UOC Cardiochirurgia Pediatrica e Cardiopatie CongeniteUniversit degli Studi di Padova

INCONTRO CON LA CARDIOCHIRURGIA PEDIATRICA DI PADOVA:

STORIE DA RACCONTARE E DA CONOSCERE

Introduzione storica: la Cardiochirurgia di Padova

Prof. Giovanni Stellin

University of Padua - Il BoUniversity of Padua - Il Bo

Il Bo - The Hall of the Forty and the Galileo Galileis podiumIl Bo - The Hall of the Forty and the Galileo Galileis podium

The tower of the

Astronomical Observatory

La Specola

The tower of the

Astronomical Observatory

La Specola

Il Bo

The Anatomy Theatre of

Girolamo Fabrici dAcquapedente

Il Bo

The Anatomy Theatre of

Girolamo Fabrici dAcquapedente

Portrait of

William Harvey

Portrait of

William Harvey

Exercitazio Anatomico de Motus

Cordis et Sanguinis in Animalibus

Carlo A. Carlon MDThe Journal of International College of

SurgeonsJuly. 1951

Pier Giuseppe Cevese and his pupils

November 14, 1985:Vincenzo Gallucci performs the first cardiac

transplant in Italy

Aldo R. Castaneda

William I. Norwood

Richard and Stella Van Praagh

Milestones in the History of Cardiac Surgery of

Congenital Heart Disease at the University of Padua

January 1964 Closure of Interatrial Defect

December 1968 Correction of Tetralogy of Fallot

November 1985 Heart transplant in Italy

September 1988 First Norwood operation in Europe

May 1989 First neonatal Htx in Italy

UOC Cardiochirurgia Pediatrica e Cardiopatie CongeniteUniversit degli Studi di Padova

INCONTRO CON LA CARDIOCHIRURGIA PEDIATRICA DI PADOVA:

STORIE DA RACCONTARE E DA CONOSCERE

Presentazione attivit UOC Cardiochirurgia Pediatrica e Cardiopatie Congenite

Correzione chirurgica precoce delle cardiopatie congenite (CAVC, TGA, TOF)

Prof. Giovanni Stellin

Il Centro Cardiopatie Congenite e Pediatriche di Padova

il Centro di Riferimento Regionale per il trattamento

di pazienti affetti da cardiopatie congenite

LA NOSTRA ATTIVITA

correzione chirurgica di TUTTE LE CARDIOPATIE

CONGENITE (presenti alla nascita) nel bambino e

nelladulto con prevalenza di interventi in et

neonatale o nei primi mesi di vita (correzione precoce).

I NOSTRI RISULTATI

circa 300 procedure allanno

Negli ultimi anni il rischio chirurgico sceso per

assestarsi attorno al 3% con risultati paragonabili ai

migliori centri in Europa e del Nord America.

ECCELLENZE

Correzione chirurgica precoce delle cardiopatie congenite

Chirurgia Mini-invasiva Il Cardiopatico congenito adulto (ACHD) Progetto Europei ARISE ed ESPOIR

Correzione del vizio anatomico

Eliminazione del sovraccarico cronico e/o

della cianosi cronica

Preserva il cuore ed altri organi ed

apparati

Garantisce il miglior risultato funzionale a

lungo termine

Vantaggi

La correzione precoce

Myocardial potential

Prenatal Diagnosis and surgical outcome: a teamwork

Mechanism of myocardial cell

hyperplasia, together with

myocites hypertrophy which

is retained for a few weeks

after birth, it is well

recognised .

Team-work

Prenatal Diagnosis and surgical outcome: a teamwork

CHDs heterogeneous and often complex

Team-work of experts is needed with the aim of :

Best medical and surgical planning

Best counseling with the parents

Team-work

Prenatal Diagnosis and surgical outcome: a teamwork

DIAGNOSIS (or

suspected)Pediatric Cardiologist

Couseling

Pediatric Cardiac Surgeon

Third level center Therapeutic Plan

Clinical history

Circa il 12% delle cardiopatie congenite diagnosticate alla nascita

Aorta nasce da VDx e arteria polmonare da VSx.

