Frequency, characteristics and risk assessment of pulmonary arterial hypertension with a left heart disease phenotype

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2024年4月15日

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Matteo Toma,
Giulio Savonitto,
Carlo Maria Lombardi,
Edoardo Airò,
Mauro Driussi,
Piero Gentile,
Luke Howard,
Martina Moschella,
Emma Di Poi,
Matteo Pagnesi,
Simonetta Monti,
Valentino Collini,
Luciana D’Angelo,
Veronica Vecchiato,
Alberto Giannoni,
Marianna Adamo,
Davide Barbisan,
Carolina Bauleo,
Andrea Garascia,
Marco Metra,
Gianfranco Sinagra,
Francesco Lo Giudice,
Davide Stolfo,
Pietro Ameri

摘要

Aim

To obtain real-world evidence about the features and risk stratification of pulmonary arterial hypertension (PAH) with a left heart disease (LHD) phenotype (PAH-LHD).

Methods and results

By reviewing the records of consecutive incident PAH patients at 7 tertiary centers from 2001 to 2021, we selected 286 subjects with all parameters needed to determine risk of death at baseline and at first follow-up with COMPERA and COMPERA 2.0 scores. Fifty seven (20%) had PAH-LHD according to the AMBITION definition. Compared with no-LHD ones, they were older, had higher BMI, more cardiovascular comorbidities, higher E/e’ ratio and left atrial area, but lower BNP concentrations and better right ventricular function and pulmonary hemodynamics. Survival was comparable between PAH-LHD and no-LHD patients, although the former were less commonly treated with dual PAH therapy. Both COMPERA and COMPERA 2.0 discriminated all-cause mortality risk of PAH-LHD at follow-up, but not at baseline. Risk profile significantly improved during follow-up only when assessed by COMPERA 2.0. At multivariable analysis with low-risk status as reference, intermediate-high and high-risk, but not LHD phenotype, were associated with higher hazard of all-cause mortality. Results were comparable in secondary analyses including patients in the last 10 years and atrial fibrillation and echocardiographic abnormalities as additional criteria for PAH-LHD.

Conclusions

In real life, PAH-LHD patients are frequent, have less severe disease and are less likely treated with PAH drug combinations than no-LHD. The COMPERA 2.0 model may be more appropriate to evaluate their mortality risk during follow-up and how it is modulated by therapy.