Ipertensione arteriosa polmonare: algoritmo diagnostico e importanza del trattamento precoce

Antonella RomanielloUOC Cardiologia

Ospedale Sant’AndreaRoma

IPERTENSIONE POLMONARECondizione emodinamica, caratterizzata dall’incremento della pressione

polmonare media, misurata con il cateterismo cardiaco destro.PAPm ≥ 25 mmHg

Malattia rara, dovuta al rimodellamento dei piccoli vasi polmonari che porta all’aumento delle resistenze polmonari

IPERTENSIONE ARTERIOSA POLMONARE

CLASSIFICAZIONE

Linee guida PH ESC 2015

GRUPPO I

Ipertensione arteriosa polmonare-Idiopatica/Ereditaria/Familiare-Da farmaci/tossine-Associata:

CTD/HIV/PoH/CHD/Schist1’ Malattia venoocclusiva polmonare

MALATTIA RARA

3,5% di tutti i casi di IP

Forma idiopatica 15-50 casi/milione

GRUPPO II

Ipertensione polmonare da malattie del cuore sinistro

78% di tutti i casi di IP

GRUPPO III

Ipertensione polmonare da malattie del polmone e/o ipossia

10% di tutti i casi di IP

GRUPPO IV

Ipertensione polmonare cronica tromboembolica e ostruzioni polmonari

MALATTIA RARA1,5% di tutti i casi di IP

GRUPPO V

Ipertensione polmonare con meccanismi multifattoriali/non chiari

7% di tutti i casi di IP

Primo: una corretta diagnosi!!

…nell’ ipertensione polmonare non così scontato

mod. Rich et al. Harrison’s Principles of Internal Medicine

….precoceStoria naturale

Ipertensione Arteriosa Polmonare

CHEST 2015; 148(4):1043–1054

CLASSE FUNZIONALE

ALGORITMO DIAGNOSTICO

Galiè et al. Eur Heart J 2016; 37 67-119

CTDCHDEPHIVFamiliarità

Galiè et al. Eur Heart J 2016; 37 67-119

ECOCARDIOGRAMMA

Probabilità di ipertensione polmonare

Galiè et al. Eur Heart J 2016; 37 67-119

SEGNI ECOCARDIOGRAFICI AGGIUNTIVI

Galiè et al. Eur Heart J 2016; 37 67-119

Algoritmo diagnostico

IP gruppo II-IIIIP gruppo II-III Approfondimenti diagnostici

Approfondimenti diagnostici

IP gruppo IVIP gruppo IV

IP gruppo IIP gruppo I

TERAPIA

Gruppo I: Ipertensione Arteriosa Polmonare

CHEST 1987 92 (2) 330–334

Lau EMT et al Nat. Rev. Cardiol. 2017 doi:10.1038/nrcardio.2017

Antagonisti recettoriali endotelina 1

Inibitori fosfodiesterasi 5/ stimolatori guanilato

ciclasi

Prostanoidi/agonisti recettori prostaciclina

Ambrisentan Sildenafil Epoprostenolo (e.v.)

Bosentan Tadalafil Treprostinil (s.c.)

Macitentan Riociguat Iloprost (in.)

Selexipag

Valutazione del rischio

…e gli altri gruppi?

Gruppo II: PH più frequente

Recommendations Classa

Levelb

Optimisation of the treatment of the underlying conditionbefore considering assessment of PH-LHD (i.e. treating structural heart disease)

I B

Identify other causes of PH (i.e. COPD, sleep apnoea syndrome, PE, CTEPH) and treat them when appropriate before considering assessment of PH-LHD

I C

Perform invasive assessment of PH in patients on optimised volume status I C

Patients with PH-LHD and a severe pre-capillary component as indicated by a high DPG and/or high PVR should be referred to an expert PH centre for a complete diagnostic workup and an individual treatment decision

IIa C

The importance and role of vasoreactivity testing is not established inPH-LHD, except in patients who are candidates for heart transplantation and/ or LV assist device implantation

III C

The use of PAH-approved therapies is not recommended in PH-LHD III C

Gruppo III

Recommendations Classa

Levelb

Echocardiography is recommended for non-invasive diagnostic assessment of suspected PH in patients with lung disease. I C

Referral to an expert center is recommended in patients with echocardiographic signs of severe PH and/or severe right ventricular dysfunction

I C

The optimal treatment of the underlying lung disease, including long-term O2 therapy in patients with chronic hypoxaemia is recommended in patients with PH due to lung diseases.

I C

Referral to PH expert center should be considered for patients with signs of severe PH/ severe right failure for individual –based treatment.

IIa C

RHC is not recommended for suspected PH in patients with lung disease, unless therapeutic consequences are to be expected (e.g. lung tranplantation, alternative diagnoses such as PAH or CTEPH, potential enrolment in a clinical trial)

III C

The use of drugs approved for PAH is not recommended in patients with PH due to lung diseases. III C

Gruppo IV

Recommendations Classa

Levelb

In PE survivors with exercise dyspnoea, CTEPH should be considered IIa C

Life –long anticoagulation is recommended in all patients with CTEPH I C

It is recommended that in all patients with CTEPH the assessment of operability and decisions regarding other treatment strategies should be made by a multidisciplinary team of experts.

I C

Surgical PEA in deep hypothermia circulatory arrest is recommended for patients with CTEPH I C

Riociguat is recommended in sympomatic patiens who have been classified as having persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH by a CTEPH team including at least one experienced PEA surgeon

I B

Off-label use of drugs approved for PAH may be considered in symtomatic patients who have been classified as having inoperable CTEPH by a CTEPH team including at least one experienced PEA surgeon.

IIb B

CONCLUSIONII. Numerose patologie possono condurre all’aumento della

pressione nel circolo polmonare.II. L’ipertensione arteriosa polmonare e’ una malattia rara,

grave, progressiva.III. Diagnosi ancora tardiva: ritardo di circa 2 aa.IV. Notevoli progressi terapeutici.V. Trattamento “moderno” migliora la sopravvivenza:

I. Corretta stratificazione del rischio:Up front combination therapy

II. Stretto follow-up:Sequential combination therapy

VI. Necessario “dialogo” e rete tra territorio e centri di riferimento ipertensione polmonare.

Grazie per l’attenzione

Dott.ssa Antonella RomanielloSapienza Facoltà di Medicina e Psicologia

Ospedale Sant’Andrea, RomaUOC Cardiologia

Email: aromaniello@ospedalesantandrea.itTel 06 33775121/5246