Raramente anomalie extracardiache associate

Alla nascita, cianosi ingravescente, polipnea, scompenso cardiaco

TRASPOSIZIONE DELLE GRANDI ARTERIEDIV

Circa il 12% delle cardiopatie congenite diagnosticate alla nascita

Aorta nasce da VDx e arteria polmonare da VSx.

Raramente anomalie extracardiache associate

Alla nascita, cianosi ingravescente, polipnea, scompenso cardiaco

TRASPOSIZIONE DELLE GRANDI ARTERIEDIV

Circolazione sistemica e polmonare non in serie ma in parallelo

Sopravvivenza assicurata dal mixing a livello atriale, ventricolare e duttale

TRASPOSIZIONE DELLE GRANDI ARTERIEDIV

Diagnosi La DIAGNOSI spesso prenatale

TGA nascita in elezione presso centri 3 livello

1) Ipossia e acidosi: entro 24 ore ( chiusura del PDA) infusione PGE1

2) Segni si scompenso cardiaco (epatomegalia, polipnea) se

presente un VSD

3) Diagnosi ecocardiografica accurata e programmazione correzione

Primi trattamenti

Infusione di PGE1 per far riaprire il dotto arterioso se presente cianosi

Atriosettostomia sec. Rashkind: procedura ecoguidata, si introduce un catetere con pallone in vena femorale e da qui lo si spinge attravero la vena cava inferiore in atrio destro. Si fa passare in catetere attraverso il forame ovale in atrio sinistro. Si gonfia il pallone e si tira con forza per lacerare la valvola del forame ovale in modo da aumentare il mixing intracardiaco.

Terapia medica antiscompenso fino allintervento.

Correzione chirurgica

Indicazioni allintervento:

CORREZIONE PRIMARIA NELLE PRIME 2 SETTIMANE DI VITA

Correzione precoce: il ventricolo sinistro perde la sua ipertrofia contro le

basse resistenze polmonari.

Non pi in grado di sostenere acutamente le resistenze sistemiche post intervento correttivo.

Utile la diagnosi prenatale

Alla nascita, PGE1 ed eventualmente Rashkind

Intervento chirurgico (Arterial Switch) deve essere effettuato nelle prime settimane di vita

Ottimi risultati chirurgici nel breve e lungo termine

TRASPOSIZIONE DELLE GRANDI ARTERIEDIV

Periodo: 1990-2017

N=260 pazienti

Simple DTGA IVS: 177

Complex DTGA: 83

Et correzione: 8 giorni (IQR 6-12 giorni)

Mortalit ospedaliera: 5.8 %

Simple DTGA: 3.4 %

Complex DTGA: 11%

Mortalit tardiva: 2 (0.8%)(complex DTGA)

Reinterventi tardivi: 6.9%

Procedure emodinamiche al follow-up: 9.8%

Risultati

Associated CHDs in complex D-TGA

VSD* 79 (95%)

LVOTO* 10 (12%)

Aortic Coarctation* 10

(12%)

Other less common CHDs* 16 (19%)

University of Padua Medical SchoolPediatric and Congenital Cardiac Surgery Unit

Tetralogia di Fallot

Descritta per la prima volta nel 1888 da Etienne-Louis Arthur Fallot.

Viene chiamata anche

Morbo blu o Maladie bleu.

Fisopatologia

La cianosi dovuta allo shunt destro-sinistro.

Lentit dello shunt destro-sinistro determinata dal grado di stenosi del RVOT e dalle resistenze vascolari sistemiche.

LVRV

AO

Anatomy

Surgical history of repair (University of Padua)Evolution of our surgical policy

Since 80s: Classic transventricular

repair in infants

Since 1991: Early transatrial one-stage

repair (6-3 months of age)

Since 2007: Further evolution: PV

preservation

Correzione chirurgica

Indicazioni allintervento:

Timing chirurgico:

a) Pazienti sintomatici (neonati cianotici, crisi ipossiche) correzione immediata

b) Pazienti asintomatici correzione elettiva nei primi 3 mesi di vita

Surgical repair: timing

The timing for complete repair is still controversial and varies from center to

center.

