Assessment of the prognostic value of the estimation of plasma volume or its variation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) after acute right heart failure.

The aim of the study is to show that a low plasma volume status, assessed by the Strauss (estimated plasma volume variation) and the Duarte (instantaneous estimated plasma volume) formulas evaluated during acute right heart failure in patients presenting with PAH or CTEPH, is associated with better long-term outcomes.

Optimization of plasma volume is important for patients with PAH and CTEPH and constitutes a prognostic factor. However, plasma volume status can be difficult to assess in clinical practice. Different formulas have been developed and have shown interest in patients with congestive heart failure with an association between plasma volume estimation and long-term prognosis.

Acute right heart failure may occur in patients with PAH and CTEPH. Thus, the investigators plan to study the prognostic value of these formulas evaluating plasma volume, in patients with PAH or CTEPH, after acute right heart failure.

Inclusion Criteria:

1. Patients with confirmed diagnosis of PAH or CTEPH, referred to the medical department of the investigators
2. Patient with acute right heart failure defined by at least 2 of the following clinical features: worsening of peripheral oedema, development or increase of ascites, weight gain of more than 2.5 kg in 1 week preceding the visit and decline in one NYHA functional class compared to the previous stable state

Exclusion Criteria:

1) Patients with another cause of pulmonary hypertension than PAH or CTEPH, either groups 2, 3 or 5 of the current clinical classification of pulmonary hypertension

One hundred patients with idiopathic, heritable, drug-induced, connective tissue disease-associated, porto-pulmonary or HIV-associated disease were enrolled. At the diagnosis visit, mean±SD age was 59±12 years, 56% of the patients were women and most patients were in World Health Organization/New York Heart Association (WHO/NYHA) Functional Class III or IV.