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

Abstract

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 > 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 > 7 or > 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). 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 < 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 ( Fig. 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.