World-wide trend favors early repair (3-6 months of life), just as for many other

complex CHDs.

Complete early repair has been advocated to avoid:

- Abolish chronic cyanosis and spells

- Systemic-to-pulmonary artery shunts and their consequences

- Chronic RV pressure overload and myocardial compensatory hypertrophy

(which requires a more extensive resection, at the time of the repair)

- Hospital costs and offering patients one instead of two operations.

Trans-atrial approach

Our standard institutional policy for TOF repair in early infancy since June 1991

(usually in patients 3 months of life) includes:

1) trans-atrial/trans-pulmonary approach to the pulmonary outflow tract

2) incision of the PV annulus with minimal right ventriculotomy (3-5 mm), when

necessary

3)trans-atrial closure of the ventricular septal defect using an autologous

pericardial patch

Trans-atrial approach

We have demonstrated a trend towards a reduced RV volume and a better

ejection fraction in the long term after transatrial repair, when compared to a

classic transventricular repair.

Minimizing or eliminating a right ventriculotomy may also reduce the substrate

for ventricular arrhythmias arising from incisions in the right ventricle.

Stellin G, Milanesi O, Rubino M, Michielon G, Bianco R, Moreolo GS, Boneva R, Sorbara C, Casarotto D. Repair of tetralogy of Fallot in the first six months of life: transatrial versus transventricular approach. Ann Thorac Surg. 1995 Dec;60(6 Suppl):S588-91.

Transannular patch

Despite the awareness of late consequences of

pulmonary valve regurgitation, TAP remains the

most prevalent technique for repairing TOF.

Leading inevitably to chronic PR

Monocusp for RVOT reconstruction

Monocusp reconstruction of the RVOT by using either pulmonary

homografts (less frequently prosthetic material)

This can avoid immediate PVR, improving the short-term clinical

outcome.

Nonetheless, when a cusp needs to be added, leaflets function

often deteriorate over time, resulting in a progressive PV

regurgitation.

PV preservation techniques

Since 2007

Transatrial / transpulmonary approach

+

PV balloon dilation (trans-atrial)

+/- valve resuspension

Vida VL, Padalino MA, Maschietto N, Biffanti R, Anderson RH, Milanesi O, Stellin G.The balloon dilation of the pulmonary valve during early repair of Tetralogy of Fallot.Catheter Cardiovasc Interv. 2012 Nov 15;80(6):915-21.

Initial indication PV Z-score -3

PV preservation techniques

Data at follow-up

58 pts (96%) at follow-up

Medianfollow-up time of 3.5 yrs (IQR 1.6-5.2 yrs)

Only 1 late reoperations for RVOT early in our experience

Themedian RVOT gradient: 20 mmHg (IQR 15-25 mmHg)

The degree of PV regurgitation (total at follow-up)

None/mild in 42 pts (75%)

Moderate in 14 pts (25%)

We believe that the preservation of the PV

function during early repair of TOF, by

combining different intra-operative surgical

maneuvers, can be extended to almost all

patients with classic TOF

Conclusions

Conclusions

Our results show that this new surgical technique:

1) can reduce early and mid-term post-operative

pulmonary regurgitation

2) improving RV function

By preserving PVfunction during early TOF repair we

should contribute toameliorate the long-term follow-up,

possibly decreasing the number of future re-interventions on

the RVOT.

A longer follow-up and a larger case-series are needed.

