444

140 Wehrens XH, Lehnart SE, Huang F, Vest JA, Reiken SR, Mohler PJ, Sun J, Guatimosim S, Song LS,

Rosemblit N, D'Armiento JM, Napolitano C, Memmi M, Priori SG, Lederer WJ, Marks AR.

FKBP12.6 deficiency and defective calcium release channel (ryanodine receptor) function linked to

exercise-induced sudden cardiac death. Cell. 2003; 113:829-40

141 Davidson C, Priori SG. Preventing sudden death: the role of the internist. Eur J Intern Med. 2003;

14:75-76

142 Guidelines Committee. “2003 European Society of Hypertension-European Society of Cardiology

guidelines for the management of arterial hypertension”. J Hypertens. 2003; 21:1011-1053

143 Zareba W, Moss AJ, Locati EH, Lehmann MH, Peterson DR, Hall WJ, Schwartz PJ, Vincent GM,

Priori SG, Benhorin J, Towbin JA, Robinson JL, Andrews ML, Napolitano C, Timothy K, Zhang L,

Medina A; International Long QT Syndrome Registry. Modulating effects of age and gender on the

clinical course of long QT syndrome by genotype. J Am Coll Cardiol. 2003; 42:103-109

144 Bloise R, Moncalvo C, Napolitano C, Priori SG. La tachicardia ventricolare polimorfa

catecolaminergica. GIAC 2003; 6:80-85

145 Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada P, Brugada R, Towbin JA, Corrado D,

Hauer RN, Kass RS, Nademanee K, Priori SG. The Brugada numbers. Circulation 2003;

Correspondence: 1

146 Priori SG, Bloise R, Memmi M, Colombi B, Napolitano C, Coltorti F, Gasparini M, Keegan R, Cruz

Filho FES, Drago F, Vignati G, Benatar A, De Simone L, DeLogu A. RYR2 and CASQ2 Mutations

in Patients suffering from Catecholaminergic Polymorphic Ventricular Tachycardia. Circulation 2003;

107(3):e29.Correspondence: 1

147 Schwartz PJ, Priori SG, Napolitano C. How Really Rare Are Rare Diseases? J Cardiovasc

Electrophysiol. 2003; 14:1120-1121

148 Naghavi M, Libby P, Falk E, Casscells SW, Litovsky S, Rumberger J, Badimon JJ, Stefanadis C,

Moreno P, Pasterkamp G, Fayad Z, Stone PH, Waxman S, Raggi P, Madjid M, Zarrabi A, Burke A,

Yuan C, Fitzgerald PJ, Siscovick DS, de Korte CL, Aikawa M, Airaksinen KE, Assmann G, Becker

CR, Chesebro JH, Farb A, Galis ZS, Jackson C, Jang IK, Koenig W, Lodder RA, March K, Demirovic

J, Navab M, Priori SG, Rekhter MD, Bahr R, Grundy SM, Mehran R, Colombo A, Boerwinkle E,

Ballantyne C, Insull W Jr, Schwartz RS, Vogel R, Serruys PW, Hansson GK, Faxon DP, Kaul S,


Drexler H, Greenland P, Muller JE, Virmani R, Ridker PM, Zipes DP, Shah PK, Willerson JT. From

Vulnerable Plaque to Vulnerable Patient: A call for new definitions and risk assessment strategies”.

“Current Perspective” Part I, Circulation 2003; 108:1664-1672

149 Naghavi M, Libby P, Falk E, Casscells SW, Litovsky S, Rumberger J, Badimon JJ, Stefanadis C,

Moreno P, Pasterkamp G, Fayad Z, Stone PH, Waxman S, Raggi P, Madjid M, Zarrabi A, Burke A,

Yuan C, Fitzgerald PJ, Siscovick DS, de Korte CL, Aikawa M, Airaksinen KE, Assmann G, Becker

CR, Chesebro JH, Farb A, Galis ZS, Jackson C, Jang IK, Koenig W, Lodder RA, March K, Demirovic

J, Navab M, Priori SG, Rekhter MD, Bahr R, Grundy SM, Mehran R, Colombo A, Boerwinkle E,

Ballantyne C, Insull W Jr, Schwartz RS, Vogel R, Serruys PW, Hansson GK, Faxon DP, Kaul S,

Drexler H, Greenland P, Muller JE, Virmani R, Ridker PM, Zipes DP, Shah PK, Willerson JT. From

Vulnerable Plaque to Vulnerable Patient: A call for new definitions and risk assessment strategies”.

“Current Perspective” Part II. Circulation. 2003; 108:1772-1778

150 Blomstrom-Lundqvist C, Scheinman MM, Aliot EM, Alpert JS, Calkins H, Camm AJ, Campbell WB,

Haines DE, Kuck KH, Lerman BB, Miller DD, Shaeffer CW, Stevenson WG, Tomaselli GF, Antman

EM, Smith SC, Alpert JS, Faxon DP, Fuster V, Gibbons RJ, Gregoratos G, Hiratzka LF, Hunt SA,

Jacobs AK, Russell RO, Priori SG, Blanc JJ, Budaj A, Burgos EF, Cowie M, Deckers JW, Garcia

MA, Klein WW, Lekakis J, Lindahl B, Mazzotta G, Morais JC, Oto A, Smiseth O, Trappe HJ.

ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias-

executive summary. a report of the American college of cardiology/American heart association task

force on practice guidelines and the European society of cardiology committee for practice guidelines

(writing committee to develop guidelines for the management of patients with supraventricular

arrhythmias) Developed in Collaboration with NASPE-Heart Rhythm Society. J Am Coll Cardiol.

2003; 42:1493-1531

151 Blomstrom-Lundqvist C, Scheinman MM, Aliot EM, Alpert JS, Calkins H, Camm AJ, Campbell WB,

Haines DE, Kuck KH, Lerman BB, Miller DD, Shaeffer CW, Stevenson WG, Tomaselli GF, Antman

EM, Smith SC, Alpert JS, Faxon DP, Fuster V, Gibbons RJ, Gregoratos G, Hiratzka LF, Hunt SA,

Jacobs AK, Russell RO, Priori SG, Blanc JJ, Budaj A, Burgos EF, Cowie M, Deckers JW, Garcia

MA, Klein WW, Lekakis J, Lindahl B, Mazzotta G, Morais JC, Oto A, Smiseth O, Trappe HJ.

ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias—

executive summary: a report of the American College of Cardiology/American Heart Association

Task Force on Practice Guidelines and the European Society of Cardiology Committee for Practice

Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With

Supraventricular Arrhythmias). Circulation. 2003; 108:1871-1909

152 Priori SG, Cerrone M. La Sindrome di Brugada. Recenti Progressi in Medicina 2003; 94;456-461

153 Cerrone M, Grillo M, Priori SG. La Sindrome di Brugada: aspetti clinici, criteri diagnostici,

stratificazione prognostica. GIAC 2003; 6:128-140

154 Zareba W, Moss AJ, Sheu G, Kaufman ES, Priori SG, Vincent GM, Towbin JA, Benhorin J, Schwartz

PJ, Napolitano C, Hall WJ, Keating MT, QI M, Robinson JL, Andrews ML for the International Long


QT Syndrome Registry, University of Rochester, New York. Location of Mutation in the KCNQ1 and

phenotypic presentation of Long QT Syndrome. J Cardiovasc Electrophysiol 2003;14:1149-1153.

155 Priori SG, Napolitano C, Vicentini A. Inherited arrhythmia syndromes: applying the molecular

biology and genetic to the clinical management. J Interv Card Electrophysiol. 2003; 9:93-101

156 Priori SG, Klein W, Bassand JP; ESC Committee for Practice Guidelines 2002-2004; ESC Committee

for Practice Guidelines 2000-2002; European Society of Cardiology 2002-2004. Medical practice

guidelines. Separating science from economics. Eur Heart J. 2003; 24:1962-4

157 Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer

WH 3rd, Spirito P, Ten Cate FJ, Wigle ED, Vogel RA, Abrams J, Bates ER, Brodie BR, Danias PG,

Gregoratos G, Hlatky MA, Hochman JS, Kaul S, Lichtenberg RC, Lindner JR, O'Rourke RA, Pohost

GM, Schofield RS, Tracy CM, Winters WL Jr, Klein WW, Priori SG, Alonso-Garcia A, Blomstrom-

Lundqvist C, De Backer G, Deckers J, Flather M, Hradec J, Oto A, Parkhomenko A, Silber S, Torbicki

A; American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents;

European Society of Cardiology Committee for Practice Guidelines. American college of

cardiology/european society of cardiology clinical expert consensus documen on hypertrophic

cardiomyopathy. A report of the american college of cardiology foundation task force on clinical

expert consensus documents and the european society of cardiology committee for practice guidelines.

Eur Heart J. 2003; 24:1965-91

158 Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer

WH 3rd, Spirito P, Ten Cate FJ, Wigle ED, Vogel RA, Abrams J, Bates ER, Brodie BR, Danias PG,

Gregoratos G, Hlatky MA, Hochman JS, Kaul S, Lichtenberg RC, Lindner JR, O'rourke RA, Pohost

GM, Schofield RS, Tracy CM, Winters WL Jr, Klein WW, Priori SG, Alonso-Garcia A, Blomstrom-

Lundqvist C, De Backer G, Deckers J, Flather M, Hradec J, Oto A, Parkhomenko A, Silber S, Torbicki

A; Task Force on Clinical Expert Consensus Documents. American College of Cardiology;

Committee for Practice Guidelines. European Society of Cardiology. American college of

cardiology/european society of cardiology clinical expert consensus document on hypertrophic

cardiomyopathy. A report of the american college of cardiology foundation task force on clinical

expert consensus documents and the european society of cardiology committee for practice guidelines.

J Am Coll Cardiol. 2003; 42:1687-713

159 Viatchenko-Karpinski S, Terentyev D, Gyorke I, Terentyeva R, Volpe P, Priori SG, Napolitano C,

Nori A, Williams SC, Gyorke S. Abnormal Calcium Signaling and Sudden Cardiac Death Associated

With Mutation of Calsequestrin. Circ Res. 2004; 94:471-477. Epub 2004 Jan 08

160 Patrono C, Bachmann F, Baigent C, Bode C, De Caterina R, Charbonnier B, Fitzgerald D, Hirsh J,

Husted S, Kvasnicka J, Montalescot G, Garcia Rodriguez LA, Verheugt F, Vermylen J, Wallentin L,

Priori SG, Alonso Garcia MA, Blanc JJ, Budaj A, Cowie M, Dean V, Deckers J, Fernandez Burgos

E, Lekakis J, Lindahl B, Mazzotta G, Morais J, Oto A, Smiseth OA, Morais J, Deckers J, Ferreira R,

Mazzotta G, Steg PG, Teixeira F, Wilcox R; European Society of Cardiology. Expert consensus

document on the use of antiplatelet agents. The task force on the use of antiplatelet agents in patients


with atherosclerotic cardiovascular disease of the European society of cardiology. Eur Heart J. 2004;

25:166-181.

161 Priori SG. Inherited arrhythmogenic diseases: the complexity beyond monogenic disorders. Circ Res.

2004; 94:140-145

162 Mok NS, Priori SG, Napolitano C, Chan KK, Bloise R, Chan HW, Fung WH, Chan YS, Chan WK,

Lam C, Chan NY, Tsang HH. Clinical profile and genetic basis of Brugada syndrome in the Chinese

population. Hong Kong Med J. 2004; 10:32-37

163 Horstkotte D, Follath F, Gutschik E, Lengyel M, Oto A, Pavie A, Soler-Soler J, Thiene G, Von

Graevenitz A, Priori SG, Angeles Alonso Garcia M, Blanc JJ, Budaj A, Cowie M, Dean V, Deckers

J, Fernandez Burgos E, Lekakis J, Lindahl B, Mazzotta G, Morais J, Oto A, Smiseth OA, Lekakis J,

Vahanian A, Delahaye F, Parkhomenko A, Filipatos G, Aldershvile J, Vardas P; Task Force Members;

ESC Committee for Practice Guidelines (CPG); Document Reviewers. Guidelines on prevention,

diagnosis and treatment of infective endocarditis executive summary; the task force on infective

endocarditis of the European society of cardiology. Eur Heart J. 2004; 25:267-276

164 Priori SG, Bossaert LL, Chamberlain DA, Napolitano C, Arntz HR, Koster RW, Monsieurs KG,

Capucci A, Wellens HJ. ESC-ERC recommendations for the use of automated external defibrillators

(AEDs) in Europe. Eur Heart J. 2004; 25:437-445

165 Priori SG, Bossaert LL, Chamberlain DA, Napolitano C, Arntz HR, Koster RW, Monsieurs KG,

Capucci A, Wellens HH. Policy statement: ESC-ERC recommendations for the use of automated

external defibrillators (AEDs) in Europe. Resuscitation. 2004; 60:245-252

166 Schwartz PJ, Priori SG, Cerrone M, Spazzolini C, Odero A, Napolitano C, Bloise R, De Ferrari GM,

Klersy C, Moss AJ, Zareba W, Robinson JL, Hall WJ, Brink PA, Toivonen L, Epstein AE, Li C, Hu

D. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-

QT syndrome. Circulation. 2004; 109:1826-1833. Epub 2004 Mar 29

167 Baroudi G, Napolitano C, Priori SG, Del Bufalo A, Chahine M. Loss of function associated with

novel mutations of the SCN5A gene in patients with Brugada syndrome. Can J Cardiol. 2004; 20:425-

430

168 Fenichel RR, Malik M, Antzelevitch C, Sanguinetti M, Roden DM, Priori SG, Ruskin JN, Lipicky

RJ, Cantilena LR; Independent Academic Task Force. Drug-induced torsades de pointes and

implications for drug development. J Cardiovasc Electrophysiol. 2004; 15:475-95

169 De Rosa G, Delogu AB, Piastra M, Chiaretti A, Bloise R, Priori SG. Catecholaminergic polymorphic

ventricular tachycardia: successful emergency treatment with intravenous propranolol. Pediatr Emerg

Care 2004; 20:175-177.

170 Priori SG. From trials to guidelines to clinical practice: the need for improvement. Europace 2004;

6:176-178.

171 Di Paolo M, Luchini D, Bloise R, Priori SG. Postmortem molecular analysis in victims of sudden

unexplained death. Am J Forensic Med Pathol. 2004;25:182-184


172 Hasdemir C, Priori SG, Overholt E, Lazzara R. Catecholaminergic Polymorphic Ventricular

Tachycardia, Recurrent Syncope, and Implantable Loop Recorder. J Cardiovasc Electrophysiol. 2004;

15:729

173 Mohler PJ, Splawski I, Napolitano C, Bottelli G, Sharpe L, Timothy K, Priori SG, Keating MT,

Bennett V. A cardiac arrhythmia syndrome caused by loss of ankyrin-B function. Proc Natl Acad Sci

U S A. 2004; 101:9137-9142

174 Priori SG, Napolitano C. Genetics of cardiac arrhythmias and sudden cardiac death. Ann N Y Acad

Sci. 2004; 1015:96-110

175 Shimizu W, Horie M, Ohno S, Takenaka K, Yamaguchi M, Shimizu M, Washizuka T, Aizawa Y,

Nakamura K, Ohe T, Aiba T, Miyamoto Y, Yoshimasa Y, Towbin JA, Priori SG, Kamakura S.

Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the

LQT1 form of congenital long QT syndrome: multicenter study in Japan. J Am Coll Cardiol. 2004;

44:117-125

176 Quaglini S, Rognoni C, Priori SG, Schwartz PJ. Cost-effectiveness of Neonatal ECG as a Screening

for Long QT-syndrome: A Decision Analysis Approach. Medinfo. 2004; 2004(CD):1821

177 Priori SG, Napolitano C, Schwartz PJ, Grillo M, Bloise R, Ronchetti E, Moncalvo C, Tulipani C,

Veia A, Bottelli G, Nastoli J. Association of long QT syndrome loci and cardiac events among patients

treated with beta-blockers. JAMA. 2004; 292:1341-1344

178 Splawski I, Timothy KW, Sharpe LM, Decher N, Kumar P, Bloise R, Napolitano C, Schwartz PJ,

Joseph RM, Condouris K, Tager-Flusberg H, Priori SG, Sanguinetti MC, Keating MT. Ca(V)1.2

calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism. Cell.

2004; 119:19-31

179 Mohler PJ, Rivolta I, Napolitano C, Lemaillet G, Lambert S, Priori SG, Bennett V. Nav1.5 E1053K

mutation causing Brugada syndrome blocks binding to ankyrin-G and expression of Nav1.5 on the

surface of cardiomyocytes. Proc Natl Acad Sci U S A. 2004; 101:17533-17538.

180 Brignole M, Alboni P, Benditt DG, Bergfeldt L, Blanc JJ, Thomsen PE, Gert van Dijk J, Fitzpatrick

A, Hohnloser S, Janousek J, Kapoor W, Kenny RA, Kulakowski P, Masotti G, Moya A, Raviele A,

Sutton R, Theodorakis G, Ungar A, Wieling W, Priori SG, Garcia MA, Budaj A, Cowie M, Deckers

J, Burgos EF, Lekakis J, Lindhal B, Mazzotta G, Morais J, Oto A, Smiseth O, Menozzi C, Ector H,

Vardas P; Task Force on Syncope, European Society of Cardiology.Guidelines on management

(diagnosis and treatment) of syncope-update 2004. Executive Summary. Eur Heart J. 2004; 25:2054-

2072

181 Gasparini M, Lunati M, Galimberti P, Bloise R, Fiore I, Priori SG Images in cardiovascular medicine.

Endocardial implantation of a cardioverter-defibrillator in a 13-month-old child affected by long-QT

syndrome and syndactyly. Circulation. 2004; 110:e525-527

182 Gang ES, Priori SG, Chen PS. Short coupled premature ventricular contraction initiating ventricular

fibrillation in a patient with Brugada syndrome. J Cardiovasc Electrophysiol. 2004;15:837


183 Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, Olschewski H, Peacock A,

Pietra G, Rubin LJ, Simonneau G, Priori SG, Garcia MA, Blanc JJ, Budaj A, Cowie M, Dean V,

Deckers J, Burgos EF, Lekakis J, Lindahl B, Mazzotta G, McGregor K, Morais J, Oto A, Smiseth OA,

Barbera JA, Gibbs S, Hoeper M, Humbert M, Naeije R, Pepke-Zaba J; Task Force. Guidelines on

diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and

Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J.

