Ipertensione arteriosa polmonare: algoritmo diagnostico e importanza del trattamento precoce

Transcript

1 Ipertensione arteriosa polmonare: algoritmo diagnostico e importanza del trattamento precoce Antonella Romaniello UOC Cardiologia Ospedale Sant Andrea Roma

2 IPERTENSIONE POLMONARE Condizione emodinamica, caratterizzata dall incremento della pressione polmonare media, misurata con il cateterismo cardiaco destro. PAPm 25 mmhg IPERTENSIONE ARTERIOSA POLMONARE Malattia rara, dovuta al rimodellamento dei piccoli vasi polmonari che porta all aumento delle resistenze polmonari

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4 CLASSIFICAZIONE

5 GRUPPO I Ipertensione arteriosa polmonare -Idiopatica/Ereditaria/Familiare -Da farmaci/tossine -Associata: CTD/HIV/PoH/CHD/Schist 1 Malattia venoocclusiva polmonare MALATTIA RARA 3,5% di tutti i casi di IP GRUPPO II Forma idiopatica casi/milione Ipertensione polmonare da malattie del cuore sinistro 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 RARA 1,5% di tutti i casi di IP GRUPPO V Ipertensione polmonare con meccanismi multifattoriali/non chiari 7% di tutti i casi di IP 78% di tutti i casi di IP Linee guida PH ESC 2015

6 Primo: una corretta diagnosi!! nell ipertensione polmonare non così scontato

7 .precoce Storia naturale Ipertensione Arteriosa Polmonare mod. Rich et al. Harrison s Principles of Internal Medicine

8 CLASSE FUNZIONALE CHEST 2015; 148(4):

9 ALGORITMO DIAGNOSTICO

10 Galiè et al. Eur Heart J 2016; CTD CHD EP HIV Familiarità

11 ECOCARDIOGRAMMA Probabilità di ipertensione polmonare Galiè et al. Eur Heart J 2016;

12 SEGNI ECOCARDIOGRAFICI AGGIUNTIVI Galiè et al. Eur Heart J 2016;

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17 Algoritmo diagnostico IP gruppo II-III Galiè et al. Eur Heart J 2016; Approfondimenti diagnostici

18 IP gruppo IV IP gruppo I

19 TERAPIA

20 Gruppo I: Ipertensione Arteriosa Polmonare CHEST (2)

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

22 Valutazione del rischio

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25 e gli altri gruppi?

26 Gruppo II: PH più frequente Recommendations Optimisation of the treatment of the underlying condition before considering assessment of PH-LHD (i.e. treating structural heart disease) Identify other causes of PH (i.e. COPD, sleep apnoea syndrome, PE, CTEPH) and treat them when appropriate before considering assessment of PH-LHD Perform invasive assessment of PH in patients on optimised volume status 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 The importance and role of vasoreactivity testing is not established in PH-LHD, except in patients who are candidates for heart transplantation and/ or LV assist device implantation The use of PAH-approved therapies is not recommended in PH- LHD Class a I I I IIa III III Level b B C C C C C

27 Recommendations Gruppo III Echocardiography is recommended for non-invasive diagnostic assessment of suspected PH in patients with lung disease. Referral to an expert center is recommended in patients with echocardiographic signs of severe PH and/or severe right ventricular dysfunction 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. Referral to PH expert center should be considered for patients with signs of severe PH/ severe right failure for individual based treatment. 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) The use of drugs approved for PAH is not recommended in patients with PH due to lung diseases. Class a I I I IIa III III Level b C C C C C C

28 Recommendations Gruppo IV In PE survivors with exercise dyspnoea, CTEPH should be considered Life long anticoagulation is recommended in all patients with CTEPH 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. Surgical PEA in deep hypothermia circulatory arrest is recommended for patients with CTEPH 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 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. Class a IIa I I I I IIb Level b C C C C B B

29 CONCLUSIONI I. 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.

30 Grazie per l attenzione Dott.ssa Antonella Romaniello Sapienza Facoltà di Medicina e Psicologia Ospedale Sant Andrea, Roma UOC Cardiologia aromaniello@ospedalesantandrea.it Tel /5246