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.
Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction / Bourji, Khalil I; Kelemen, Benjamin W; Mathai, Stephen C; Damico, Rachel L; Kolb, Todd M; Mercurio, Valentina; Cozzi, Franco; Tedford, Ryan J; Hassoun, Paul M. - In: PULMONARY CIRCULATION. - ISSN 2045-8940. - 7:2(2017), p. 409-420. [10.1177/2045893217700438]
Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction
Mercurio, Valentina;
2017
Abstract
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.File | Dimensione | Formato | |
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7) 2017 Bourji KI, ...Mercurio V...Hassoun PM. Pulm Circ 2017.pdf
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