2004; 25:2243-2278

184 Santomauro M, Ottaviano L, Borrelli A, Riganti C, Priori SG, Napolitano C, Chiariello M. Evaluation

of BLS-D training in lay people. Ital Heart J Suppl. 2004; 5:527-533

185 Nieminen MS, Bohm M, Cowie MR, Drexler H, Filippatos GS, Jondeau G, Hasin Y, Lopez-Sendon

J, Mebazaa A, Metra M, Rhodes A, Swedberg K, Priori SG, Garcia MA, Blanc JJ, Budaj A, Cowie

MR, Dean V, Deckers J, Burgos EF, Lekakis J, Lindahl B, Mazzotta G, Morais J, Oto A, Smiseth OA,

Garcia MA, Dickstein K, Albuquerque A, Conthe P, Crespo-Leiro M, Ferrari R, Follath F, Gavazzi

A, Janssens U, Komajda M, Morais J, Moreno R, Singer M, Singh S, Tendera M, Thygesen K; ESC

Committe for Practice Guideline (CPG). Executive summary of the guidelines on the diagnosis and

treatment of acute heart failure: the Task Force on Acute Heart Failure of the European Society of

Cardiology. Eur Heart J. 2005; 26:384-416

186 Corrado D, Pelliccia A, Bjornstad HH, Vanhees L, Biffi A, Borjesson M, Panhuyzen-Goedkoop N,

Deligiannis A, Solberg E, Dugmore D, Mellwig KP, Assanelli D, Delise P, van-Buuren F, Anastasakis

A, Heidbuchel H, Hoffmann E, Fagard R, Priori SG, Basso C, Arbustini E, Blomstrom-Lundqvist C,

McKenna WJ, Thiene G. Cardiovascular pre-participation screening of young competitive athletes for

prevention of sudden death: proposal for a common European protocol: Consensus Statement of the

Study Group of Sport Cardiology of the Working Group of Cardiac Rehabilitation and Exercise

Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society

of Cardiology. Eur Heart J. 2005; 26:516-24.

187 Priori SG, Pandit SV, Rivolta I, Berenfeld O, Ronchetti E, Dhamoon A, Napolitano C, Anumonwo

J, di Barletta MR, Gudapakkam S, Bosi G, Stramba-Badiale M, Jalife J. A novel form of short QT

syndrome (SQT3) is caused by a mutation in the KCNJ2 gene. Circ Res. 2005; 96:800-7. Epub 2005

Mar 10

188 Francis J, Sankar V, Krishnan Nair V, Priori SG. Catecholaminergic polymorphic ventricular

tachycardia. Heart Rhythm 2005; 2:550-4.

189 Priori SG, Cerrone M Molecular genetics: is it making an impact in the management of inherited

arrhythmogenic syndromes? Hell J Cardiol. 2005; 46:83-7

190 Priori SG, Cerrone M. Genetic arrhythmias. Ital Heart J. 2005; 6:241-8

191 Priori SG. From Genes to Cell Therapy: Molecular Medicine Meets Clinical EP. J Cardiovasc

Electrophysiol. 2005; 16:552


192 Cerrone M, Colombi B, Santoro M, Raffaele di Barletta M, Scelsi M, Villani L, Napolitano C, Priori

SG. Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model

Carrier of a Mutation in the Cardiac Ryanodine Receptor (RyR2). Circ Res. 2005; 96:e77-82

193 Lane RD, Laukes C, Marcus FI, Chesney MA, Sechrest L, Gear K, Fort CL, Priori SG, Schwartz PJ,

Steptoe A. Psychological stress preceding idiopathic ventricular fibrillation. Psychosom Med. 2005;

67:359-65

194 Priori SG, Napolitano C. Should patients with an asymptomatic Brugada electrocardiogram undergo

pharmacological and electrophysiological testing? Circulation. 2005; 112:279-292; discussion 279-

292

195 Stramba-Badiale M, Priori SG. Gender-specific prescription for cardiovascular diseases? Eur Heart

J. 2005; 26:1571-1572. Epub 2005 Jul 19

196 Priori SG, Napolitano C. Cardiac and skeletal muscle disorders caused by mutations in the

intracellular Ca release channels. J Clin Invest. 2005; 115: 2033–2038

197 Priori SG, Napolitano C. Intracellular calcium handling dysfunction and arrhythmogenesis: a new

challenge for the electrophysiologist. Circ Res. 2005; 97:1077-1079

198 Frustaci A, Priori SG, Pieroni M, Chimenti C, Napolitano C, Rivolta I, Sanna T, Bellocci F, Russo

MA. Cardiac Histological Substrate in Patients With Clinical Phenotype of Brugada Syndrome.

Circulation 2005; 112:3680-3687

199 Gasparini M, Regoli F, Galimberti P, Priori SG. Endocardial implantation of a cardioverter

defibrillator in early childhood. J Cardiovasc Electrophysiol 2005; 16:1381-1383

200 Napolitano C, Priori SG, Schwartz PJ, Bloise R, Ronchetti E, Nastoli J, Bottelli G, Cerrone M,

Leonardi S. Genetic testing in the long QT syndrome: development and validation of an efficient

approach to genotyping in clinical practice. JAMA 2005; 294:2975-2980

201 Napolitano C, Bloise R, Priori SG. Gene-specific therapy for inherited arrhythmogenic diseases.

Pharmacol Ther. 2006 Apr;110(1):1-13. Epub 2005 Sep 15

202 Priori SG, Napolitano C. Role of genetic analyses in cardiology: part I: mendelian diseases: cardiac

channelopathies. Circulation 2006; 113:1130-1135

203 Stramba-Badiale, Fox KM, Priori SG, Collin P, Daly C, Graham I, Jonnson B, Schneck-Gustaffson

K, Tendera M. Cardiovascular diseases in women: a statement from the policy conference of the

European Society of Cardiology. Eur Heart J 2006; 27:994-1005

204 Vecchietti S, Rivolta I, Severi S, Napolitano C, Priori SG, Cavalcanti S. Computer simulation of

wild-type and mutant human cardiac Na+ current. Med Biol Eng Comput. 2006; 44:35-44

205 Terentyev D, Nori A, Santoro M, Viatchenko-Karpinski S, Kubalova Z, Gyorke I, Terentyeva R,

Vedamoorthyrao S, Blom NA, Valle G, Napolitano C, Williams SC, Volpe P, Priori SG, Gyorke S.

Abnormal Interactions of Calsequestrin With the Ryanodine Receptor Calcium Release Channel

Complex Linked to Exercise-Induced Sudden Cardiac Death. Circ Res 2006; 98:1151-1158

206 Priori SG, Napolitano C, Cerrone M. Experimental therapy of genetic arrhythmias: disease-specific

pharmacology. Handb Exp Pharmacol. 2006; (171):267-86


207 Auricchio A, Gropp M, Ludgate S, Vardas P, Brugada J, Priori SG, Writing Committee for the

European Heart Rhythm Association Guidance Document on Cardiac Rhythm Management Product

Performance. European Heart Rhythm Association Guidance Document on cardiac rhythm

management product performance. Europace 2006; 8(5):313-22

208 Priori SG, Napolitano C, Gasparini M. Letter regarding article by Coronel et al, "right ventricular

fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined

electrophysiological, genetic, histopathologic, and computational study". Circulation. 2006;

113(16):e726; author reply 726-7

209 Napolitano C. Bloise R, Priori SG. Long QT syndrome and short QT syndrome: how to make correct

diagnosis and what about eligibility for sports activity. J Cardiovasc Med 2006; 7:250-256. Review

210 Liu N, Colombi B, Memmi M, Zissimopoulos S, Rizzi N, Negri S, Imbriani M, Napolitano C, Lai FA,

Priori SG. Arrhythmogenesis in Catecholaminergic Polymorphic Ventricular Tachycardia: insights

from a RyR2 R4496C knock-in mouse model. Circ Res 2006; 99:292-298

211 Raffaele di Barletta M, Viatchenko-Karpinski S, Nori A, Memmi M, Terentyev D, Turcato F, Valle

G, Napoltitano C, Gyorke S, Volpe P, Priori SG. Clinical phenotype and functional characterization

of CASQ2 mutations associated with Catecholaminergic Polymorphic Ventricular Tachycardia.

Circulation. 2006; 114:1012-1019

212 Fox K, Garcia MA, Ardissino D, Buszman P, Camici PG, Crea F, Daly C, De Backer G, Hjemdahl P,

Lopez-Sendon J, Marco J, Morais J, Pepper J, Sechtem U, Simoons M, Thygesen K, Priori SG, Blanc

JJ, Budaj A, Camm J, Dean V, Deckers J, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J,

Osterspey A, Tamargo J, Zamorano JL; Task Force on the Management of Stable Angina Pectoris of

the European Society of Cardiology; ESC Committee for Practice Guidelines (CPG). Guidelines on

the management of stable angina pectoris: executive summary: the Task Force on the Management of

Stable Angina Pectoris of the European Society of Cardiology. Eur Heart J. 2006; 27:1341-1381.

213 Quaglini S, Rognoni C, Spazzolini C, Priori SG, Mannarino S, Schwartz PJ. Cost-effectiveness of

neonatal ECG screening for the long QT syndrome. Eur Heart J. 2006; 27:1824-1832

214 Priori SG, Napolitano C. Molecular underpinning of "good luck". Circulation. 2006; 114:360-362

215 European Heart Rhythm Association; Heart Rhythm Society; Fuster V, Ryden LE, Cannom DS, Crijns

HJ, Curtis AB, Ellenbogen KA, Halperin JL, Le Heuzey JY, Kay GN, Lowe JE, Olsson SB,

Prystowsky EN, Tamargo JL, Wann S, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Antman

EM, Hunt SA, Nishimura R, Ornato JP, Page RL, Riegel B, Priori SG, Blanc JJ, Budaj A, Camm AJ,

Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey

A, Zamorano JL; American College of Cardiology; American Heart Association Task Force on

Practice Guidelines; European Society of Cardiology Committee for Practice Guidelines; Writing

Committee to Revise the 2001 Guidelines for the Management of Patients With Atrial Fibrillation.

ACC/AHA/ESC 2006 guidelines for the management of patients with atrial fibrillation--executive

summary: a report of the American College of Cardiology/American Heart Association Task Force

on Practice Guidelines and the European Society of Cardiology Committee for Practice Guidelines


(Writing Committee to Revise the 2001 Guidelines for the Management of Patients With Atrial

Fibrillation).J Am Coll Cardiol. 2006; 48:854-906

216 Fuster V, Ryden LE, Cannom DS, Crijns HJ, Curtis AB, Ellenbogen KA, Halperin JL, Le Heuzey JY,

Kay GN, Lowe JE, Olsson SB, Prystowsky EN, Tamargo JL, Wann S, Smith SC Jr, Jacobs AK, Adams

CD, Anderson JL, Antman EM, Halperin JL, Hunt SA, Nishimura R, Ornato JP, Page RL, Riegel B,

Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J,

McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL; American College of

Cardiology/American Heart Association Task Force on Practice Guidelines; European Society of

Cardiology Committee for Practice Guidelines; European Heart Rhythm Association; Heart Rhythm

Society. ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation: a

report of the American College of Cardiology/American Heart Association Task Force on Practice

Guidelines and the European Society of Cardiology Committee for Practice Guidelines (Writing

Committee to Revise the 2001 Guidelines for the Management of Patients With Atrial Fibrillation):

developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm

Society. Circulation. 2006; 114:e257-354.

217 Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss

AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Blanc JJ, Budaj A, Camm

AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J,

Osterspey A, Tamargo JL, Zamorano JL, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson

JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B. ACC/AHA/ESC 2006

guidelines for management of patients with ventricular arrhythmias and the prevention of sudden

cardiac death--executive summary: A report of the American College of Cardiology/American Heart

Association Task Force and the European Society of Cardiology Committee for Practice Guidelines

(Writing Committee to Develop Guidelines for Management of Patients with Ventricular Arrhythmias

and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart

Rhythm Association and the Heart Rhythm Society.Eur Heart J. 2006; 27:2099-2140


AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Blanc JJ, Budaj A, Camm

AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J,

Osterspey A, Tamargo JL, Zamorano JL, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson

JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B. ACC/AHA/ESC 2006

guidelines for management of patients with ventricular arrhythmias and the prevention of sudden

cardiac death: A report of the American College of Cardiology/American Heart Association Task

Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee

to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention

of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association

and the Heart Rhythm Society. Europace. 2006; 8:746-837



AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK,

Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL,

Riegel B, Blanc JJ, Budaj A, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K,

Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL; American College of

Cardiology/American Heart Association Task Force; European Society of Cardiology Committee for

Practice Guidelines; European Heart Rhythm Association; Heart Rhythm Society. ACC/AHA/ESC

2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of

Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association

Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing

committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the

Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm

Association and the Heart Rhythm Society. Circulation. 2006; 114:e385-484

220 European Heart Rhythm Association; Heart Rhythm Society; Zipes DP, Camm AJ, Borggrefe M,

Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG,

Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM,

Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Priori SG, Blanc

JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra

M, Morais J, Osterspey A, Tamargo JL, Zamorano JL; American College of Cardiology; American

Heart Association Task Force; European Society of Cardiology Committee for Practice Guidelines.

ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the

prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart

Association Task Force and the European Society of Cardiology Committee for Practice Guidelines

(Writing Committee to Develop Guidelines for Management of Patients With Ventricular

Arrhythmias and the Prevention of Sudden Cardiac Death).J Am Coll Cardiol. 2006; 48:e247-346

221 Hobbs JB, Peterson DR, Moss AJ, McNitt S, Zareba W, Goldenberg I, Qi M, Robinson JL, Sauer AJ,

Ackerman MJ, Benhorin J, Kaufman ES, Locati EH, Napolitano C, Priori SG, Towbin JA, Vincent

GM, Zhang L. Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-

QT syndrome.JAMA. 2006; 296:1249-1254

222 Napolitano C, Priori SG. Brugada syndrome. Orphanet J Rare Dis. 2006; 1:35


Kay GN, Lowe JE, Olsson SB, Prystowsky EN, Tamargo JL, Wann S, Smith SC Jr, Jacobs AK, Adams

CD, Anderson JL, Antman EM, Halperin JL, Hunt SA, Nishimura R, Ornato JP, Page RL, Riegel B,

Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J,

McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL. ACC/AHA/ESC 2006

guidelines for the management of patients with atrial fibrillation: full text: A report of the American

College of Cardiology/American Heart Association Task Force on practice guidelines and the

European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Revise the


2001 Guidelines for the Management of Patients With Atrial Fibrillation) Developed in collaboration

with the European Heart Rhythm Association and the Heart Rhythm Society.Europace. 2006; 8:651-

745.

224 Choe CU, Schulze-Bahr E, Neu A, Xu J, Zhu ZI, Sauter K, Bahring R, Priori SG, Guicheney P,

Monnig G, Napolitano C, Heidemann J, Clancy CE, Pongs O, Isbrandt D. C-terminal HERG (LQT2)

mutations disrupt IKr channel regulation through 14-3-3{epsilon}. Hum Mol Genet. 2006; 15:2888-

2902

225 Stramba Badiale M, Priori SG. Current Strategies to Diminish the Impact of Cardiovascular Diseases

in Women. Rev Esp Cardiol. 2006; 59(11):1190-1193

226 Task Force per il Trattamento dell'Angina Pectoris Stabile della Societa Europea di Cardiologia; Fox

K, Alonso Garcia MA, Ardissino D, Buszman P, Camici PG, Crea F, Daly C, De Backer G, Hjemdahl

P, Lopez-Sendon J, Marco J, Morais J, Pepper J, Sechtem U, Simoons M, Thygesen K, Priori SG,

Blanc JJ, Budaj A, Camm J, Dean V, Deckers J, Dickstein K, Lekakis J, McGregor K, Metra M,

Osterspey A, Tamargo J, Zamorano JL, Andreotti F, Becher H, Dietz R, Fraser A, Gray H, Hernandez

Antolin RA, Huber K, Kremastinos DT, Maseri A, Nesser HJ, Pasierski T, Sigwart U, Tubaro M,

Weis M. Guidelines on the management of stable angina pectoris: executive summary G Ital Cardiol

(Rome). 2006; 7(8):535-583


AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C. Guidelines for

Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death.

Executive Summary. Rev Esp Cardiol. 2006; 59(12):1328

228 Ryden L, Standl E, Bartnik M, Van den Berghe G, Betteridge J, de Boer MJ, Cosentino F, Jonsson B,

Laakso M, Malmberg K, Priori S, Ostergren J, Tuomilehto J, Thrainsdottir I, Vanhorebeek I, Stramba-

Badiale M, Lindgren P, Qiao Q, Priori SG, Blanc JJ, Budaj A, Camm J, Dean V, Deckers J, Dickstein

K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo J, Zamorano JL, Deckers JW,

Bertrand M, Charbonnel B, Erdmann E, Ferrannini E, Flyvbjerg A, Gohlke H, Juanatey JR, Graham

I, Monteiro PF, Parhofer K, Pyorala K, Raz I, Schernthaner G, Volpe M, Wood D; Task Force on

Diabetes and Cardiovascular Diseases of the European Society of Cardiology (ESC); European

Association for the Study of Diabetes (EASD). Guidelines on diabetes, pre-diabetes, and

cardiovascular diseases: executive summary. The Task Force on Diabetes and Cardiovascular

Diseases of the European Society of Cardiology (ESC) and of the European Association for the Study

of Diabetes (EASD). European Heart J 2007; 28:88-136

229 Vahanian A, Baumgartner H, Bax J, Butchart E, Dion R, Filippatos G, Flachskampf F, Hall R, Iung

B, Kasprzak J, Nataf P, Tornos P, Torracca L, Wenink A, Priori SG, Blanc JJ, Budaj A, Camm J,

Dean V, Deckers J, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo

J, Zamorano JL, Angelini A, Antunes M, Fernandez MA, Gohlke-Baerwolf C, Habib G, McMurray

J, Otto C, Pierard L, Pomar JL, Prendergast B, Rosenhek R, Uva MS, Tamargo J. Guidelines on the


management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease

of the European Society of Cardiology. Europ Heart J 2007; 28(2):230-68. Epub 2007 Jan 26

230 Sauer AJ, Moss AJ, McNitt S, Peterson DR, Zareba W, Robinson JL, Qi M, Goldenberg I, Hobbs JB,

Ackerman MJ, Benhorin J, Hall WJ, Kaufman ES, Locati EH, Napolitano C, Priori SG, Schwartz PJ,

Towbin JA, Vincent GM, Zhang L. Long QT syndrome in adults. J Am Coll Cardio 2007; 49(3):329-

37. Epub 2007 Jan 4

231 Rossenbacker T, Priori SG. Clinical diagnosis of Long QT Syndrome: back to the caliper. Eur Heart

J 2007; 28(5):527-528. Epub 2007 Feb 20

232 Seth R, Moss AJ, McNitt S, Zareba W, Andrews ML, Qi M, Robinson JL, Goldenberg I, Ackerman

MJ, Benhorin J, Kaufman ES, Locati EH, Napolitano C, Priori SG, Schwartz PJ, Towbin JA, Vincent

GM, Zhang L. Long QT syndrome and pregnancy.J Am Coll Cardiol. 2007; 49(10):1092-8. Epub

2007 Feb 27

233 Rossenbacker T, Priori SG. The Brugada syndrome. Curr Opin Cardiol. 2007; 22(3):163-70

234 Napolitano C, Priori SG. Diagnosis and treatment of catecholaminergic polymorphic ventricular

tachycardia. Heart Rhythm. 2007; 4(5):675-8. Epub 2007 Jan 7

235 Liu N, Colombi B, Raytcheva-Buono EV, Bloise R, Priori SG. Catecholaminergic Polymorphic

Ventricular Tachycardia. Herz. 2007; 32(3):212-217

236 Ruan Y, Liu N, Bloise R, Napolitano C, Priori SG. Gating Properties of SCN5A Mutations and the

Response to Mexiletine in Long-QT Syndrome Type 3 Patients.Circulation. 2007; 116(10):1137-44.