Conclusions

Anatomical Theatre Palazzo Bo - Padova Galileo Galileis chair Palazzo Bo - Padova

Forma parziale

Forma completa

Difetti del setto atrioventricolare (Canale A-V)

Canale atrio-ventricolare completo

Canale atrio-ventricolare completo

Segni di iperafflusso polmonare

RX torace

Anomalie associate

Sindrome di Down (70%)

Tetralogia of Fallot

Ventricolo destro a doppia entrata e trasposizioni delle grandi arterie

Ostruzione del tratto defflusso del ventricolo sinistro

DIV multipli

Valvola Mitrale a doppio orifizio

Muscolo papillare singolo

Canale atrio-ventricolare completo

Approccio chirurgico

Correzione elettiva entro 2-3 mesi di vita

PA banding in rari casi con controindicazione BPCPT

Diagnosi ecocardiografica esaustiva

Canale atrio-ventricolare completo

Canale atrio-ventricolare completo

Correzione chirurgica

Periodo: 1992-2014 (22 anni)

N=159 pazienti con CAVC

133 CAVC

21 CAVC forma transizionale

Et correzione: 96 giorni (IQR 73-128 giorni)

Mortalit ospedaliera: 1.9%

CAVC: 2.3 %

CAVC forma transizionale: 0%

Mortalit tardiva: 7.7%

Reinterventi sulla valvola AV sx: 10%

Risultati

U.O.A. Cardiochirurgia Pediatrica, Universit degli Studi di Padova

1950 The surgery of TGAcommenced in 1950 when AlfredBlalock and Rollins Hanlon described a closed method of atrialseptectomy at the Johns Hopkins Hospital.

Blalock A, Hanlon CR:The surgical treatment of completetransposition of the aorta and the pulmonaryartery.Surg Gynaecol Obstet 1950, 90: 1

Transposition of the great arteries: history

First palliative procedure thatpermitted survival of children withTGA.

Surgical atrial septectomy

Although the Blalock-Hanlonoperation and its modificationswere eventually superseded byRashkinds technique of enlargingthe foramen ovale with a ballooncatheter, they produced goodintermediate-term palliation andwere applied in some institutionsfor decades after their firstdescription.

Transposition of the great arteries: historySurgical atrial septectomy

In the 50s : Early arterial switch attempts and techniques of venous return transfer:

The initial attempts were all universally fatal.

1952: Mustard (Hospital for Sick Children, Toronto): several attempts in infancyby transferring the LCA into the neoaorta, in isolation, using a monkey lung as abiological oxygenator.

Mustard WT et AlThe surgical approach to transposition of the great vessels with extracorporeal circuit.Surgery 1954;36:3951.

Transposition of the great arteries: historyEarly arterial switch attempts

In the 50s: Early arterial switch attempts and techniques of venous return transfer:

The initial attempts were all universally fatal.

1955: Ake Senning (Karolinska Institutet, Sweden): after several attemptsconcluded that the obstacle to the anatomical correctiontransfer of the coronaryarteries

Senning A.Surgical correction of transposition of the great vessels.Surgery 1959;45:96680.

Transposition of the great arteries: historyEarly arterial switch attempts

1953: Walton Lillehei and Richard Varco described the first attempt of aphysiological repair, consisting of the anastomosis of right pulmonary veins toRA and the IVC to LA, a technique that became known as the Baffesoperation (without CPBP, 38 pts, 19 survivers).

Lillehei CW, Varco RL:Certain physiologic, pathologic, and surgicalfeatures of complete transposition of the greatarteriesSurgery 1953, 34: 376

Baffes TG:New method for surgical correction oftransposition of aorta and pulmonary artery.Surg. Gynec & Obst, 1956, 102: 227

Transposition of the great arteries: historyEarly atrial switch attempts

1954 Harold M. Albert (Children's Memorial Hospital Chicag o): flap ofthe atrial septum for systemic and pulmonary venous flow redirection in dogs.

Albert HM .Surgical correction of transposition of thegreat vessels.Surg Forum 1954;5:747

Transposition of the great arteries: historyEarly atrial switch attempts

1957 Alvin Merendino (University of Washington in Seattle):first attempted to clinically apply a modified Alberts technique.

Merendino used apremodeled atrial septal prosthesis.(2 Attempts; both patients died)

Meredino KA et AlInteratrial venous transposition.Surgery 1957;42:898909.

