Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction

Pulmonary hypertension due to heart failure with preserved ejection fraction (PH‐HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH‐HFpEF (SSc‐PH‐HFpEF) versus pulmonary arterial hypertension (SSc‐PAH). We hypothesized that patients with SSc‐PH‐HFpEF have a similar poor overall prognosis compared with patients with SSc‐PAH when matched for total right ventricular load. The analysis included 117 patients with SSc‐PH (93 with SSc‐PAH versus 24 with SSc‐PH‐HFpEF) enrolled prospectively in the Johns Hopkins PH Registry. We examined baseline demographics and hemodynamics at diagnostic right heart catheterization (RHC), two‐dimensional echocardiographic characteristics, six‐minute walking distance (6MWD), treatment modalities, and laboratory values (serum NT‐proBNP, creatinine, uric acid, and sodium), and assessed survival. Demographics and clinical features were similar between the two groups. Baseline RHC showed significantly higher pulmonary and right heart pressures in the SSc‐PH‐HFpEF compared with the SSc‐PAH group. Trans‐pulmonary gradient (TPG), however, was equally elevated without significant difference between the groups. SSc‐PH‐HFpEF patients had left atrial enlargement on echocardiography compared with SSc‐PAH patients. No significant differences were found between groups for 6MWD, NT‐proBNP, and other laboratory values. Although overall median survival time was 4.6 years with no difference in mortality rate between the two groups (SSc‐PH‐HFpEF versus SSc‐PAH: 75% versus 59%; P  = 0.26), patients with SSc‐PH‐HFpEF had a twofold increased risk of death compared with SSc‐PAH patients after adjusting for hemodynamics. Concomitant intrinsic pulmonary vascular disease and HFpEF likely contribute to very poor survival in patients with SSc‐PH‐HFpEF.