237 Mohamed U, Napolitano C, Priori SG. Molecular and electrophysiological bases of

catecholaminergic polymorphic ventricular tachycardia.J Cardiovasc Electrophysiol. 2007;

18(7):791-7. Review.

238 Rossenbacker T, Priori SG. New insights into the long-QT syndrome

Rev Esp Cardiol. 2007; 60(7):675-82. Review. Spanish


Kay GN, Lowe JE, Olsson SB, Prystowsky EN, Tamargo JL, Wann S, Priori SG, Blanc JJ, Budaj A,

Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais

J, Osterspey A, Zamorano JL, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Antman EM, Hunt

SA, Nishimura R, Ornato JP, Page RL, Riegel B; American College of Cardiology; American Heart

Association; European Society of Cardiology. ACC/AHA/ESC 2006 guidelines for the management

of patients with atrial fibrillation--excutive summary] Rev Port Cardiol. 2007; 26(4):383-446.

Portuguese.

240 Authors/Task Force Members, Graham I, Atar D, Borch-Johnsen K, Boysen G, Burell G, Cifkova R,

Dallongeville J, De Backer G, Ebrahim S, Gjelsvik B, Herrmann-Lingen C, Hoes A, Humphries S,

Knapton M, Perk J, Priori SG, Pyorala K, Reiner Z, Ruilope L, Sans-Menendez S, Scholte Op Reimer

W, Weissberg P, Wood D, Yarnell J, Zamorano JL; Other experts who contributed to parts of the

guidelines:, Walma E, Fitzgerald T, Cooney MT, Dudina A; European Society of Cardiology (ESC)

Committee for Practice Guidelines (CPG):, Vahanian A, Camm J, De Caterina R, Dean V, Dickstein


K, Funck-Brentano C, Filippatos G, Hellemans I, Kristensen SD, McGregor K, Sechtem U, Silber S,

Tendera M, Widimsky P, Zamorano JL; Document reviewers:, Hellemans I, Altiner A, Bonora E,

Durrington PN, Fagard R, Giampaoli S, Hemingway H, Hakansson J, Kjeldsen SE, Larsen ML,

Mancia G, Manolis AJ, Orth-Gomer K, Pedersen T, Rayner M, Ryden L, Sammut M, Schneiderman

N, Stalenhoef AF, Tokgozoglu L, Wiklund O, Zampelas A. European guidelines on cardiovascular

disease prevention in clinical practice: executive summary: Fourth Joint Task Force of the European

Society of Cardiology and Other Societies on Cardiovascular Disease Prevention in Clinical Practice

(Constituted by representatives of nine societies and by invited experts).Eur Heart J. 2007;

28(19):2375-414. Epub 2007 Aug 28

241 Authors/Task Force Members, Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H,

Gasparini M, Linde C, Morgado FB, Oto A, Sutton R, Trusz-Gluza M; ESC Committee for Practice

Guidelines (CPG), Vahanian A, Camm J, De Caterina R, Dean V, Dickstein K, Funck-Brentano C,

Filippatos G, Hellemans I, Kristensen SD, McGregor K, Sechtem U, Silber S, Tendera M, Widimsky

P, Zamorano JL; Document Reviewers, Priori SG, Blomstrom-Lundqvist C, Brignole M, Terradellas

JB, Camm J, Castellano P, Cleland J, Farre J, Fromer M, Le Heuzey JY, Lip GY, Merino JL,

Montenero AS, Ritter P, Schalij MJ, Stellbrink C. Guidelines for cardiac pacing and cardiac

resynchronization therapy: The Task Force for Cardiac Pacing and Cardiac Resynchronization

Therapy of the European Society of Cardiology. Developed in Collaboration with the European Heart

Rhythm Association.Eur Heart J. 2007; 28(18):2256-95. Epub 2007 Aug 28.

242 Authors/Task Force Members, Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H,

Gasparini M, Linde C, Morgado FB, Oto A, Sutton R, Trusz-Gluza M; ESC Committee for Practice

Guidelines (CPG), Vahanian A, Camm J, De Caterina R, Dean V, Dickstein K, Funck-Brentano C,

Filippatos G, Hellemans I, Kristensen SD, McGregor K, Sechtem U, Silber S, Tendera M, Widimsky

P, Zamorano JL; Document Reviewers, Priori SG, Blomstrom-Lundqvist C, Brignole M, Terradellas

JB, Camm J, Castellano P, Cleland J, Farre J, Fromer M, Le Heuzey JY, Lip GY, Merino JL,

Montenero AS, Ritter P, Schalij MJ, Stellbrink C. Guidelines for cardiac pacing and cardiac

resynchronization therapy: The Task Force for Cardiac Pacing and Cardiac Resynchronization

Therapy of the European Society of Cardiology. Developed in Collaboration with the European Heart

Rhythm Association.Europace. 2007; 9(10):959-98. Epub 2007 Aug 28

243 Cerrone M, Noujaim SF, Tolkacheva EG, Talkachou A, O'connell R, Berenfeld O, Anumonwo J,

Pandit SV, Vikstrom K, Napolitano C, Priori SG, Jalife J. Arrhythmogenic Mechanisms in a Mouse

Model of Catecholaminergic Polymorphic Ventricular Tachycardia. Circ Res. 2007; 101(10):1039-

48. Epub 2007 Sep 13

244 Graham I, Atar D, Borch-Johnsen K, Boysen G, Burell G, Cifkova R, Dallongeville J, De Backer G,

Ebrahim S, Gjelsvik B, Herrmann-Lingen C, Hoes A, Humphries S, Knapton M, Perk J, Priori SG,

Pyorala K, Reiner Z, Ruilope L, Sans-Menendez S, Op Reimer WS, Weissberg P, Wood D, Yarnell

J, Zamorano JL; ESC Committee for Practice Guidelines.European guidelines on cardiovascular


disease prevention in clinical practice: executive summary.Atherosclerosis. 2007; 194(1):1-45. Epub

2007 Sep 18. No abstract available.

245 Thygesen K, Alpert JS, White HD; Joint ESC/ACCF/AHA/WHF Task Force for the Redefinition of

Myocardial Infarction, Jaffe AS, Apple FS, Galvani M, Katus HA, Newby LK, Ravkilde J, Chaitman

B, Clemmensen PM, Dellborg M, Hod H, Porela P, Underwood R, Bax JJ, Beller GA, Bonow R, Van

der Wall EE, Bassand JP, Wijns W, Ferguson TB, Steg PG, Uretsky BF, Williams DO, Armstrong

PW, Antman EM, Fox KA, Hamm CW, Ohman EM, Simoons ML, Poole-Wilson PA, Gurfinkel EP,

Lopez-Sendon JL, Pais P, Mendis S, Zhu JR, Wallentin LC, Fernández-Avilés F, Fox KM,

Parkhomenko AN, Priori SG, Tendera M, Voipio-Pulkki LM, Vahanian A, Camm AJ, De Caterina

R, Dean V, Dickstein K, Filippatos G, Funck-Brentano C, Hellemans I, Kristensen SD, McGregor K,

Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano JL, Morais J, Brener S, Harrington R,

Morrow D, Lim M, Martinez-Rios MA, Steinhubl S, Levine GN, Gibler WB, Goff D, Tubaro M,

Dudek D, Al-Attar N.Universal definition of myocardial infarction.Circulation. 2007; 116(22):2634-

53. Epub 2007 Oct 19. No abstract available.




J, Zamorano JL, Walma E, Fitzgerald T, Cooney MT, Dudina A, Vahanian A, Camm J, De Caterina

R, Dean V, Dickstein K, Funck-Brentano C, Filippatos G, Hellemans I, Kristensen SD, McGregor K,

Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano JL, Altiner A, Bonora E, Durrington PN,

Fagard R, Giampaoli S, Hemingway H, Hakansson J, Kjeldsen SE, Larsen ML, Mancia G, Manolis

AJ, Orth-Gomer K, Pedersen T, Rayner M, Ryden L, Sammut M, Schneiderman N, Stalenhoef AF,

Tokgözoglu L, Wiklund O, Zampelas A; European Society of Cardiology (ESC); European

Association for Cardiovascular Prevention and Rehabilitation (EACPR); Council on Cardiovascular

Nursing; European Association for Study of Diabetes (EASD); International Diabetes Federation

Europe (IDF-Europe); European Stroke Initiative (EUSI); International Society of Behavioural

Medicine (ISBM); European Society of Hypertension (ESH); European Society of General

Practice/Family Medicine (ESGP/FM/WONCA); European Heart Network (EHN).European

guidelines on cardiovascular disease prevention in clinical practice: executive summary. Fourth Joint

Task Force of the European Society of Cardiology and other societies on cardiovascular disease

prevention in clinical practice (constituted by representatives of nine societies and by invited

experts).Eur J Cardiovasc Prev Rehabil. 2007; 14 Suppl 2:E1-40.




J, Zamorano JL, Walma E, Fitzgerald T, Cooney MT, Dudina A, Vahanian A, Camm J, De Caterina

R, Dean V, Dickstein K, Funck-Brentano C, Filippatos G, Hellemans I, Kristensen SD, McGregor K,

Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano JL, Altiner A, Bonora E, Durrington PN,


Fagard R, Giampaoli S, Hemingway H, Hakansson J, Kjeldsen SE, Larsen L, Mancia G, Manolis AJ,

Orth-Gomer K, Pedersen T, Rayner M, Ryden L, Sammut M, Schneiderman N, Stalenhoef AF,

Tokgözoglu L, Wiklund O, Zampelas A; European Society of Cardiology (ESC); European

Association for Cardiovascular Prevention and Rehabilitation (EACPR); Council on Cardiovascular

Nursing; European Association for Study of Diabetes (EASD); International Diabetes Federation

Europe (IDF-Europe); European Stroke Initiative (EUSI); Society of Behavioural Medicine (ISBM);

European Society of Hypertension (ESH);WONCA Europe (European Society of General

Practice/Family Medicine); European Heart Network (EHN); European Atherosclerosis Society

(EAS).European guidelines on cardiovascular disease prevention in clinical practice: full text. Fourth

Joint Task Force of the European Society of Cardiology and other societies on cardiovascular disease

prevention in clinical practice (constituted by representatives of nine societies and by invited

experts).Eur J Cardiovasc Prev Rehabil. 2007; 14 Suppl 2:S1-113.

248 Rydén L, Standl E, Bartnik M, Van den Berghe G, Betteridge J, de Boer MJ, Cosentino F, Jönsson B,

Laakso M, Malmberg K, Priori SG, Ostergren J, Tuomilehto J, Thrainsdottir I; Grupo de trabalho da

European Society of Cardiology; European Association for the Study of Diabetes. Guidelines for the

management of patients with ventricular arrhythmias and the prevention of sudden cardiac death--

executive summary Rev Port Cardiol. 2007; 26(11):1213-74

249 Giada F, Carlon R, Delise P, Biffi A, Priori SG, Raviele A, Santini M, Fattirolli F, Guiducci U, Penco

M, Belardinelli R, Agostoni PG; Task Force Multisocietaria; Federazione Medico Sportiva Italiana;

Società Italiana di Cardiologia dello Sport; Associazione Nazionale Cardiologia Extra-Ospedalieri;

Associazione Nazionale Medici Cardiologi Ospedalieri; Gruppo Italiano di Cardiologia Riabilitativa;

Società Italiana di Cardiologia.Consensus Statement of Multisocietary Task Force--prescription of

physical exercise in the cardiological environment (third part) Monaldi Arch Chest Dis. 2007;

68(3):134-48

250 Liu N, Priori SG. Disruption of calcium homeostasis and arrhythmogenesis induced by mutations in

the cardiac ryanodine receptor and calsequestrin. Cardiovasc Res. 2008; 77(2):293-301. Epub 2007

Aug 14

251 Boveri L, Priori SG. Molecular strategies for the electrophysiologist. Nat Clin Pract Cardiovasc Med.

2008; 5(3):117

252 Roguin A, Schwitter J, Vahlhaus C, Lombardi M, Brugada J, Vardas P, Auricchio A, Priori SG,

Sommer T. Magnetic resonance imaging in individuals with cardiovascular implantable electronic

devices. Europace. 2008; 10(3):336-46

253 Grupa Robocza Europejskiego Towarzystwa Kardiologicznego ds. stymulacji serca i resynchronizacji

we współpracy; Europejskim Towarzystwem Rytmu Serca, Vardas PE, Auricchio A, Blanc JJ,

Daubert JC, Drexler H, Ector H, Gasparini M, Linde C, Morgado FB, Oto A, Sutton R, Trusz-Gluza

M, Vahanian A, Camm J, De Caterina R, Dean V, Dickstein K, Funck-Brentano C, Filippatos G,

Hellemans I, Kristensen SD, McGregor K, Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano

JL, Priori SG, Blomström-Lundqvist C, Brignole M, Terradellas JB, Camm J, Castellano P, Cleland


J, Farre J, Fromer M, Le Heuzey JY, Lip GY, Merino JL, Montenero AS, Ritter P, Schlij MJ, Stellbrink

C; European Cardiac Society.Guidelines in cardiac pacing and resynchronization therapy.Kardiol Pol.

2007; 65(12):1449-87; discussion 1488-9.

254 Qin J, Valle G, Nani A, Nori A, Rizzi N, Priori SG, Volpe P, Fill M. Luminal Ca2+ regulation of

single cardiac ryanodine receptors: insights provided by calsequestrin and its mutants.J Gen Physiol.

2008; 131(4):325-34. Epub 2008 Mar 17

255 Chen PS, Priori SG. The Brugada syndrome. J Am Coll Cardiol. 2008; 51(12):1176-80

256 Thygesen K, Alpert JS, White HD; ESC; ACCF; AHA; WHF. Kardiol Pol. 2008; 66(1):47-62



Pyorala K, Reiner Z, Ruilope L, Sans-Menendez S, Reimer WS, Weissberg P, Wood D, Yarnell J,

Zamorano JL; Quarta Task Force Congiunta della Società Europea di Cardiologia e; di altre Società

sulla Prevenzione nelle Malattie Cardiovascolari nella Pratica Clinica (constituita da rappresentanti di

nove società e da esperti invitati).G Ital Cardiol (Rome). 2008; 9(1):11-59

258 Valle G, Galla D, Nori A, Priori SG, Gyorke S, De Filippis V, Volpe P. Catecholaminergic

polymorphic ventricular tachycardia-related mutations R33Q and L167H alter calcium sensitivity of

human cardiac calsequestrin. Biochem J. 2008; 413(2):291-303 Epub Apr 9

259 Goldenberg I, Moss AJ, Bradley J, Polonsky S, Peterson DR, McNitt S, Zareba W, Andrews ML,

Robinson JL, Ackerman MJ, Benhorin J, Kaufman ES, Locati EH, Napolitano C, Priori SG, Qi M,

Schwartz PJ, Towbin JA, Vincent GM, Zhang L. Long-QT syndrome after age 40.Circulation. 2008;

117(17):2192-201. Epub 2008 Apr 21

260 Goldenberg I, Moss AJ, Peterson DR, McNitt S, Zareba W, Andrews ML, Robinson JL, Locati EH,

Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Priori SG, Qi M, Schwartz PJ, Towbin JA,

Vincent GM, Zhang L. Risk factors for aborted cardiac arrest and sudden cardiac death in children

with the congenital long-QT syndrome. Circulation. 2008; 117(17):2184-91. Epub 2008 Apr 21

261 Wilkoff BL, Auricchio A, Brugada J, Cowie M, Ellenbogen KA, Gillis AM, Hayes DL, Howlett JG,

Kautzner J, Love CJ, Morgan JM, Priori SG, Reynolds DW, Schoenfeld MH, Vardas PE.HRS/EHRA

Expert Consensus on the Monitoring of Cardiovascular Implantable Electronic Devices (CIED):

Description of Techniques, Indications, Personnel, Frequency and Ethical Considerations. Europace.

2008; 10(6):707-25. Epub May 14

262 Kaufman ES, McNitt S, Moss AJ, Zareba W, Robinson JL, Hall WJ, Ackerman MJ, Benhorin J, Locati

ET, Napolitano C, Priori SG, Schwartz PJ, Towbin JA, Vincent GM, Zhang L. Risk of death in the

long QT syndrome when a sibling has died. Heart Rhythm. 2008; 5(6):831-6. Epub 2008 Mar 4.

