Pulmonary hypertension related to heart and lung disease has the worst prognosis compared to the others forms including pulmonary arterial hypertension

Background 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. Purpose To compare the prognostic significance of each forms of PH in a prospective regional cohort from a referral centre. Methods 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 u003e 25 and u003e 35 mm Hg for those with PH related to lung disease. PH patients with left heart disease, only those with diastolic or transpulmonary pressure gradient u003e 7 or u003e 12 mm 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). Results A total of 74% patients were in NYHA class 3–4. The 6-min walking test averaged 294 ± 139 m, the NTproBNP was increased up to 2990 ± 5894 pg/L. Mean pulmonary artery pressure was 43.3 ± 12.8 mm Hg and cardiac index was 2.95 ± 0.91 L/min/m 2 . Right atrial pressure and pulmonary vascular resistance averaged 9.5 ± 5.9 mm Hg and 6.5 ± 3.8 WU, 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 Fig. 1 ). Conclusion 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.