Transposition of the great arteries: historyEarly atrial switch attempts

1957, Ake Senning (Karolinska Hospital): using flaps ofautogenous atrial tissue.

Senning A.Surgical correction of transposition of thegreat vessels.Surgery 1959;45:96680.

Transposition of the great arteries: history

First successful atrial switch.

Atrial switch

1961 John W. Kirklin used the Senningoperation at the Mayo Clinic and by 1961 hadoperated on 11 infants, with 4 survivors.

Arterial and atrial switch

Kirklin JWOpen intracardiac repair of transposition of thegreat vessels.Surgery 1961;50: 5866.

Transposition of the great arteries: history

1963, Bill Mustard (Hospital for Sick Children, Toronto)atrial switch using an autologous pericardial baffle upon an 18-month-old girl who had previously undergone a Blalock-Hanlonoperation.

Mustard WT.Successful two-stage correction of thetransposition of the great vessels.Surgery 1964;55:46972

Transposition of the great arteries: historyAtrial switch

First successful atrial switch using apantaloon patch .

In the 60s the Senning operation waslargely abandoned to favor a lesscomplicated Mustard operation.For the next decade, the Mustard operationwas universally employed.

In the middle 70s the Senning operationwas eventually revived for the potentialadvantage of avoiding prosthetic baffles,especially in small children.

Quaegebeur JM, Rohmer J, Brom AG.Revival of the Senning operation in thetreatment of transposition of the greatarteries.Thorax 1977;32:51724.

Transposition of the great arteries: historyAtrial switch

1966: Rashkind and Miller in Philadelphia performed the first balloon atrialseptectomy

1975: Parkmodified this procedure introducing a blade catheter.

A revolution in palliation

Rashkind WJ, Miller WW .Creation of an atrial septal defect withoutthoracotomy. A Palliative Approach toComplete Transposition of the Great Arteries.JAMA. 1966

Park SC.A new atrial septostomy technique.Cath Cardiovasc Diagn 1: 195, 1975

Transposition of the great arteries: history

1976, Adib Jatene (Hospital das Clnicas, So Paulo):firstsuccesfull arterial switch in patient with a VSD.

Jatene ADAnatomic correction of transposition of the great vessels.J Thorac Cardiovasc Surg. 1976 Sep;72(3):364-70.

Transposition of the great arteries: history

At that time, switch operation was performedin the presence only either of a VSD orsubpolmonary obstruction

Arterial switch

Jatene AD, Paulista et AlSuccessful anatomic correction of TGA: a preliminary report.Arq Bras Cardiol. 1975

In the 70s: high early operative mortality for switch due todifficulties in transferring coronary arteries. Alternativetechniques avoiding the transfer of the coronary arterieswere developed By:

Transposition of the great arteries: historyArterial switch

1978 Aubert JTransposition of the great arteries. Newtechnique for anatomical correction.Br Heart J. 1978 Feb;40(2):204-8.

1980 Bex JP, Lecompte Y.Anatomical correction of transposition of thegreat arteries.Ann Thorac Surg. 1980 Jan;29(1):86-8.

1975 Stansel HC Jr.A new operation for d-loop transposition of thegreat vessels.Ann Thorac Surg. 1975 May;19(5):565-7.

1981, Lecompte :important technical modification avoiding aan RV-PA conduit.

Transposition of the great arteries: historyArterial switch

Lecompte Y, et AlAnatomic correction of transposition of the great arteries.J Thorac Cardiovasc Surg. 1981 Oct;82(4):629-31.

Castaneda AR, Norwood WI, Jonas RA,Colon SD, Sanders SP, Lang P.Transposition of the great arteries andintact ventricular septum: anatomical repairin the neonate.Ann Thorac Surg. 1984 Nov;38(5):438-43.

Transposition of the great arteries: historyArterial switch

1984, Aldo Castaneda (Boston Childrens Hospital) and PaulEbert demonstrated the feasibility of switch operation for d-TGA and IVS provided it was performed within the first 3-4weeks of life.