263 Wilkoff BL, Auricchio A, Brugada J, Cowie M, Ellenbogen KA, Gillis AM, Hayes DL, Howlett JG,

Kautzner J, Love CJ, Morgan JM, Priori SG, Reynolds DW, Schoenfeld MH, Vardas PE; Heart

Rhythm Society; European Heart Rhythm Association; American College of Cardiology; American

Heart Association; European Society of Cardiology; Heart Failure Association of ESC; Heart Failure

Society of America.HRS/EHRA expert consensus on the monitoring of cardiovascular implantable


electronic devices (CIEDs): description of techniques, indications, personnel, frequency and ethical

considerations. Heart Rhythm. 2008; 5(6):907-25

264 Rizzi N, Liu N, Napolitano C, Nori A, Turcato F, Colombi B, Bicciato S, Arcelli D, Spedito A, Scelsi

M, Villani L, Esposito G, Boncompagni S, Protasi F, Volpe P, Priori SG. Unexpected structural and

functional consequences of the R33Q homozygous mutation in cardiac calsequestrin: a complex

arrhythmogenic cascade in a knock in mouse model. Circ Res. 2008; 103(3):298-306. Epub 2008 Jun

26

265 Liu N, Ruan Y, Priori SG.Catecholaminergic polymorphic ventricular tachycardia. Prog Cardiovasc

Dis. 2008; 51(1):23-30. Review

266 Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H, Gasparini M, Linde C, Morgado

FB, Oto A, Sutton R, Trusz-Gluza M; Grupo de Trabalho para Pacing Cardíaco e Terapia de

Ressincronização Cardíaca da European Society of Cardiology; European Heart Rhythm Association,

Vahanian A, Camm J, De Caterina R, Dean V, Dickstein K, Funck-Brentano C, Filippatos G,

Hellemans I, Kristensen SD, McGregor K, Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano

JL, Priori SG, Blomström-Lundqvist C, Brignole M, Terradellas JB, Camm J, Castellano P, Cleland

J, Farre J, Fromer M, Le Heuzey JY, Lip GY, Merino JL, Montenero AS, Ritter P, Schalij MJ,

Stellbrink C.Guidelines for cardiac pacing and cardiac resynchronization therapy Rev Port Cardiol.

2008; 27(5):639-87. Portuguese

267 Losito L, De Rinaldis M, Gennaro L, Priori SG, Bloise R, Bassi MT, Bresolin N, Trabacca A.

Charcot-Marie-Tooth type 1a in a child with Long QT syndrome. Eur J Paediatr Neurol. 2008;

13(5):459-62. Epub 2008 Sep 16.

268 Task Force for Diagnosis and Treatment of Acute and Chronic Heart Failure 2008 of European Society

of Cardiology, Dickstein K, Cohen-Solal A, Filippatos G, McMurray JJ, Ponikowski P, Poole-Wilson

PA, Strömberg A, van Veldhuisen DJ, Atar D, Hoes AW, Keren A, Mebazaa A, Nieminen M, Priori

SG, Swedberg K; ESC Committee for Practice Guidelines, Vahanian A, Camm J, De Caterina R,

Dean V, Dickstein K, Filippatos G, Funck-Brentano C, Hellemans I, Kristensen SD, McGregor K,

Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano JL.ESC Guidelines for the diagnosis and

treatment of acute and chronic heart failure 2008: the Task Force for the Diagnosis and Treatment of

Acute and Chronic Heart Failure 2008 of the European Society of Cardiology. Developed in

collaboration with the Heart Failure Association of the ESC (HFA) and endorsed by the European

Society of Intensive Care Medicine (ESICM).Eur Heart J. 2008 ; 29(19):2388-442. Epub 2008 Sep 17

269 Priori SG, Auricchio A, Nisam S, Yong P. To Replace or Not to Replace: A Systematic Approach to

Respond to Device Advisories. J Cardiovasc Electrophysiol. 2008; 20(2):164-70. Epub 2008 Sep 17

270 Thottathil P, Acharya J, Moss AJ, Jons C, McNitt S, Goldenberg I, Zareba W, Kaufman E, Qi M,

Robinson JL; International Long QT Syndrome Investigative Group Ackerman MJ, Benhorin J, Locati

E, Napolitano C, Priori S, Schwartz PJ, Spazzolini C, Towbin J, Vincent GM, Zhang L.Risk of cardiac

events in patients with asthma and long-QT syndrome treated with beta(2) agonists. Am J Cardiol.

2008; 102(7):871-4. Epub 2008 Jul 17.


271 Sze E, Moss AJ, Goldenberg I, McNitt S, Jons C, Zareba W, Qi M, Robinson JL; International Long

QT Syndrome Investigative Group. Long QT syndrome in patients over 40 years of age: increased

risk for LQTS-related cardiac events in patients with coronary disease.Ackerman MJ, Benhorin J,

Kaufman E, Locati E, Napolitano C, Priori S, Schwartz PJ, Spazzolini C, Towbin J, Vincent GM,

Zhang L. Ann Noninvasive Electrocardiol. 2008; 13(4):327-31

272 Swedberg K, Cleland J, Cowie MR, Nieminen M, Priori SG, Tavazzi L, van Veldhuisen DJ, Alonso-

Pulpon L, Camm J, Dickstein K, Drexler H, Filippatos G, Linde C, Lopez-Sendon J, Santini M,

Zannad F. Successful treatment of heart failure with devices requires collaboration. Eur J Heart Fail.

2008;10(12):1229-35. Epub 2008 Nov 12

273 Liu N, Rizzi N, Boveri L, Priori SG. Ryanodine receptor and calsequestrin in arrhythmogenesis: What

we have learnt from genetic diseases and transgenic mice. J Mol Cell Cardiol. 2009;46(2):149-59.

Epub 2008 Nov 5

274 Fernández-Velasco M, Rueda A, Rizzi N, Benitah JP, Colombi B, Napolitano C, Priori SG, Richard

S, Gómez AM. Increased Ca2+ Sensitivity of the Ryanodine Receptor Mutant RyR2R4496C

Underlies Catecholaminergic Polymorphic Ventricular Tachycardia. Circ Res. 2008 Dec 18

275 Grupo de Trabajo de ESC para el Diagnóstico y Tratamiento de Insuficiencia Cardiaca; Heart Failure

Association (HFA) de ESC; European Society of Intensive Care Medicine (ESICM), Dickstein K,

Cohen-Solal A, Filippatos G, McMurray JJ, Ponikowski P, Poole-Wilson PA, Strömberg A, van

Veldhuisen DJ, Atar D, Hoes AW, Keren A, Mebazaa A, Nieminen M, Priori SG, Swedberg K. ESC

guidelines for the diagnosis and treatment of acute and chronic heart failure 2008. Rev Esp Cardiol.

2008; 61(12):1329.e1-70

276 Fowler SJ, Priori SG. Clinical spectrum of patients with a Brugada ECG. Curr Opin Cardiol. 2009;

24(1):74-81

277 Vincent GM, Schwartz PJ, Denjoy I, Swan H, Bithell C, Spazzolini C, Crotti L, Piippo K, Lupoglazoff

JM, Villain E, Priori SG, Napolitano C, Zhang L. High Efficacy of {beta}-Blockers in Long-QT

Syndrome Type 1. Contribution of Noncompliance and QT-Prolonging Drugs to the Occurrence of

{beta}-Blocker Treatment "Failures" Circulation. 2009; 119(2):215-21. Epub 2008 Dec 31.

278 Postema PG, Ritsema van Eck HJ, Opthof T, van Herpen G, van Dessel PF, Priori SG, Wolpert C,

Borggrefe M, Kors JA, Wilde AA. IK1 modulates the U-wave: insights in a 100-year-old

enigma.Heart Rhythm. 2009; 6(3):393-400. Epub 2008 Nov 27

279 Dickstein K, Priori S, Auricchio A, Terradellas JB, Cleland J, Derumeaux G, Gras D, Komajda M,

Linde C, Morgan J, van Veldhuisen DJ, Gitt A, Bogale N, Fruhwald F, Strohmer B, Goethals M,

Vijgen J, Gal TB, Glikson M, McDonnald K, Keane D, Metra M, Gasparini M, Persson H, Gadler F,

Trochu JN, Gras D, Maass A, Jordaens L, Larsen AI, Faerestrand S, Brunner-La Rocca HP, Osswald

S, Kindermann M, Stellbrink C, Delgado J, Mont L, Squire I, Morgan J. European cardiac

resynchronization therapy survey: rationale and design. Eur J Heart Fail. 2009; 11(3):326-30.

280 Ruan Y, Liu N, Priori SG. Sodium channel mutations and arrhythmias. Nat Rev Cardiol. 2009;

6(5):337-48


281 Dickstein K, Cohen-Solal A, Filippatos G, McMurray JJ, Ponikowski P, Poole-Wilson PA, Strömberg

A, van Veldhuisen DJ, Atar D, Hoes AW, Keren A, Mebazaa A, Nieminen M, Priori SG, Vahanian

A, Camm J, De Caterina R, Dean V, Funck-Brentano C, Hellemans I, Kristensen SD, McGregor K,

Sechtem U, Silber S, Tendera M, Widimsky P, Zamorano JL, Auricchio A, Bax J, Böhm M, Corrà U,

della Bella P, Elliott PM, Follath F, Gheorghiade M, Hasin Y, Hernborg A, Jaarsma T, Komajda M,

Kornowski R, Piepoli M, Prendergast B, Tavazzi L, Vachiery JL, Verheugt FW, Zannad F; Task Force

per la Diagnosi e il Trattamento dello Scompenso Cardiaco Acuto e Cronico 2008 della Societá

Europea di Cardiologia. Guidelines for the diagnosis and treatment of acute and chronic heart failure

2008. G Ital Cardiol (Rome). 2009; 10(3):141-98; discussion 199-202. Italian

282 Catalano O, Antonaci S, Moro G, Mussida M, Frascaroli M, Baldi M, Cobelli F, Baiardi P, Nastoli J,

Bloise R, Monteforte N, Napolitano C, Priori SG. Magnetic resonance investigations in Brugada

syndrome reveal unexpectedly high rate of structural abnormalities. Eur Heart J. 2009; 30(18):2241-

8. Epub 2009 Jun 26

283 Spazzolini C, Mullally J, Moss AJ, Schwartz PJ, McNitt S, Ouellet G, Fugate T, Goldenberg I, Jons

C, Zareba W, Robinson JL, Ackerman MJ, Benhorin J, Crotti L, Kaufman ES, Locati EH, Qi M,

Napolitano C, Priori SG, Towbin JA, Vincent GM. Clinical implications for patients with long QT

syndrome who experience a cardiac event during infancy. J Am Coll Cardiol. 2009; 54(9):832-7

284 Cerrone M, Priori SG. Routine electrocardiogram and medical history in syncope: a simple approach

can identify most high-risk patients. Europace. 2009; 11(11):1411-2

285 Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, Priori SG, Tan HL, Hiraoka

M, Brugada J, Wilde AA. Drugs and Brugada syndrome patients: review of the literature,

recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm. 2009;

6(9):1335-41. Epub 2009 Jul 8

286 Dickstein K, Bogale N, Priori S, Auricchio A, Cleland JG, Gitt A, Limbourg T, Linde C, van

Veldhuisen DJ, Brugada J; on behalf of the Scientific Committee and National Coordinators. The

European cardiac resynchronization therapy survey. Eur Heart J. 2009; 30(20):2450-60. Epub 2009

Aug 31

287 Ruan Y, Liu N, Napolitano C, Priori SG. Therapeutic strategies for long-QT syndrome: does the

molecular substrate matter? Circ Arrhythm Electrophysiol. 2009; 1(4):290-7

288 Bai R, Napolitano C, Bloise R, Monteforte N, Priori SG. Yield of Genetic Screening in Inherited

Cardiac Channelopathies. Circ Arrhythm Electrophysiol. 2009; 2(1):6-15. Epub 2009 Feb 10.

289 Priori SG. Arrhythmias: Unexplained sudden cardiac death--back to clinical evaluation. Nat Rev

Cardiol. 2009; 6(11):678-9

290 Cerrone M, Napolitano C, Priori SG. Catecholaminergic polymorphic ventricular tachycardia: A

paradigm to understand mechanisms of arrhythmias associated to impaired Ca(2+) regulation. Heart

Rhythm. 2009; 6(11):1652-9. Epub 2009 Jun 30.

291 Van Veldhuisen DJ, Maass AH, Priori SG, Stolt P, van Gelder IC, Dickstein K, Swedberg K.

Implementation of device therapy (cardiac resynchronization therapy and implantable cardioverter


defibrillator) for patients with heart failure in Europe: changes from 2004 to 2008. Eur J Heart Fail.

2009; 11(12):1143-51. Epub 2009 Nov 1.

292 Fowler SJ, Napolitano C, Priori SG. The genetics of cardiomyopathy: genotyping and genetic

counseling. Curr Treat Options Cardiovasc Med. 2009; 11(6):433-46

293 Sedej S, Heinzel FR, Walther S, Dybkova N, Wakula P, Groborz J, Gronau P, Maier LS, Vos MA,

Anthony Lai F, Napolitano C, Priori SG, Kockskämper J, Pieske B. Na+-dependent SR Ca2+

overload induces arrhythmogenic events in mouse cardiomyocytes with a human CPVT mutation.

Cardiovasc Res. 2010 Jul 1;87(1):50-9. doi: 10.1093/cvr/cvq007. Epub 2010 Jan 15

294 Napolitano C, Priori SG, Bloise R. Catecholaminergic Polymorphic Ventricular Tachycardia. In:

Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA):

University of Washington, Seattle; 1993-.

2004[updated 2009 Jul 7].

295 Splawski I, Timothy KW, Priori SG, Napolitano C, Bloise R. Timothy Syndrome. In: Pagon RA,

Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of

Washington, Seattle; 1993-.2006 [updated 2009 Aug 20].

296 Ruan Y, Denegri M, Liu N, Bachetti T, Seregni M, Morotti S, Severi S, Napolitano C, Priori SG.

Trafficking defects and gating abnormalities of a novel SCN5A mutation question gene-specific

therapy in long QT syndrome type 3. Circ Res. 2010; 106(8):1374-83. Epub 2010 Mar 25

297 Fowler SJ, Cerrone M, Napolitano C, Priori SG. Genetic testing for inherited cardiac arrhythmias.

Hellenic J Cardiol. 2010; 51(2):92-103

298 Arcelli D, Farina A, Cappuzzello C, Bresin A, De Sanctis P, Perolo A, Prandstraller D, Valentini D,

Zucchini C, Priori S, Rizzo N. Identification of circulating placental mRNA in maternal blood of

pregnancies affected with fetal congenital heart diseases at the second trimester of pregnancy:

implications for early molecular screening. Prenat Diagn. 2010; 30(3):229-34

299 Fowler SJ, Napolitano C, Priori SG. When is genetic testing useful in patients suspected to have

inherited cardiac arrhythmias?. Curr Opin Cardiol. 2010; 25(1):37-45

300 Thomas SH, Drici MD, Hall GC, Crocq MA, Everitt B, Lader MH, Jeunne CL, Naber D, Priori S,

Sturkenboom M, Thibaut F, Peuskens J, Mittoux A, Tanghøj P, Toumi M, Moore ND, Mann RD.

Safety of sertindole versus risperidone in schizophrenia: principal results of the sertindole cohort

prospective study (SCoP). Acta Psychiatr Scand. 2010 Nov;122(5):345-55. doi: 10.1111/j.1600-

0447.2010.01563.x.

301 Herron TJ, Milstein ML, Anumonwo J, Priori SG, Jalife J. Purkinje cell calcium dysregulation is the

cellular mechanism that underlies catecholaminergic polymorphic ventricular tachycardia. Heart

Rhythm. 2010; 7(8):1122-8. Epub 2010 Jun 9

302 Tomás M, Napolitano C, De Giuli L, Bloise R, Subirana I, Malovini A, Bellazzi R, Arking DE,

Marban E, Chakravarti A, Spooner PM, Priori SG. Polymorphisms in the NOS1AP gene modulate

QT interval duration and risk of arrhythmias in the long QT syndrome. J Am Coll Cardiol. 2010;

55(24):2745-52


303 Kang G, Giovannone SF, Liu N, Liu FY, Zhang J, Priori SG, Fishman GI. Purkinje cells from RyR2

mutant mice are highly arrhythmogenic but responsive to targeted therapy. Circ Res. 2010;

107(4):512-9. Epub 2010 Jul 1

304 Priori SG. The fifteen years of discoveries that shaped molecular electrophysiology: time for

appraisal. Circ Res. 2010; 107(4):451-6.

305 Authors/Task Force Members, Dickstein K, Vardas PE, Auricchio A, Daubert JC, Linde C, McMurray

J, Ponikowski P, Priori SG, Sutton R, van Veldhuisen DJ; ESC Committee for Practice Guidelines

(CPG), Vahanian A, Auricchio A, Bax J, Ceconi C, Dean V, Filippatos G, Funck-Brentano C, Hobbs

R, Kearney P, McDonagh T, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P,

Widimsky P; Document Reviewers, Tendera M, Anker SD, Blanc JJ, Gasparini M, Hoes AW, Israel

CW, Kalarus Z, Merkely B, Swedberg K, Camm AJ. 2010 Focused Update of ESC guidelines on

device therapy in heart failure: An update of the 2008 ESC guidelines for the diagnosis and treatment

of acute and chronic heart failure and the 2007 ESC guidelines for cardiac and resynchronization

therapy Developed with the special contribution of the Heart Failure Association and the European

Heart Rhythm Association.Eur Heart J. 2010 (21):2677-87.

306 Schwartz PJ, Spazzolini C, Priori SG, Crotti L, Vicentini A, Landolina M, Gasparini M, Wilde AA,

Knops RE, Denjoy I, Toivonen L, Mönnig G, Al-Fayyadh M, Jordaens L, Borggrefe M, Holmgren C,

Brugada P, De Roy L, Hohnloser SH, Brink PA. Who are the long-QT syndrome patients who receive

an implantable cardioverter-defibrillator and what happens to them? Data From the European Long-

QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry. Circ. Res. 2010;

122(13):1272-82. Epub 2010 Sep 13

307 Crocq MA, Naber D, Lader MH, Thibaut F, Drici M, Everitt B, Hall GC, Le Jeunne C, Mittoux A,

Peuskens J, Priori S, Sturkenboom M, Thomas SH, Tanghøj P, Toumi M, Mann R, Moore ND.

Suicide attempts in a prospective cohort of patients with schizophrenia treated with sertindole or

risperidone. Eur Neuropsychopharmacol. 2010; 20(12):829-38

308 Dickstein K, Vardas PE, Auricchio A, Daubert JC, Linde C, McMurray J, Ponikowski P, Priori SG,

Sutton R, van Veldhuisen DJ. 2010 focused update of ESC Guidelines on device therapy in heart

failure: an update of the 2008 ESC Guidelines for the diagnosis and treatment of acute and chronic

heart failure and the 2007 ESC Guidelines for cardiac and resynchronization therapy. Developed with

the special contribution of the Heart Failure Association and the European Heart Rhythm Association.

Eur J Heart Fail. 2010; 12(11):1143-53

309 Kashimura T, Briston SJ, Trafford AW, Napolitano C, Priori SG, Eisner DA, Venetucci LA. In the

RyR2R4496C Mouse Model of CPVT, {beta}-Adrenergic Stimulation Induces Ca Waves by

Increasing SR Ca Content and Not by Decreasing the Threshold for Ca Waves. Circ Res. 2010.

