Ipertensione arteriosa polmonare: algoritmo diagnostico e importanza del trattamento precoce
Antonella RomanielloUOC Cardiologia
Ospedale SantAndreaRoma
IPERTENSIONE POLMONARECondizione emodinamica, caratterizzata dallincremento della pressione
polmonare media, misurata con il cateterismo cardiaco destro.PAPm 25 mmHg
Malattia rara, dovuta al rimodellamento dei piccoli vasi polmonari che porta allaumento 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 IPGRUPPO V
Ipertensione polmonare con meccanismi multifattoriali/non chiari
7% di tutti i casi di IP
Primo: una corretta diagnosi!!
nell ipertensione polmonare non cos scontato
mod.Rich et al.HarrisonsPrinciples of Internal Medicine
.precoceStoria naturale
Ipertensione Arteriosa Polmonare
CHEST 2015; 148(4):10431054
CLASSE FUNZIONALE
ALGORITMO DIAGNOSTICO
Gali et al. Eur Heart J 2016; 37 67-119
CTDCHDEPHIVFamiliarit
Gali et al. Eur Heart J 2016; 37 67-119
ECOCARDIOGRAMMA
Probabilit di ipertensione polmonare
Gali et al. Eur Heart J 2016; 37 67-119
SEGNI ECOCARDIOGRAFICI AGGIUNTIVI
Gali 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) 330334
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 pi frequente
Recommendations Classa LevelbOptimisation 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 allaumento della
pressione nel circolo polmonare.II. Lipertensione 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 lattenzione
Dott.ssa Antonella RomanielloSapienza Facolt di Medicina e Psicologia
Ospedale SantAndrea, RomaUOC Cardiologia
Email: [email protected] 06 33775121/5246
