Pulmonary hypertension (PH) still has a poor prognosis. To prioritize effort in detection and management of PH, it is important to point out which of the 5 phenotypes has the worst prognosis and must retain our attention.

To compare the prognostic significance of each forms of PH in a prospective regional cohort from a referral centre.

A toal of 328 patients (age=63.4±14 yo, 47% of male) were prospectively included from 2002 to 2017, after right heart catheterisation if they had mean pulmonary artery pressure>25 and>35mm Hg for those with PH related to lung disease. PH patients with left heart disease, only those with diastolic or transpulmonary pressure gradient>7 or>12mm Hg respectively, were analysed. Patients were routinely followed-up after therapeutic optimisation. Five-year survival rate was then compared using the log-rank test and adjusted hazard ratio (HR).

A total of 74% patients were in NYHA class 3–4. The 6-min walking test averaged 294±139m, the NTproBNP was increased up to 2990±5894pg/L. Mean pulmonary artery pressure was 43.3±12.8mm Hg and cardiac index was 2.95±0.91L/min/m². Right atrial pressure and pulmonary vascular resistance averaged 9.5±5.9mm Hg and 6.5±3.8WU, respectively. A total of 138 patients (42%) belonged to the pulmonary arterial hypertension group (PAH), 53 (16%) to left heart disease group (LHD-PH) while 47 (14%) were associated to lung disease (LD-PH). Chronic thromboembolic PH (CTPH) was found in 71 patients (22%) and 19 patients (6%) had unclassified PH (U-PH). The 5-year survival rate was 75% (PAH), 70% (LHD-PH), 62% (LD-PH), 85% (CTPH), and 79% (U-PH), giving a log-rank P<0.001, and adjusted HR of 2.43 95% CI (1.24–4.73) for LHD-PH and 2.95 95% CI (1.43–6.07) for LD-PH (Figure 1).

PH related to left heart disease and lung disease had 2-fold and 3-fold increase in mortality, respectively, compared with others forms of PH including PAH. Thereby, efforts should concentrate on those two prevalent phenotypes.