107(12):1483-9

310 Dickstein K, Vardas PE, Auricchio A, Daubert JC, Linde C, McMurray J, Ponikowski P, Priori SG,

Sutton R, van Veldhuisen DJ; ESC Committee for Practice Guidelines, Vahanian A, Auricchio A, Bax

J, Ceconi C, Dean V, Filippatos G, Funck-Brentano C, Hobbs R, Kearney P, McDonagh T, Popescu


BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P, Widimsky P, Tendera M, Anker SD,

Blanc JJ, Gasparini M, Hoes AW, Israel CW, Kalarus Z, Merkely B, Swedberg K, Camm AJ.2010

Focused Update of ESC Guidelines on device therapy in heart failure: an update of the 2008 ESC

Guidelines for the diagnosis and treatment of acute and chronic heart failure and the 2007 ESC

Guidelines for cardiac and resynchronization therapy. Developed with the special contribution of the

Heart Failure Association and the European Heart Rhythm Association. Europace. 2010; 12(11):1526-

36.

311 Sedej S, Heinzel FR, Walther S, Dybkova N, Wakula P, Groborz J, Gronau P, Maier LS, Vos MA,

Lai FA, Napolitano C, Priori SG, Kockskämper J, Pieske B. Na+-dependent SR Ca2+ overload

induces arrhythmogenic events in mouse cardiomyocytes with a human CPVT mutation. Cardiovasc

Res. 2010; 87(1):50-9. Epub 2010 Jan 15

312 Goldenberg I, Bradley J, Moss A, McNitt S, Polonsky S, Robinson JL, Andrews M, Zareba W;

International LQTS Registry Investigators. Beta-blocker efficacy in high-risk patients with the

congenital long-QT syndrome types 1 and 2: implications for patient management J Cardiovasc

Electrophysiol. 2010; 21(8):893-901. Epub 2010 Mar 5

313 Huang H, Priori SG, Napolitano C, O'Leary ME, Chahine M. Y1767C, a novel SCN5A mutation

induces a persistent sodium current and potentiates ranolazine inhibition of Nav1.5 channels. Am J

Physiol Heart Circ Physiol. 2011. 300(1):H288-99. Epub 2010 Nov,12

314 Liu N, Ruan Y, Denegri M, Bachetti T, Li Y, Colombi B, Napolitano C, Coetzee WA, Priori SG.

Calmodulin kinase II inhibition prevents arrhythmias in RyR2(R4496C+/-) mice with

catecholaminergic polymorphic ventricular tachycardia. J Mol Cell Cardiol. 2011. 50(1):214-22. Epub

2010 Oct 23

315 Goldenberg I, Horr S, Moss AJ, Lopes CM, Barsheshet A, McNitt S, Zareba W, Andrews ML,

Robinson JL, Locati EH, Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Platonov PG, Priori

SG, Qi M, Schwartz PJ, Shimizu W, Towbin JA, Vincent GM, Wilde AA, Zhang L. Risk for life-

threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range

corrected QT intervals. J Am Coll Cardiol. 2011. 28;57(1):51-9

316 Dybkova N, Sedej S, Napolitano C, Neef S, Rokita AG, Hünlich M, Brown JH, Kockskämper J, Priori

SG, Pieske B, Maier LS. Overexpression of CaMKIIδc in RyR2R4496C+/- knock-in mice leads to

altered intracellular Ca2+ handling and increased mortality. J Am Coll Cardiol. 2011; 57(4):469-79

317 Liu JF, Jons C, Moss AJ, McNitt S, Peterson DR, Qi M, Zareba W, Robinson JL, Barsheshet A,

Ackerman MJ, Benhorin J, Kaufman ES, Locati EH, Napolitano C, Priori SG, Schwartz PJ, Towbin

J, Vincent M, Zhang L, Goldenberg I. Risk Factors for Recurrent Syncope and Subsequent Fatal or

Near-Fatal Events in Children and Adolescents With Long QT Syndrome.; International Long QT

Syndrome Registry. J Am Coll Cardiol. 2011; 57(8):941-50

318 Priori SG. Genetic determinants of cardiac (electric) conduction. Circ Res. 2011;108(4):402-3

319 Fuster V, Rydén LE, Cannom DS, Crijns HJ, Curtis AB, Ellenbogen KA, Halperin JL, Kay GN, Le

Huezey JY, Lowe JE, Olsson SB, Prystowsky EN, Tamargo JL, Wann LS, Smith SC Jr, Priori SG,


Estes NA 3rd, Ezekowitz MD, Jackman WM, January CT, Lowe JE, Page RL, Slotwiner DJ,

Stevenson WG, Tracy CM, Jacobs AK, Anderson JL, Albert N, Buller CE, Creager MA, Ettinger SM,

Guyton RA, Halperin JL, Hochman JS, Kushner FG, Ohman EM, Stevenson WG, Tarkington LG,

Yancy CW. 2011 ACCF/AHA/HRS focused updates incorporated into the ACC/AHA/ESC 2006

guidelines for the management of patients with atrial fibrillation: a report of the American College of

Cardiology Foundation/ American Heart Association Task Force on practice guidelines. American

College of Cardiology Foundation/American Heart Association Task Force. Circulation. 2011 Mar

15;123(10):e269-367. Epub 2011.

320 Napolitano C, Liu N, Priori SG. Role of caldmodulin Kinase in catecholaminergic polymorphic

ventricular tachycardia. Heart Rhythm. 2011 Oct;8(10):1601-5. Epub 2011 Mar 10

321 Priori SG, Chen SR. Inherited dysfunction of sarcoplasmic reticulum Ca2+handling and

arrhythmogenesis. Circ Res. 2011.108(7):871-83

322 Cerrone M, Priori SG. Genetics of sudden death: focus on inherited channelopathies. Eur Heart J.

2011 Sep;32(17):2109-18. doi: 10.1093/eurheartj/ehr082. Epub 2011 Apr 9

323 Segagni D, Ferrazzi F, Larizza C, Tibollo V, Napolitano C, Priori SG, Bellazzi R. R engine cell:

integrating R into the i2b2 software infrastructure. J Am Med Inform Assoc. 2011. 18(3):314-7. Epub

2011 Jan 24

324 Fernàndez-Velasco M, Ruiz-Hurtado G, Rueda A, Neco P, Mercado-Morales M, Delgado C,

Napolitano C, Priori SG, Richard S, Marìagòmez A, Benitah JP. RyRCa Leak Limits Cardiac Ca

Windows Current Overcoming the Tonic Effect of Calmodulin Mice. PLoS One. 2011. 6(6):e20863.

Epub 2011 Jun 6

325 Cummings S, Priori S. Genetics of cardiac arrhythmias. Minerva Med. 2011 Jun, 102(3):209-22.

326 Liu N, Denegri M, Ruan Y, Avelino-cruz JE, Perissi A, Negri s, Napolitano C, Coetzee WA, Boyden

PA, Priori SG. Short Communication:Flecainide Exerts an Antiarrhythmic Effect in a Mouse Model

of Catecholaminergic Polymorphic Ventricular Tachycardia by Increasing the Threshold for

Triggered Activity. Circ Res. 2011. Jul 22;109(3):291-5. Epub 2011 Jun 16

327 Wolpert C, Lubinski A, Bissinger A, Merkely B, Priori S, Brugada J. Barriers to implementation of

evidence-based electrical therapies and the need for outcome research: role of European registries.

Europace. 2011 May; 13 Suppl 2:ii18-20

328 Kaltman JR, Thompson PD, Lantos J, Berul CI, Botkin J, Cohen JT, Cook NR, Corrado D, Drezner

J, Frick KD, Goldman S, Hlatky M, Kannankeril PJ, Leslie L, Priori S, Saul JP, Shapiro- Mendoza

CK, Siscovick D, Vetter VL, Boineau R, Burns KM, Friedman RA. Screening for sudden cardiac

deatch in the young: report from a national heart, lung, and blood institute working group. Circulation.

2011; 123(17):1911-8.

329 Bogale N, Priori S, Gitt A, Alings M, Linde C, Dickstein K.; on behalf of the Scientific Committee,

National coordinators, and the investigators. The European cardiac resynchronization therapy survey:

patient selection and implantation practice vary according to centre volume. Europace.

2011;13(10):1445-53. doi: 10.1093/europace/eur173. Epub 2011 Jun 28


330 Traub D, Fonseka N, Priori S. ST-Segment Elevation in the Setting of a Febrile Illness. Ann

Noninvasive Electrocardiol. 2011 Jul;16(3):303-4. doi: 10.1111/j.1542-474X.2011.00445.x

331 Bogale N, Witte K, Priori S, Cleland J, Auricchio A, Gadler F, Gitt A, Limbourg T, Linde C,

Dickstein K.; on behalf of the Scientific Committee, National coordinators and the investigators. The

European Cardiac Resynchronization Therapy Survey: comparison of outcomes between de novo

cardiac resynchronization therapy implantations and upgrades. Eur J Heart Fail. 2011 Sep; 13(9):974-

83. Epub 2011 Jul 19.

332 Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob

M, Hamilton R, Hershberger RE, Judge DP, Le Marec H, McKenna WJ, Schulze-Bahr E, Semsarian

C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP. HRS/EHRA Expert Consensus Statement

on the State of Genetic Testing for the Channelopathies and Cardiomyopathies: This document was

developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm

Association (EHRA). Europace. 2011 Aug;13(8):1077-109

333 Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob

M, Hamilton R, Hershberger RE, Judge DP, Le Marec H, McKenna WJ, Schulze-Bahr E, Semsarian

C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP. HRS/EHRA Expert Consensus Statement

on the State of Genetic Testing for the Channelopathies and Cardiomyopathies This document was

developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm

Association (EHRA). Heart Rhythm. 2011 Aug;8(8):1308-39

334 Endorsed by the European Society of Gynecology (ESG), the Association for European Paediatric

Cardiology (AEPC), and the German Society for Gender Medicine (DGesGM);Authors/Task Force

Members, Regitz-Zagrosek V, Lundqvist CB, Borghi C, Cifkova R, Ferreira R, Foidart JM, Gibbs JS,

Gohlke-Baerwolf C, Gorenek B, Iung B, Kirby M, Maas AH, Morais J, Nihoyannopoulos P, Pieper

PG, Presbitero P, Roos-Hesselink JW, Schaufelberger M, Seeland U, Torracca L; ESC Committee for

Practice Guidelines (CPG), Bax J, Auricchio A, Baumgartner H, Ceconi C, Dean V, Deaton C, Fagard

R, Funck-Brentano C, Hasdai D, Hoes A, Knuuti J, Kolh P, McDonagh T, Moulin C, Poldermans D,

Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Torbicki A, Vahanian A, Windecker S; Document

Reviewers, Baumgartner H, Deaton C, Aguiar C, Al-Attar N, Garcia AA, Antoniou A, Coman I,

Elkayam U, Gomez-Sanchez MA, Gotcheva N, Hilfiker-Kleiner D, Kiss RG, Kitsiou A, Konings KT,

Lip GY, Manolis A, Mebaaza A, Mintale I, Morice MC, Mulder BJ, Pasquet A, Price S, Priori SG,

Salvador MJ, Shotan A, Silversides CK, Skouby SO, Stein JI, Tornos P, Vejlstrup N, Walker F,

Warnes C. ESC Guidelines on the management of cardiovascular diseases during pregnancy: The

Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society

of Cardiology (ESC). Eur Heart J. 2011 Dec;32(24):3147-97. doi: 10.1093/eurheartj/ehr218. Epub

2011 Aug 26

335 Segagni D, Tibollo V, Dagliati A, Perinati L, Zambelli A, Priori S, Bellazzi R. The ONCO-I2b2

Project: Integrating Biobank Information and Clinical Data to Support Translational Research in

Oncology. Stud Health Technol Inform. 2011;169:887-91


336 Priori SG. Induced Pluripotent Stem Cell Derived CArdiomyocytes and Long QT Syndrome: Is

Personalized Medicine Ready for Prime Time? Circ Res. 2011;109(8):822-4.

337 Bogale N, Priori S, Cleland JG, Brugada J, Linde C, Auricchio A, van Veldhuisen DJ, Limbourg T,

Gitt A, Gras D, Stellbrink C, Gasparini M, Metra M, Derumeaux G, Gadler F, Buga L, Dickstein K;

on behalf of the Scientific Committee, National Coordinators, and Investigators. The European CRT

Survey: 1 year (9-15 months) follow-up results. Eur J Heart Fail. 2012;14(1):61-73

338 Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone

C, Mascioli G, Rossetti G, De Nardis R, Colombo M. Risk Stratification in Brugada Syndrome Results

of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) Registry. J Am Coll

Cardiol. 2012;59(1):37-45

339 Monteforte N, Napolitano C, Priori SG. Genetics and Arrhythmias: Diagnostic and Prognostic

Applications. Rev Esp Cardiol. 2012; 65:278-286

340 Denegri M, Avelino-Cruz JE, Boncompagni S, De Simone SA, Auricchio A, Villani L, Volpe P,

Protasi F, Napolitano C, Priori SG. Viral Gene Transfer Rescues Arrhythmogenic Phenotype and

Ultrastructural Abnormalities in Adult Calsequestrin-Null Mice With Inherited Arrhythmias. Circ

Res. 2012;110:663-668

341 Cerrone M, Napolitano C, Priori SG.Genetics of ion-channel disorders. Curr Opin Cardiol.

2012;27(3):242-52. Review

342 Priori SG, Napolitano C. Meandering pathway leading from genotyping to personalized management

of long-QT syndrome. Circulation. 2012;125(16):1961-3. Epub 2012 Mar 28.

343 Napolitano C, Bloise R, Monteforte N, Priori SG. Sudden cardiac death and genetic ion

channelopathies: long QT, Brugada, short QT, catecholaminergic polymorphic ventricular

tachycardia, and idiopathic ventricular fibrillation. Circulation 2012;125(16):2027-34

344 Segagni D, Tibollo V, Dagliati A, Zambelli A, Priori SG, Bellazzi R. An ICT infrastructure to

integrate clinical and molecular data in oncology research. BMC Bioinformatics. 2012 Mar 28;13

Suppl 4:S5

345 Catalano O, Moro G, Perotti M, Frascaroli M, Ceresa M, Antonaci S, Baiardi P, Napolitano C, Baldi

M, Priori SG. Late gadolinium enhancement by cardiovascular magnetic resonance is complementary

to left ventricle ejection fraction in predicting prognosis of patients with stable coronary artery disease.

J Cardiovasc Magn Reson. 2012;14:29.

346 Neco P, Torrente AG, Mesirca P, Zorio E, Liu N, Priori SG, Napolitano C, Richard S, Benitah JP,

Mangoni ME, Gómez AM. Paradoxical effect of increased diastolic Ca(2+) release and decreased

sinoatrial node activity in a mouse model of catecholaminergic polymorphic ventricular tachycardia.

Circulation2012;126(4):392-401. Epub 2012 Jun 18

347 Venetucci L, Denegri M, Napolitano C, Priori SG. Inherited calcium channelopathies in the

pathophysiology of arrhythmias. Nat Rev Cardiol. 2012;9(10):561-75. Epub 2012 Jun 26.


348 Liu N, Napolitano C, Venetucci LA, Priori SG. Flecainide and antiarrhythmic effects in a mouse

model of catecholaminergic polymorphic ventricular tachycardia. Trends Cardiovasc Med.

2012;22(2):35-9. Epub 2012 Aug 4

349 Segagni D, Tibollo V, Dagliati A, Napolitano C, Priori S, Bellazzi R. CARDIO-i2b2: integrating

arrhythmogenic disease data in i2b2. Stud Health Technol Inform. 2012;180:1126-8

350 Mazzanti A, Priori SG. Molecular Autopsy for Sudden Unexplained Death? Time to Discuss Pros

and Cons. J Cardiovasc Electrophysiol. 2012;23(10):1099-102. doi: 10.1111/j.1540-

8167.2012.02430.x. Epub 2012 Sep 11

351 Segagni D, Ferrazzi F, Larizza C, Tibollo V, Napolitano C, Priori SG, Bellazzi R. R engine cell:

integrating R into the i2b2 software infrastructure. J Am Med Inform Assoc. 2011;18(3):314-7. Epub

2011 Jan 24

352 Cerrone M, Cummings S, Alansari T, Priori SG. A clinical approach to inherited arrhythmias. Circ

Cardiovasc Genet. 2012;5(5):581-90

353 Priori SG, Napolitano C, Di Pasquale E, Condorelli G. Induced pluripotent stem cell-derived

cardiomyocytes in studies of inherited arrhythmias. J Clin Invest. 2013 Jan 2;123(1):84-91. Epub 2013

Jan 2

354 Deo M, Ruan Y, Pandit SV, Shah K, Berenfeld O, Blaufox A, Cerrone M, Noujaim SF, Denegri M,

Jalife J, Priori SG. KCNJ2 mutation in short QT syndrome 3 results in atrial fibrillation and

ventricular proarrhythmia. Proc Natl Acad Sci U S A. 2013 Mar 12;110(11):4291-6. Epub 2013 Feb

25

355 Napolitano C, Bloise R, Memmi M, Priori SG. Clinical utility gene card for: Catecholaminergic

polymorphic ventricular tachycardia (CPVT). Eur J Hum Genet. 2013 Apr 3 doi:

10.1038/ejhg.2013.55

356 Liu N, Denegri M, Dun W, Boncompagni S, Lodola F, Protasi F, Napolitano C, Boyden P, Priori SG.

Abnormal Propagation of Calcium Waves and Ultrastructural Remodeling in Recessive

Catecholaminergic Polymorphic Ventricular Tachycardia. Circ Res. 2013 Jul 5;113(2):142-52 Epub

2013 May 14

357 Brunello L, Slabaugh JL, Radwanski PB, Ho HT, Belevych AE, Lou Q, Chen H, Napolitano C, Lodola

F, Priori SG, Fedorov VV, Volpe P, Fill M, Janssen PM, Györke S. Decreased RyR2 refractoriness

determines myocardial synchronization of aberrant Ca2+ release in a genetic model of arrhythmia.