Yacoub MHTwo-stage operation for anatomical correction oftransposition of the great arteries with intactinterventricular septum.Lancet. 1977 Jun 18;1(8025):1275-8

Transposition of the great arteries: historyArterial switch

1977, Yacoub et Al introduced the two-stage approach bybanding the MPA(with or without systemic-pulmonary arteryshunt) to stimulate the development of left ventricle mass,followed by arterial switch a fewmonths later.

The majority of patients with TGA have an intact ventricularseptum and no pulmonary stenosis.

Jonas RA, Giglia TM, Sanders SP, Wernovsky G,Nadal-Ginard B, Mayer JE Jr, Castaneda AR.Rapid, two-stage arterial switch for transposition ofthe great arteries and intact ventricular septum beyondthe neonatal period.Circulation. 1989 Sep;80(3 Pt 1):I203-8

Transposition of the great arteries: historyArterial switch

1988, the Boston Childrens Hospitalintroduced the concept ofa two-stage arterial switch operation with IVS (PAbanding + B-T shunt) limiting the interval between the first and secondoperation to an average of 7 days.

1974 Vincenzo Gallucciperformed the first Mustard operation.

Transposition of the great arteries: historyHistory of the University of Padua

In late 70s Mustard operation wasabandoned to favor the Senning operationwith excellent outcome (Prof. V. Gallucci;Prof. A. Mazzucco).

In early 80s Padua was pioneering thearterial switch operation in Italy to becomethe preferred operation in the presence ofnormal coronary arteries anatomy.

1993: The first successful arterial switch operationfor d-TGA and intramural coronary arteries.

Since 1991, over 340 arterial switch operation havebeen performed in our institution with a more recentoperative mortality of 2% and excellent long termresults.

Transposition of the great arteries: historyHistory of the University of Padua

Transposition of the great arteries: historyArterial switch

1984, Aldo Castaneda (Boston Childrens Hospital) and PaulEbert demonstrated the feasibility of switch operation for d-TGA and IVS provided it was performed within the first 3-4weeks of life.

Diagnosi

Radiografia del Torace

Cuore a scarpa (coeur en sabot)

Riduzione della trama vascolare polmonare

Elective early repair within the first 3 months ofage (in asymptomatic infants)

Earlier repair when symptoms No B-T shunt (except for LBW, associated

morbidities) (3 B-T shunts since 2000) Cyanotic crises are controlled with blocker No RVOT stenting

PGE at birth;neonatal repair

PDA stenting (since 2011);early repair

Current criteria for ToF approachCurrent protocol of treatment

Classic ToF

ToF + PV atresia(no MAPCAs)

Quadro clinicoI quadri clinici dipendono dal grado di stenosi del RVOT

Fallot rosa

Fallot bluStenosi severa

Stenosi moderata

Stenosi lieve

Cianosi neonatale

Crisi ipossiche

No crisi ipossiche, lieve cianosi

Et di presentazione

1 sett di vita

Dopo i 3 mesi di vita

Canale atrio-ventricolare completo

Difetto del setto atrioventricolare

1. DIA tipo Ostium Primum

2. DIV tipo inlet

3. Valvola AV comune

Anomalie associate

1. Difetto interatriale e/o forame ovale pervio (DIA)

2. Dotto arterioso pervio (PDA)

3. Origine anomala delle coronarie

4. Difetto del setto interventricolare (VSD)

5. Stenosi della valvola polmonare

Diagnosi

Ecocardiogramma

Permette il corretto inquadramento anatomico visualizzando

Sbandamento anteriore del setto infundibulare Presenza del VSD Entit della stenosi del RVOT

- stenosi della valvola polmonare,

- valvola polmonare bicuspide, displasica, atresica o aplasica

- ipoplasia dellannulus polmonare

- stenosi dinamiche di tipo muscolare

Grado di ipertrofia ventricolare destro Dimensioni delle arterie polmonari Anomalie associate