Proc Natl Acad Sci U S A. 2013;110(25):10312-7. Epub 2013 Jun 3

358 European Society of Cardiology; Committee for Education; Authors/Task Force Members:, Gillebert

TC, Brooks N, Fontes-Carvalho R, Fras Z, Gueret P, Lopez-Sendon J, Salvador MJ, van den Brink

RB, Smiseth OA; Observer on behalf of the UEMS (Cardiology Section):, Griebenow R; Review

Coordinators:, Kearney P, Vahanian A; Contributors and reviewers:, Bauersachs J, Bax J, Burri H,

Caforio AL, Calvo F, Charron P, Ertl G, Flachskampf F, Giannuzzi P, Gibbs S, Gonçalves L,

González-Juanatey JR, Hall J, Herpin D, Iaccarino G, Iung B, Kitsiou A, Lancellotti P, McDonough

T, Monsuez JJ, Nuñez IJ, Plein S, Porta-Sánchez A, Priori S, Price S, Regitz-Zagrosek V, Reiner Z,


Ruilope LM, Schmid JP, Sirnes PA, Sousa-Ouva M, Stepinska J, Szymanski C, Taggart D, Tendera

M, Tokgözoglu L, Trindade P, Zeppenfeld K; ESC staff:, Joubert L, Carrera C. ESC Core Curriculum

for the General Cardiologist (2013). Eur Heart J. 2013;34(30):2381-411. Epub 2013 Jul 11

359 Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, Blom N, Brugada J, Chiang CE, Huikuri

H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C.

Executive Summary: HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and

Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm. 2013

Dec;10(12):e85-108. doi: 10.1016/j.hrthm.2013.07.021. Epub 2013 Jul 31


H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C;

Document Reviewers, Ackerman M, Belhassen B, Estes NA 3rd, Fatkin D, Kalman J, Kaufman E,

Kirchhof P, Schulze-Bahr E, Wolpert C, Vohra J, Refaat M, Etheridge SP, Campbell RM, Martin ET,

Quek SC. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and

management of patients with inherited primary arrhythmia syndromes. Europace. 2013

Oct;15(10):1389-406. doi: 10.1093/europace/eut272. Epub 2013 Aug 30.


H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C.

HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with

Inherited Primary Arrhythmia SyndromesExpert Consensus Statement on Inherited Primary

Arrhythmia Syndromes: Document endorsed by HRS, EHRA, and APHRS in May 2013 and by

ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10(12):1932-63. doi:

10.1016/j.hrthm.2013.05.014. Epub 2013 Aug 30

362 Di Pasquale E, Lodola F, Miragoli M, Denegri M, Avelino-Cruz JE, Buonocore M, Nakahama H,

Portararo P, Bloise R, Napolitano C, Condorelli G, Priori SG. CaMKII inhibition rectifies arrhythmic

phenotype in a patient-specific model of catecholaminergic polymorphic ventricular tachicardia. Cell

Death Dis. 2013 Oct 10;4:e843. doi: 10.1038/cddis.2013.369

363 Cerrone M, Lin X, Zhang M, Agullo-Pascual E, Pfenniger A, Chkourko Gusky H, Novelli V, Kim C,

Tirasawadischai T, Judge DP, Rothenberg E, Chen HV, Napolitano C, Priori SG, Delmar M.

Missense mutations in plakophilin-2 can cause brugada syndrome phenotype by decreasing sodium

current and nav1.5 membrane localization. Circulation. 2014 Mar 11;129(10):1092-103. doi:

10.1161/CIRCULATIONAHA.113.003077. Epub 2013 Dec 18

364 Mazzanti A, Kanthan A, Monteforte N, Memmi M, Bloise R, Novelli V, Miceli C, O'Rourke S, Borio

G, Zienciuk-Krajka A, Curcio A, Surducan AE, Colombo M, Napolitano C, Priori SG. Novel insights

in the natural history of Short QT Syndrome. J Am Coll Cardiol. 2014 Apr 8;63(13):1300-8. doi:

10.1016/j.jacc.2013.09.078. Epub 2013 Nov 28

365 Segagni D, Tibollo V, Dagliati A, Malovini A, Zambelli A, Napolitano C, Priori SG, Bellazzi R.

Clinical and research data integration: the i2b2-FSM experience. AMIA Summits Transl Sci Proc.

2013 Mar 18;2013:239-40. eCollection 2013


366 Sedej S, Schmidt A, Denegri M, Walther S, Matovina M, Arnstein G, Gutschi EM, Windhager I,

Ljubojević S, Negri S, Heinzel FR, Bisping E, Vos MA, Napolitano C, Priori SG, Kockskämper J,

Pieske B. Subclinical abnormalities in sarcoplasmic reticulum Ca2+ release promote eccentric

myocardial remodeling and pump failure death in response to pressure overload. J Am Coll Cardiol.

2014 Apr 22;63(15):1569-79. doi: 10.1016/j.jacc.2013.11.010. Epub 2013 Dec 4

367 Ambrosetti M, Temporelli PL, Faggiano P, Febo O, Diaco T, Favretto G, Calisi P, Gabriele M, Greco

C, Tavazzi L; THINKPAD investigators. Lower extremities peripheral arterial disease among patients

admitted to cardiac rehabilitation: the THINKPAD registry. Int J Cardiol. 2014 Feb 1;171(2):192-8.

doi: 10.1016/j.ijcard.2013.12.004. Epub 2013 Dec 12

368 Priori SG. Genetic testing to predict sudden cardiac death: current perspectives and future goals.

Indian Heart J. 2014 January - February;66S1:S58-S60. doi: 10.1016/j.ihj.2013.11.004. Epub 2013

Dec 22. Review

369 Mazzanti A, O'Rourke S, Ng K, Miceli C, Borio G, Curcio A, Esposito F, Napolitano C, Priori SG.

The usual suspects in sudden cardiac death of the young: a focus on inherited arrhythmogenic diseases.

Expert Rev Cardiovasc Ther. 2014 Apr;12(4):499-519. doi: 10.1586/14779072.2014.894884

370 Ambrosini E, Sicca F, Brignone MS, D'Adamo MC, Napolitano C, Servettini I, Moro F, Ruan Y,

Guglielmi L, Pieroni S, Servillo G, Lanciotti A, Valvo G, Catacuzzeno L, Franciolini F, Molinari P,

Marchese M, Grottesi A, Guerrini R, Santorelli FM, Priori S, Pessia M. Genetically induced

dysfunctions of Kir2.1 channels: implications for short QT3 syndrome and autism-epilepsy

phenotype. Hum Mol Genet. 2014 Sep 15;23(18):4875-86. doi: 10.1093/hmg/ddu201. Epub 2014 May

2

371 Denegri M, Bongianino R, Lodola F, Boncompagni S, De Giusti VC, Avelino-Cruz JE, Liu N,

Persampieri S, Curcio A, Esposito F, Pietrangelo L, Marty I, Villani L, Moyaho A, Baiardi P,

Auricchio A, Protasi F, Napolitano C, Priori SG. A Single Delivery of an Adeno-Associated Viral

Construct to Transfer the CASQ2 Gene to Knock-In Mice Affected by Catecholaminergic

Polymorphic Ventricular Tachycardia is Able to Cure the Disease from Birth to Advanced Age.

Circulation. 2014 Jun 24;129(25):2673-81. doi: 10.1161/CIRCULATIONAHA.113.006901. Epub

2014 Jun

372 Bonny A, Noah DN, Ngantcha M, Ateh R, Saka C, Wa J, Fonga R, Amougou SN, Winkel BG,

Lambiase P, Priori SG on behalf of the Douala Sudden Unexplained Death (Douala-SUD) study

group. Epidemiology of sudden cardiac death in Cameroon: Rationale and design of the Douala-SUD

survey. Arch Cardiovasc Dis. 2014;107(8-9):433-42. doi: 10.1016/j.acvd.2014.05.005. Epub 2014 Jul

19

373 Kirchhof P, Sipido KR, Cowie MR, Eschenhagen T, Fox KA, Katus H, Schroeder S, Schunkert H,

Priori S. The continuum of personalized cardiovascular medicine: a position paper of the European

Society of Cardiology. Eur Heart J. 2014 Dec 7;35(46):3250-7. doi: 10.1093/eurheartj/ehu312. Epub

2014 Aug 22


374 Authors/Task Force members, Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F,

Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J,

Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H;

Authors/Task Force members. O'Mahony C, Zamorano JL, Achenbach S, Baumgartner H, Bax JJ,

Bueno H, Dean V, Deaton C, Erol C, Fagard R, Ferrari R, Hasdai D, Hoes AW, Kirchhof P, Knuuti J,

Kolh P, Lancellotti P, Linhart A, Nihoyannopoulos P, Piepoli MF, Ponikowski P, Sirnes PA, Tamargo

JL, Tendera M, Torbicki A, Wijns W, Windecker S, Hasdai D, Ponikowski P, Achenbach S, Alfonso

F, Basso C, Cardim NM, Gimeno JR, Heymans S, Holm PJ, Keren A, Kirchhof P, Kolh P, Lionis C,

Muneretto C, Priori S, Salvador MJ, Wolpert C, Zamorano JL. 2014 ESC Guidelines on diagnosis

and management of hypertrophic cardiomyopathy: The Task Force for the Diagnosis and Management

of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J.

2014;35(39):2733-79. doi: 10.1093/eurheartj/ehu284. Epub 2014 Aug 29

375 Bonny A, Ngantcha M, Amougou SN, Kane A, Marrakchi S, Okello E, Taty G, Gehani A, Diakite M,

Talle M, Lambiase P, Houenassi M, Chin A, Otieno H, Temu G, Owusu IK, Karaye KM, Awad AA,

Winkel BG, Priori SG; Pan‐African Society of Cardiology (PASCAR) Task Force on Sudden Cardiac

Death. Rationale and design of the Pan-African Sudden Cardiac Death survey: the Pan-African SCD

study. Cardiovasc J Afr. 2014;25(4):176-84. doi: 10.5830/CVJA-2014-035

376 Sacchi L, Rubrichi S, Rognoni C, Panzarasa S, Parimbelli E, Mazzanti A, Napolitano C, Priori SG,

Quaglini S. From decision to shared-decision: Introducing patients' preferences into clinical decision

analysis. Artif Intell Med. 2014 Nov 1. pii: S0933-3657(14)00107-9. doi:

10.1016/j.artmed.2014.10.004

377 Park DS, Cerrone M, Morley G, Vasquez C, Fowler S, Liu N, Bernstein SA, Liu FY, Zhang J, Rogers

CS, Priori SG, Chinitz LA, Fishman GI. Genetically engineered SCN5A mutant pig hearts exhibit

conduction defects and arrhythmias. J Clin Invest. 2015 Jan;125(1):403-12. doi: 10.1172/JCI76919.

Epub 2014 Dec 15

378 Napolitano C, Priori SG. Role of standard resting ECG in the assessment of sudden cardiac death

riskG Ital Cardiol (Rome). 2014 Dec;15(12):670-7. doi: 10.1714/1718.18768. Review. Italian

379 Radwański PB, Brunello L, Veeraraghavan R, Ho HT, Lou Q, Makara MA, Belevych AE, Anghelescu

M, Priori SG, Volpe P, Hund TJ, Janssen PM, Mohler PJ, Bridge JH, Poelzing S, Györke S. Neuronal

Na+ channel blockade suppresses arrhythmogenic diastolic Ca2+ release. Cardiovasc Res. 2015 Apr

1;106(1):143-52. doi: 10.1093/cvr/cvu262. Epub 2014 Dec 23

380 Alper K, Bai R, Liu N, Fowler SJ, Huang XP, Priori SG, Ruan Y. hERG Blockade by Iboga Alkaloids.

Cardiovasc Toxicol. 2015 Jan 31.

381 Corona-Rivera JR, Barrios-Prieto E, Nieto-García R, Bloise R, Priori S, Napolitano C, Bobadilla-

Morales L, Corona-Rivera A, Zapata-Aldana E, Peña-Padilla C, Rivera-Vargas J, Chavana-Naranjo

E. Unusual retrospective prenatal findings in a male newborn with Timothy syndrome type 1. Eur J

Med Genet. 2015 58(6-7):332-5. doi: 10.1016/j.ejmg.2015.04.001. Epub 2015 Apr 13


382 Bongianino R, Priori SG. Gene therapy to treat cardiac arrhythmias. Nat Rev Cardiol. 2015 Apr 28.

12(9):531-46. doi: 10.1038/nrcardio.2015.61. Epub 2015 Apr 28

383 Harrell DT, Ashihara T, Ishikawa T, Tominaga I, Mazzanti A, Takahashi K, Oginosawa Y, Abe H,

Maemura K, Sumitomo N, Uno K, Takano M, Priori SG, Makita N. Genotype-dependent differences

in age of manifestation and arrhythmia complications in short QT syndrome. Int J Cardiol.

2015;190:393-402. doi: 10.1016/j.ijcard.2015.04.090. Epub 2015 Apr 15

384 Rehm HL, Berg JS, Brooks LD, Bustamante CD, Evans JP, Landrum MJ, Ledbetter DH, Maglott DR,

Martin CL, Nussbaum RL, Plon SE, Ramos EM, Sherry ST, Watson MS; ClinGen. Collaborators

(212) Andersen E, Aradhya S, Aronson S, Ashley E, Azzariti D, Babb L, Bale S, Bell G, Berg J,

Berger M, Biesecker LG, Birsoy O, Bizon C, Brandon R, Broeckel U, Brooks LD, Brothers KB,

Brudno M, Buchanan A, Burgess T, Burnside R, Bustamante C, Butte A, Caleshu C, Care M,

Carpenter ML, Carter P, Cherry J, Clark E, Coakley TR, Couch FJ, Coughlin CR 2nd, Craigen WJ,

Daily R, Dalton K, Daneshjou R, Das S, De Backer J, Denny JC, Dietz H, Dobrowolski SF, Dwight

S, Ehrlich A, Eilbeck K, Enns G, Evans JP, Faucett W, Feigenbaum A, Feng X, Feolo M, Ferber MJ,

Freimuth R, Funke BH, Ghosh R, Giovanni M, Goddard K, Goehringer S, Gollob M, Green RC,

Greenblatt M, Hammond N, Hamosh A, Harris M, Harrison S, Heale B, Hegde M, Hercher L, Herriges

J, Hershberger R, Hindorff L, Hong B, Hudgins L, Hunter JE, Ingles J, Ioannidis N, Irving S, Jackson

A, Jamal SM, Janze A, Jongbloed J, Joshi S, Kanagawa KM, Kantarci S, Kaufman D, Kearney H,

Kelly M, Khoury MJ, Kirkpatrick B, Klee E, Klein TE, Krautscheid P, Krier JB, Krotoski D, Kulkarni

S, Landrum M, Lebo M, Ledbetter D, Lee J, Lee C, Lee K, Levy HP, Liu K, Loeys B, Lyon E, Lyon

M, Madhavan S, Maglott D, Manolio T, Mao R, Martin CL, Maye U, McGarvey P, McKenna B,

McLeod HL, McManus K, Messiaen L, Milewicz D, Milko L, Miller D, Milosavljevic A, Mitkowski

J, Montgomery SB, Morales A, Muessig K, Murray MF, Nagarajan R, Nandi P, Nathanson KL, Nelson

T, Niehaus A, Norwig-Eastaugh E, Novelli V, Nussbaum R, O'Daniel J, O'Fallon B, Oasan M, Offit

K, Ormond K, Overby CL, Paithankar S, Pasquali M, Peay HL, Pineda-Alvarez D, Piper MA, Plon

SE, Priori S, Ramos EM, Rashkin M, Rehm H, Reithmaier D, Relling MV, Riggs ER, Riley G, Risheg

H, Robinson P, Roche MI, Rodriguez LL, Rubinstein W, Rychkova A, Rynearson S, Sam CM, Santani

A, Sarkar IN, Savage SA, Schloss JA, Schmitt C, Schully SD, Scott A, Scott S, Semsarian C, Serrano

MA, Shaw C, Sherry S, Sikora-Wohlfield W, Sirota M, Slavotinek A, Smith ME, Smith-Packard B,

Sneddon TP, Sobriera N, South S, Speevak M, Stavropoulos J, Steiner RD, Stosic M, Strande N,

Sturm A, Tan C, Tanaka F, Thorland E, Tinker S, Uhlmann WR, Urv T, Valle D, Van Vooren S, van

Tintelen P, Varugheese M, Vaydylevich Y, Vincent L, Wain K, Walton S, Watson M, Weaver M,

Webber E, Whirl-Carrillo M, Wilde A, Wiley K, Wilhelmsen K, Willems P, Williams MS, Wise AL,

Zellner S. ClinGen--the Clinical Genome Resource. N Engl J Med. 2015;372(23):2235-42. doi:

10.1056/NEJMsr1406261. Epub 2015 May 27

385 Sturm AC, Kline CF, Glynn P, Johnson BL, Curran J, Kilic A, Higgins RS, Binkley PF, Janssen PM,

Weiss R, Raman SV, Fowler SJ, Priori SG, Hund TJ, Carnes CA, Mohler PJ. Use of whole exome


sequencing for the identification of Ito-based arrhythmia mechanism and therapy. J Am Heart Assoc.

2015;4(5). pii: e001762. doi: 10.1161/JAHA.114.001762

386 Bezzina CR, Lahrouchi N, Priori SG. Genetics of sudden cardiac death. Circ Res.

2015;116(12):1919-36. doi: 10.1161/CIRCRESAHA.116.304030

387 Napolitano C, Novelli V, Francis MD, Priori SG. Genetic modulators of the phenotype in the long

QT syndrome: state of the art and clinical impact. Curr Opin Genet Dev. 2015;33:17-24. doi:

10.1016/j.gde.2015.06.009.

388 Priori SG, Blomstrom-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm JA, Elliott

PM, Fitzsimons D , Hatala R, Hindricks G, Kirchhof P, Kjeldsen K, Kuck KH, Hernandez Madrid

A ,Nikolaou N , Norekva TM, Spaulding C and Van Veldhuisen DJ. 2015 ESC guidelines for the

management of patients with ventricular arrhythmias and the prevention of sudden cardiac death”.

European Heart Journal 2015 Nov 1;36(41):2793-867. doi: 10.1093/eurheartj/ehv316. Epub 2015 Aug

29.

389 Authors/Task Force Members, Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe

M, Camm J, Elliott PM, Fitzsimons D, Hatala R, Hindricks G, Kirchhof P, Kjeldsen K, Kuck KH,

Hernandez-Madrid A, Nikolaou N, Norekvål TM, Spaulding C, Van Veldhuisen DJ. Europace. 2015

Nov;17(11):1601-87. doi: 10.1093/europace/euv319. Epub 2015 Aug 29.

390 Ferrantini C, Coppini R, Scellini B, Ferrara C, Pioner JM, Mazzoni L, Priori S, Cerbai E, Tesi C,

Poggesi C. R4496C RyR2 mutation impairs atrial and ventricular contractility. J Gen Physiol. 2016

Jan;147(1):39-52. doi: 10.1085/jgp.201511450. Epub 2015 Dec 14.

391 Sroubek J, Probst V, Mazzanti A, Delise P, Castro Hevia J, Ohkubo K, Zorzi A, Champagne J,

Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT,

Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori S, Lubitz SA.

Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled

Analysis. Circulation. 2016 Feb 16;133(7):622-30. doi: 10.1161/CIRCULATIONAHA.115.017885.

Epub 2016 Jan 21.

400 Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J, Elliott PM,

Fitzsimons D, Hatala R, Hindricks G, Kirchhof P, Kjeldsen K, Kuck KH, Hernandez-Madrid A,

Nikolaou N, Norekvål TM, Spaulding C, Van Veldhuisen DJ. 2015 ESC Guidelines for the

Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death.

Rev Esp Cardiol (Engl Ed). 2016 Feb;69(2):176. doi: 10.1016/j.rec.2016.01.001.

401 Spaulding C, Blomstrom Lundqvist C, Priori SG. Programmed ventricular stimulation early after

myocardial infarction: authors' reply. Europace. 2016 Feb 8. pii: euv454. [Epub ahead of print]

402 Mazzanti A, Maragna R, Faragli A, Monteforte N, Bloise R, Memmi M, Novelli V, Baiardi P,

Bagnardi V, Etheridge SP, Napolitano C, Priori SG. Gene-Specific Therapy With Mexiletine Reduces

Arrhythmic Events in Patients With Long QT Syndrome Type 3. J Am Coll Cardiol. 2016 Mar

8;67(9):1053-8. doi: 10.1016/j.jacc.2015.12.033


403 Imberti JF, Underwood K, Mazzanti A, Priori SG. Clinical Challenges in Catecholaminergic

Polymorphic Ventricular Tachycardia. Heart Lung Circ. 2016 Feb 16. pii: S1443-9506(16)00066-4.

doi: 10.1016/j.hlc.2016.01.012. [Epub ahead of print] Review

404 Priori SG, Blomstrom-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J, Elliott PM,

Fitzsimons D, Hatala R, Hindricks G, Kirchhof P, Kjeldsen K, Kuck KH, Hernandez-Madrid A,

Nikolaou N, Norekval TM, Spaulding C, Van Veldhuisen DJ. 2015 ESC Guidelines for the

management of patients with ventricular arrhythmias and the prevention of sudden cardiac Death. The

Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of

Sudden Cardiac Death of the European Society of Cardiology. G Ital Cardiol (Rome). 2016

Feb;17(2):108-70. doi: 10.1714/2174.23496

405 Curcio A, Mazzanti A, Bloise R, Monteforte N, Indolfi C, Priori SG, Napolitano C. Clinical

presentation and outcome of Brugada syndrome diagnosed with the new 2013 criteria. J Cardiovasc

Electrophysiol. 2016 Apr 21. doi: 10.1111/jce.12997. [Epub ahead of print]

406 Mosca B, Eckhardt J, Bergamelli L, Treves S, Bongianino R, De Negri M, Priori SG, Protasi F,

Zorzato F. Role of the JP45-calsequestrin complex on calcium entry in slow twitch skeletal muscles.

J Biol Chem. 2016 Sep 23;291(39):20824. doi: 10.1074/jbc.A115.709071.

407 Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JG, Coats AJ, Falk V, González-Juanatey JR,

Harjola VP, Jankowska EA, Jessup M, Linde C, Nihoyannopoulos P, Parissis JT, Pieske B, Riley JP,

Rosano GM, Ruilope LM, Ruschitzka F, Rutten FH, van der Meer P; Authors/Task Force Members;

Document Reviewers.Collaborators: Filippatos G, McMurray JJ, Aboyans V, Achenbach S, Agewall

S, Al-Attar N, Atherton JJ, Bauersachs J, John Camm A, Carerj S, Ceconi C, Coca A, Elliott P, Erol

Ç, Ezekowitz J, Fernández-Golfín C, Fitzsimons D, Guazzi M, Guenoun M, Hasenfuss G, Hindricks

G, Hoes AW, Iung B, Jaarsma T, Kirchhof P, Knuuti J, Kolh P, Konstantinides S, Lainscak M,

Lancellotti P, Lip GY, Maisano F, Mueller C, Petrie MC, Piepoli MF, Priori SG, Torbicki A, Tsutsui

H, van Veldhuisen DJ, Windecker S, Yancy C, Zamorano JL, Zamorano JL, Aboyans V, Achenbach

S, Agewall S, Badimon L, Barón-Esquivias G, Baumgartner H, Bax JJ, Bueno H, Carerj S, Dean V,

Erol Ç, Fitzsimons D, Gaemperli O, Kirchhof P, Kolh P, Lancellotti P, Lip GY, Nihoyannopoulos P,

Piepoli MF, Ponikowski P, Roffi M, Torbicki A, Vaz Carneiro A, Windecker S, Sisakian HS, Isayev

E, Kurlianskaya A, Mullens W, Tokmakova M, Agathangelou P, Melenovsky V, Wiggers H,

Hassanein M, Uuetoa T, Lommi J, Kostovska ES, Juillière Y, Aladashvili A, Luchner A, Chrysohoou

C, Nyolczas N, Thorgeirsson G, Marc Weinstein J, Di Lenarda A, Aidargaliyeva N, Bajraktari G,

Beishenkulov M, Kamzola G, Abdel-Massih T, Čelutkienė J, Noppe S, Cassar A, Vataman E, Abir-

Khalil S, van Pol P, Mo R, Straburzyńska-Migaj E, Fonseca C, Chioncel O, Shlyakhto E, Otasevic P,

Goncalvesová E, Lainscak M, Díaz Molina B, Schaufelberger M, Suter T, Yılmaz MB, Voronkov L,

Davies C. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure:

The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European

Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association

(HFA) of the ESC. Eur Heart J. 2016 May 20. pii: ehw128. [Epub ahead of print]


408 Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JG, Coats AJ, Falk V, González-Juanatey

JR, Harjola VP, Jankowska EA, Jessup M, Linde C, Nihoyannopoulos P, Parissis JT, Pieske B,

Riley JP, Rosano GM, Ruilope LM, Ruschitzka F, Rutten FH, van der Meer P, Filippatos G,

McMurray JJ, Aboyans V, Achenbach S, Agewall S, Al-Attar N, Atherton JJ, Bauersachs J, John

Camm A, Carerj S, Ceconi C, Coca A, Elliott P, Erol Ç, Ezekowitz J, Fernández-Golfín C,

Fitzsimons D, Guazzi M, Guenoun M, Hasenfuss G, Hindricks G, Hoes AW, Iung B, Jaarsma T,

Kirchhof P, Knuuti J, Kolh P, Konstantinides S, Lainscak M, Lancellotti P, Lip GY, Maisano F,

Mueller C, Petrie MC, Piepoli MF, Priori SG, Torbicki A, Tsutsui H, van Veldhuisen DJ,

Windecker S, Yancy C, Zamorano JL. 2016 ESC Guidelines for the diagnosis and treatment of

acute and chronic heart failure: The Task Force for the diagnosis and treatment of acute and chronic

heart failure of the European Society of Cardiology (ESC). Developed with the special contribution

of the Heart Failure Association (HFA) of the ESC.

Eur J Heart Fail. 2016 May 20. doi: 10.1002/ejhf.592. [Epub ahead of print]

409 Chyou JY, Friedman D, Cerrone M, Slater W, Guo Y, Taupin D, O'Rourke S, Priori SG, Devinsky

O. Electrocardiographic features of sudden unexpected death in epilepsy. Epilepsia. 2016 May 24. doi:

10.1111/epi.13411. [Epub ahead of print]

410 Faragli A, Underwood K, Priori SG, Mazzanti A. Is there a role for genetics in the prevention of

sudden cardiac death? J Cardiovasc Electrophysiol. 2016 27(9):1124-32. doi: 10.1111/jce.13028

411 Mazzanti A, Priori SG. Brugada Syndrome: The Endless Conundrum. J Am Coll Cardiol. 2016 Aug

9;68(6):624-5. doi: 10.1016/j.jacc.2016.02.087

412 Ackerman MJ, Priori SG, Dubin A, Kowey P, Linker NJ, Slotwiner D, Triedman J, Van Hare GF,

Gold MR. Beta-blocker therapy for long QT syndrome and catecholaminergic polymorphic

ventricular tachycardia: Are all beta-blockers equivalent? Heart Rhythm. 2017; 14(1):e41-e44. doi:

10.1016/j.hrthm.2016.09.012.

413 Lodola F, Morone D, Denegri M, Bongianino R, Nakahama H, Rutigliano L, Gosetti R, Rizzo G,

Vollero A, Buonocore M, Napolitano C, Condorelli G, Priori SG, Di Pasquale E. Adeno-associated

virus-mediated CASQ2 delivery rescues phenotypic alterations in a patient-specific model of

recessive catecholaminergic polymorphic ventricular tachycardia. Cell Death Dis. 2016;7(10):e2393.

doi: 10.1038/cddis.2016.304.

414 Napolitano C, Priori SG, Bloise R. Catecholaminergic Polymorphic Ventricular Tachycardia. In:

Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Ledbetter N,

Mefford HC, Smith RJH, Stephens K, editors.GeneReviews® [Internet]. Seattle (WA): University of

Washington, Seattle; 1993-2016.2004 Oct 14 [updated 2016 Oct 13]. PMID:20301466

415 Radwański PB, Ho HT, Veeraraghavan R, Brunello L, Liu B, Belevych AE, Unudurthi SD, Makara

MA, Priori SG, Volpe P, Armoundas AA, Dillmann WH, Knollmann BC, Mohler PJ, Hund TJ,

Györke S. Neuronal Na+ Channels Are Integral Components of Pro-arrhythmic Na+/Ca2+ Signaling

Nanodomain That Promotes Cardiac Arrhythmias During β-adrenergic Stimulation. JACC Basic

Transl Sci. 2016;1(4):251-266


416 Mont L, Pelliccia A, Sharma S, Biffi A, Borjesson M, Terradellas JB, Carré F, Guasch E, Heidbuchel

H, Gerche A, Lampert R, McKenna W, Papadakis M, Priori SG, Scanavacca M, Thompson P,

Sticherling C, Viskin S, Wilson M, Corrado D; Reviewers:., Lip GY, Gorenek B, Lundqvist CB,

Merkely B, Hindricks G, Hernández-Madrid A, Lane D, Boriani G, Narasimhan C, Marquez MF,

Haines D, Mackall J, Marques-Vidal PM, Corra U, Halle M, Tiberi M, Niebauer J, Piepoli M. Pre-

participation cardiovascular evaluation for athletic participants to prevent sudden death: Position

paper from the EHRA and the EACPR, branches of the ESC. Endorsed by APHRS, HRS, and

SOLAECE. Europace. 2016 Nov 4. pii: euw243. [Epub ahead of print]

417 Mont L, Pelliccia A, Sharma S, Biffi A, Borjesson M, Brugada Terradellas J, Carré F, Guasch E,

Heidbuchel H, La Gerche A, Lampert R, McKenna W, Papadakis M, Priori SG, Scanavacca M,

Thompson P, Sticherling C, Viskin S, Wilson M, Corrado D; Reviewers, Lip GY, Gorenek B,

Blomström Lundqvist C, Merkely B, Hindricks G, Hernández-Madrid A, Lane D, Boriani G,

Narasimhan C, Marquez MF, Haines D, Mackall J, Manuel Marques-Vidal P, Corra U, Halle M, Tiberi

M, Niebauer J, Piepoli M. Pre-participation cardiovascular evaluation for athletic participants to

prevent sudden death: Position paper from the EHRA and the EACPR, branches of the ESC. Endorsed

by APHRS, HRS, and SOLAECE. Eur J Prev Cardiol 2017;24(1):41-69

418 Mazzanti A, Ng K, Faragli A, Maragna R, Chiodaroli E, Orphanou N, Monteforte N, Memmi M,

Gambelli P, Novelli V, Bloise R, Catalano O, Moro G, Tibollo V, Morini M, Bellazzi R, Napolitano

C, Bagnardi V, Priori SG. Arrhythmogenic Right Ventricular Cardiomyopathy: Clinical Course and

Predictors of Arrhythmic Risk. J Am Coll Cardiol. 2016;68(23):2540-2550. doi:

10.1016/j.jacc.2016.09.951.

419 Mazzanti A, Maragna R, Napolitano C, Priori SG. Reply: Did Mutation Type Affect the Efficacy of

Mexiletine Observed in Patients With LQTS Type 3? J Am Coll Cardiol. 2017;69(2):248-249. doi:

10.1016/j.jacc.2016.09.981

420 Mazzanti A, Maragna R, Priori SG. Genetic causes of sudden cardiac death in the young. Curr Opin

Cardiol. 2017 Mar 6. doi: 10.1097/HCO.0000000000000391. [Epub ahead of print]

421 Kannankeril PJ, Moore JP, Cerrone M, Priori SG, Kertesz NJ, Ro PS, Batra AS, Kaufman ES,

Fairbrother DL, Saarel EV, Etheridge SP, Kanter RJ, Carboni MP, Dzurik MV, Fountain D, Chen H,

Ely EW, Roden DM, Knollmann BC. Efficacy of Flecainide in the Treatment of Catecholaminergic

Polymorphic Ventricular Tachycardia: A Randomized Clinical Trial. JAMA Cardiol. 2017;2(7):759-

766. doi: 10.1001/jamacardio.2017.1320

422 Mazzanti A, Underwood K, Nevelev D, Kofman S, Priori SG. The new kids on the block of

arrhythmogenic disorders: Short QT syndrome and early repolarization. J Cardiovasc Electrophysiol.

2017. doi: 10.1111/jce.13265. [Epub ahead of print]

423 Mazzanti A, Priori SG. Diagnosis of Long QT Syndrome: Time to Stand Up! Rev Esp Cardiol (Engl

Ed). 2017 Jun 8. pii: S1885-5857(17)30271-2. doi: 10.1016/j.rec.2017.05.004.

424 Bongianino R, Denegri M, Mazzanti A, Lodola F, Vollero A, Boncompagni S, Fasciano S, Rizzo G,

Mangione D, Barbaro S, Di Fonso A, Napolitano C, Auricchio A, Protasi F, Priori SG. Allele Specific


Silencing of Mutant mRNA Rescues Ultrastructural and Arrhythmic Phenotype in Mice Carriers of

the R4496C Mutation in the Ryanodine Receptor Gene (RYR2). Circ Res. 2017; 121(5):525-536. pii:

CIRCRESAHA.117.310882. doi: 10.1161/CIRCRESAHA.117.310882.

425 Vacanti G, Maragna R, Mazzanti A, Priori SG. Genetic causes of sudden cardiac death in children:

inherited arrhythmogenic diseases. Curr Opin Pediatr. 2017 J. doi: 10.1097/MOP.0000000000000537.

[Epub ahead of print]

BOOKS 1 Sudden Cardiac Death. A handbook for clinical practice. Editors: Priori SG, Zipes DP. Blackwell

publishing 2006. 1st Edition. ISBN: 1-4051-3292-2

2 Sudden Cardiac Death. A handbook for clinical practice. Editors: Priori SG, Zipes DP. Blackwell

publishing 2007. Chinese Eition. ISBN: 978-7-117-09132-9

3 Genetics of Cardiac Arrhythmias. Guest ED: Priori SG. W.B. Saunders Company a Division of

Elsevier 2010. ISSN: 1877-9182 ISBN-13:978-1-4557-03043

4 Long QT Syndrome. Guest ED: Priori SG. W.B. Saunders Company a Division of Elsevier 2012.

ISSN: 1877-9182 ISBN-13:978-1-4557-3835-9

BOOK CHAPTERS 1 Schwartz PJ, Priori SG, Zaza A and Zuanetti G: Calcio antagonisti e aritmie da riperfusione

miocardica. In: I CALCIO-ANTAGONISTI IN CARDIOLOGIA (Fazzini PF and L’Abbate A, Eds.).

OIC Medical Press, Firenze, pp 193-200, 1986

2 Zuanetti G, De Ferrari GM, Priori SG and Schwartz PJ: Protective effect of vagal hyperactivity on

reperfusion arrhythmias. In: NEURAL MECHANISMS AND CARDIOVASCULAR DISEASE.

(Lown B, Malliani A and Prosdocimi M, Eds.) Fidia Research Series 5. Liviana Press- Springer

Verlag, Berlin, pp 633-635, 1986.

3 Priori SG and Schwartz PJ: Sistema nervoso e ripolarizzazione ventricolare. In:

ELETTROCARDIOGRAFIA CENT'ANNI DOPO (Croce L, Ed.) SEI Torino, pp 47-49, 1988

4 Schwartz PJ and Priori SG: Adrenergic Arrhythmogenesis and The Long QT syndrome. In:

HANDBOOK OF EXPERIMENTAL PHARMACOLOGY. (Vaughan-Williams EM and Campbell

TJ, Eds.) Springer-Verlag, Berlin, ch. 23, pp 519-543, 1989.

5 Priori SG and Schwartz PJ: Sympathetic nervous system and malignant arrhythmias: Evidence for

further links. In: HEART BRAIN AND BRAIN HEART (Sulg I, Ed.) Springer-Verlag, Berlin, pp 98-

107, 1989

6 Priori SG and Schwartz PJ: Role of afterdepolarizations recorded in vivo in ventricular

arrhythmogenesis. In: CLINICAL ASPECTS OF VENTRICULAR REPOLARIZATION (Butrous

GS and Schwartz PJ, Eds.) Farrand Press, London, pp 289-294, 1989

7 Priori SG and Schwartz PJ: Catecholamine-Dependent Cardiac Arrhythmias: Mechanisms and

Implications. In: ADRENERGIC SYSTEM AND VENTRICULAR ARRHYTHMIAS IN


MYOCARDIAL INFARCTION. (Brachmann J, Schomig A, Eds.) Springer-Verlag, Berlin, pp 239-

247, 1989

8 Schwartz PJ and Priori SG: Sympathetic nervous system and sudden cardiac arrhythmias death. In:

CARDIAC ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE (Zipes DP and Jalife J, Eds.)

WB Saunders Co., Philadelphia, pp 330-342, 1990

9 Schwartz PJ, Locati E, Priori SG and Zaza A: The long QT syndrome. In: CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE (Zipes DP, Jalife J, Eds.) W.B. Saunders

Co., Philadelphia, pp 589-605, 1990

10 Schwartz PJ, Priori SG: Sistema nervoso autonomo e morte cardiaca improvvisa. In: MORTE

CARDIACA IMPROVVISA (Schwartz PJ, Facchini M, Eds.). Masson, Milano, pp 117-147, 1993.

11 Schwartz PJ, Priori SG, Napolitano C: Role of the autonomic nervous system in sudden death. In:

SUDDEN CARDIAC DEATH. (ME Josephson, Ed). Blackwell Scientific Publications, Inc,

Cambridge, pp 16-37, 1993

12 Priori SG, Napolitano C, Schwartz PJ: Evolving concepts in the long QT syndrome. In:

ELECTROPHARMACOLOGIC CONTROL OF CARDIAC ARRHYTHMIAS (Singh BN, Wellens

HJJ, Hiraoka M, Eds). Futura Publishing Co. Inc., Mount Kisco, pp 183-203, 1993

13 Priori SG, Diehl L, Schwartz PJ: Dispersione dell'attivazione e della ripolarizzazione nella sindrome

del QT lungo. In: LA STIMOLAZIONE CARDIACA TRANSESOFAGEA E

L'ELETTROCARDIOGRAFIA AD ALTA RISOLUZIONE 1993 (Disertori M, Marconi P, Eds.) pp

199-205, 1993

14 Priori SG, Paganini V, Schwartz PJ: La fibrillation ventriculaire idiopathique: des descriptions isolées

à l'èvaluation prospective. In: LA MORT SUBITE D'ORIGINE RYTHMIQUE (Chauvin M, Ed.)

Editions Frison-Roche, Paris, pp 109-117, 1994

15 Schwartz PJ, Locati EH, Napolitano C, Priori SG: The long QT syndrome. In: CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. II EDITION (Zipes DP and Jalife J, Eds.)


16 Priori SG, Diehl L, Schwartz PJ: Torsade de pointes. In: CARDIAC ARRHYTHMIA.

MECHANISMS, DIAGNOSIS AND MANAGEMENT (Podrid PJ, Kowey PR, Eds.) Williams &

Wilkins, Baltimore, pp 951-963, 1995

17 Priori SG on behalf of the Steering Committee of UCARE (Borggrefe M, Camm AJ, Hauer RNW,

Klein H, Kuck KH, Schwartz PJ, Touboul P, Wellens HJJ): UCARE - Unexplained Cardiac Arrest

Registry of Europe. In: TRANSVENOUS DEFIBRILLATION AND RADIOFREQUENCY

ABLATION (Camm AJ, Lindemans F, Eds.) Futura Publishing Co, Armonk, NY, pp 159-164, 1995

18 Schwartz PJ, Priori SG: Clinical utility of QT interval monitoring. In: AMBULATORY

ELECTROCARDIOGRAPHIC MONITORING (Moss AJ, Stern S, Eds.) W.B. Saunders Ltd,

London, pp 465-474, 1995

19 Schwartz PJ, Priori SG, Vanoli E, Zaza A: Influence of the autonomic nervous system on the action

of antiarrhythmic drugs. In: ANTIARRHYTHMIC DRUGS. MECHANISMS OF


ANTIARRHYTHMIC AND PROARRHYTHMIC ACTIONS (Breithardt G, Camm AJ, Borggrefe

M, Rosen MR, Eds.) Springer-Verlag, Berlin, pp 240-250, 1995

20 Priori SG and Paganini V: Idiopathic Ventricular Fibrillation: Which Prognosis and Treatment. In

CARDIAC ARRHYTHMIAS 1995. (Raviele A, Ed.) Springer, Verlag, Berlin, pp 17-22, 1995

21 Priori SG and Paganini V: Applicazioni cliniche dello studio della ripolarizzazione ventricolare: il

significato clinico dell'intervallo QT e dell'alternanza dell'onda T. In: LE TACHIARITMIE

VENTRICOLARI MALIGNE DOPO INFARTO MIOCARDICO: MECCANISMI,

STRATIFICAZIONE PROGNOSTICA E PROFILASSI. (Pedretti RFE, Della Bella P, Eds.)

Fondazione Salvatore Maugeri Edizioni - PIME Editrice, Pavia, pp 67-75, 1995

22 Priori SG and Paganini V: La fibrillazione ventricolare idiopatica: tre anni di esperienza UCARE In

CARDIOLOGIA PER IMMAGINI: Atti del Congresso Nazionale, (Gambelli G, Ed.) AITEF Ed.

Roma, pp 28-34, 1996

23 Priori SG. Idiopatic ventricular fibrillation. In: PROGRESS IN CLINICAL PACING 1998. (Santini

M, Ed.). Futura Media Services Inc. Armonk, NY, pp 325-329, 1998

24 Schwartz PJ, Napolitano C, Priori SG: The long QT syndrome. In: THE McGRAW-HILL

CLINICAL MEDICINE SERIES: CARDIOLOGY (Bayes de Luna A, Ed.). McGraw Hill Italia SpA,

Milan, pp 249-254, 1999

25 Priori SG, Napolitano C. Genetica e Aritmie. In: CLINICA E TERAPIA DELLE ARITMIE. Atti del

VII convegno Internazionale. Bologna 26-27, Marzo 1999. (Bracchetti D, Ed.). Litografia Due Torri,

Bologna, pp 119-131, 1999

26 Priori SG, Lucca E: Long QT Syndrome: Molecular Bases. In: ELECTROCARDIOLOGY.

Proceedings of the XXV International Congress on Electrocardiology (Istvàn Prèda, Ed.), World

Scientific Publishing, London, pp 361-365, 1999

27 Priori SG, Bloise R: Long QT Syndrome: Diagnosis and management. In:

ELECTROCARDIOLOGY. Proceedings of the XXV International Congress on Electrocardiology

(Istvàn Prèda, Ed.), World Scientific Publishing, London, pp 366-370, 1999

28 Priori SG, Crotti L: Molecular basis of atrial fibrillation. In: PROCEEDINGS OF THE

INTERNATIONAL MEETING ON ATRIAL FIBRILLATION. Ed. CEPI, Bologna 16-17 Settembre

1999. Tipolitografia di Fernando Begliomini, Roma, pp 73-77, 1999

29 Priori SG, Crotti L: Brugada and Long QT Syndrome are two different diseases: True or False? In:

CARDIAC ARRHYTHMIAS 1999 (Raviele A, Ed.) Springer-Verlag, Milano, Vol.1, pp 291-298,

1999

30 Priori SG, Napolitano C: Principal component analysis of Ventricular Repolarization: can it

overcome the need to measure the QT Interval? In: CARDIAC ARRHYTHMIAS 1999, (Raviele A,

Ed.), Springer-Verlag, Milano, Vol.2, pp 38-46, 1999

31 Priori SG, Crotti L: QT Lungo: cosa c’è di nuovo? In: CARDIOLOGIA PER IMMAGINI. Atti del

Convegno Nazionale, Roma 20-22 Ottobre 1999, a cura di G. Gambelli, Roma, pp 43-50, 1999


32 Schwartz PJ, Priori SG, Napolitano C: The long QT syndrome. In: CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. II EDITION (Zipes DP and Jalife J, Eds.)


33 Napolitano C, Priori SG, Schwartz PJ. Spatial Dispersion of Ventricular Repolarization in the Long

QT Syndrome. In: DISPERSION OF VENTRICULAR REPOLARIZATION: STATE OF THE ART.

(Olsson SB, Yuan S and Amlie JP, Eds). Futura Publishing Company, Inc.-Armonk NY, pp 199-207,

2000

34 Priori SG, Crotti L. Idiopathic Ventricular Fibrillation. In FIGHTING SUDDEN CARDIAC

DEATH: A WORLDWIDE CHALLENGE. (Aliot E, Clementy J, Prystowsky EN, Eds). Futura

Publishing Company, Armonk, NY, pp 239-247, 2000

35 Napolitano C, Lucca E, Priori SG. Evaluation of QT dispersion in the clinical practice: a critical

review. In PROGRESS AND CLINICAL PACING 2000 (Santini M Ed.) CEPI, Roma, pp.139-147,

2000

36 Priori SG, Grillo M. Brugada Syndrome: when to use ICD therapy? In PROGRESS AND CLINICAL

PACING 2000 (Santini M Ed.) CEPI, Roma, pp.319-324, 2000

37 Priori SG, Napolitano C. Genetic and Molecular Basis of arrhythmias. In 2000

ELECTROCARDIOLOGY. Proceedings of the XXVII International Congress on Electrocardiology.

(De Ambroggi L Ed.) CESI, Roma, pp. 11-17, 2001

38 Naccarella F, Lepera G, Iachetti F, Sdringola Maranga S, Gatti M, Priori SG. QT/QU duration and

QT/QU dispersion evaluated by 12-Leads ECG monitoring in Amiodarone induced TdP. In 2000

ELECTROCARDIOLOGY. Proceedings of the XXVII International Congress on Electrocardiology.

(De Ambroggi L Ed.) CESI, Roma, pp. 321-325, 2001

39 Naccarella F, Lepera G, Gatti M, Coluccini M, Sdringola Maranga S, Priori SG. Torsades de Pointes

Induced by Amiodarone. Clinical, 12-Leads Holter monitoring evaluation and genetic

characterization. In 2000 ELECTROCARDIOLOGY. Proceedings of the XXVII International

Congress on Electrocardiology. (De Ambroggi L Ed.) CESI, Roma, pp. 327-330, 2001

40 Priori SG, Napolitano C. Genetics of Arrhythmogenic Disorders. In CARDIAC ARRHYTHMIA.

MECHANISMS, DIAGNOSIS & MANAGEMENT. (Podrid PJ – Kowey PR Eds.) Second

Edition.Lippincott Williams & Wilkins, Philadelphia, pp.81-107,2001

41 Schwartz PJ, Priori SG. Sindrome del QT Lungo e Sindrome di Brugada. In: TRATTATO DI

CARDIOLOGIA, (Santini M-Vergara G Ed) Excerpta Medica, Milano, pp.2657-2672, 2001

42 Napolitano C, Priori SG. Catecholaminergic Polymorphic Ventricular Tachycardia: Another

Inherited Arrhythmia? In: CARDIAC ARRHYTHMIAS 2001, (Raviele A, Ed.), Springer-Verlag,

Milano, pp 187-190, 2001

43 Napolitano C, Priori SG. Will the Spectrum of Ion Channel Diseases Increase Further in the Near

Future? In: CARDIAC ARRHYTHMIAS 2001, (Raviele A, Ed.), Springer-Verlag, Milano, pp 212-

218, 2001


44 Priori SG, Napolitano C, Memmi M, Ronchetti E. Cardiac Ion Channel Diseases: Clinical Impact. In

ATRIAL FIBRILLATION AND VENTRICULAR TACHYARRHYTHMIA S. (Del Bufalo A. Ed)

Locarno, pp.104-115, 2001

45 Schwartz PJ, Priori SG. Newer approaches to management of the long QT Syndrome. In

HARRISON’S ADVANCES IN CARDIOLOGY. (Braunwald E. Ed). McGraw Hill, New York,

pp.393-398, 2002

46 Napolitano C, Priori SG. The Long QT Syndrome. In CONTEMPORARY CARDIOLOGY:

CARDIAC REPOLARIZATION: BRIDGING BASIC AND CLINICAL SCIENCE. (I. Gussak et al.

Ed.) Humana Press Inc. Totowa, NJ, pp.169-185, 2002

47 Priori SG, Napolitano C, Grillo M. Morte Improvvisa: dimensioni del problema, cause e meccanismi.

In CARDIOLOGIA 2003. Dir. S. Kulgmann. Milano, pp.346-354, 2003

48 Priori SG, Bloise R. Test diagnostici in cardiologia. In TEST DIAGNOSTICI IN CARDIOLOGIA.

(E.H. Locati, Stramba Badiale M Ed.) Grafica F.lli Vaghi, Cesano Maderno, pp.281-290, 2003.

49 Priori SG, Rivolta R, Napolitano C. Genetic of Long QT, Brugada and Other Channelopathies. In

CARDIAC ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP and Jalife J, Eds.)


50 Napolitano C, Priori SG. Catecholaminergic Polymorphic Ventricular Tachycardia and Short-

coupled Torsades de Pointes. In CARDIAC ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE.

(Zipes DP and Jalife J, Eds.) WB Saunders Co., Philadelphia, pp 633-639, 2004

51 Schwartz PJ, Priori SG. Long QT Syndrome: Genotype-Phenotype correlations. In CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP and Jalife J, Eds.) WB Saunders

Co., Philadelphia, pp 651-659, 2004

52 Priori SG, Napolitano C, Schwartz PJ. Genetics of Cardiac Arrhythmias. In HEART DISEASE. A

TEXTBOOK OF CARDIOVASCULAR MEDICINE. (Zipes DP, Libby P, Bonow RO, Braunwald E

Eds.) Elsevier, Philadelphia, pp 689-695, 2004

53 Napolitano C, Priori SG. Long QT Syndrome. In THE ENCYCLOPEDIA OF MEDICAL

GENOMICS AND PROTEINOMICS. M Dekker Ed. New York; pp 740-744, 2004

54 Priori SG, Napolitano C, Cerrone M. Experimental Therapy of genetic arrhythmias: disease-specific

pharmacology. In HANDBOOK OF EXPERIMENTAL PHARMACOLOGY-BASIS AND

TREATMENT OF CARDIAC ARRHYTHMIAS, vol 171; Springer Verlag, Berlin-Heilderberg, pp

268-286,2005

55 Priori SG, Antzelevitch C. Inherited arrhythmogenic diseases. In SUDDEN CARDIAC DEATH- A

HANDBOOK FOR CLINICAL PRACTICE. (Priori SG, Zipes DP Eds) Blackwell Publishing,

Malden, Massachusetts, pp 132-146, 2005

56 Priori SG, Napolitano C, Humphries S, Di Giglio MG, Mario B. Genetics of Cardiovascular Diseases.

In the ESC TEXTBOOK OF CARDIOVASCULAR MEDICINE. (Camm A.J., Lüscher TF and

Serruys PN, Eds.) Blackwell Publishing, Malden, Massachusetts, pp 189-218, 2006


57 Priori SG, Napolitano C. Inherited arrhythmia: present and future perspectives for genetic therapy.

In SUDDEN CARDIAC DEATH. (Ed. Capucci A.) Informa Healthcare, Abingdon, Oxon, United

Kingdom, pp 161-176, 2007

58 Priori SG, Napolitano C Genetic of Inherited Arrhythmias CARDIOVASCULAR

THERAPEUTICS- A Companion to Braunwald's Heart Disease- Antman E.M. ED.- (III Edition)

Saunders-Elsevier, Philadelphia, USA, pp.502-513

59 Kane L.A, Priori SG, Napolitano G, Arking DE, Van Eyk JE. Genetics, genomics and proteomics in

sudden cardiac death CARDIAC ARREST.The Science and Practice of Resucitation Medicine.

(Paradis NA, Halperin HR, Kern KB, Wenzel V, Chamberlain DA ED) Cambridge Medicine,

Cambridge, UK, pp. 70-89

60 Priori SG, Napolitano C, Schwartz PJ. Genetics of Cardiac Arrhythmias. In BRAUNWALD’S

HEART DISEASES. A TEXTBOOK OF CARDIOVASCULAR MEDICINE. (Libby P, Bonow RO,

Mann DL & Zipes DP Eds) VIII Edition Saunders-Elsevier, Philadelphia, USA, pp.101-109, 2008

61 Ruan Y, Bloise R, Napolitano C, Priori SG. L-Type Calcium Channel disease. In: I Gussak, C

Antzelevitch, ELECTRICAL DISEASES OF THE HEART: Springer, London UK, p 187-193, 2008

62 Liu N, Napolitano C, Priori SG. Catecholaminergic Polymorphic Ventricular Tachycardia. In: I

Gussak, C Antzelevitch, ELECTRICAL DISEASES OF THE HEART: Springer, London UK, p 536-

544, 2008

63 Liu N, Napolitano C, Priori SG. Inheritable Diseases of Intracellular Calcium Regulation. In

CARDIAC ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP, Jalife J Eds) V

Edition. Saunders-Elsevier, Philadelphia, USA, pp.535-545, 2009

64 Napolitano C, Ruan Y, Priori SG. Catecholaminergic Polymorphic Ventricular Tachycardia. In

CARDIAC ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP, Jalife J Eds) V

Edition. Saunders-Elsevier, Philadelphia, USA, pp.745-751, 2009

65 Priori SG, Splawski I, Bloise R, Napolitano C. Timothy Syndrome. In CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP, Jalife J Eds) V Edition.

Saunders-Elsevier, Philadelphia, USA, pp.757-761, 2009

66 Priori SG, Cerrone M. La Sindrome di Brugada. In TRATTATO ITALIANO DI

ELETTROFISIOLOGIA ED ELETTROSTIMOLAZIONE CARDIACA, Volume 3. Centro

Scientifico Editore, Coordinatore Generale Massimo Santini, Torino, pp. 1086-1096, 2009

67 Napolitano C., Bloise R., Priori SG. Tachicardia Ventricolare Polimorfa Catecolaminergica. In

TRATTATO ITALIANO DI ELETTROFISIOLOGIA ED ELETTROSTIMOLAZIONE

CARDIACA Volume 3. Centro Scientifico Editore, Coordinatore Generale Massimo Santini, Torino,

pp. 1111-1116, 2009

68 Cerrone M, Yaghoubian S, Priori SG. Long QT syndrome and catecholaminergic VT. A wise and

sensitive approach to the patient and family. In A PRACTICAL APPROACH TO CLINICAL

ARRHYTHMOLOGY. (MONT L., BRUGADA J.) I EDITION. Marge Medica Books, Valencia, pp.

191-200, 2010


69 Priori SG, Napolitano C. Catecholaminergic Polymorphic Ventricular Tachycardia. In CARDIAC

ELECTROPHYSIOLOGY. FROM CELL TO BEDSIDE. (Zipes DP, Jalife J Eds) VI Edition.

Saunders-Elsevier, Philadelphia, USA, pp.521-528, 2013

70 Ruan F, Liu N, Bai R, Priori SG, Napolitano C. Congenital Long QT Syndrome Type 3. In CARDIAC

SODIUM CHANNEL DISORDERS. (H. ABRIEL Ed). Elsevier, Philadelphia, USA, pp.705-713,

2014

71 Priori SG, Ruan Y, O'Rourke S, Liu N, Napolitano C. Sodium current disorders: Geneticist's view.

In CARDIAC SODIUM CHANNEL DISORDERS. (H. ABRIEL Ed). Elsevier, Philadelphia, USA,

pp.825-833, 2014

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