Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation

Pulmonary hypertension (PH) has been associated with increased mortality in patients with advanced pulmonary sarcoidosis. Sarcoidosis-associated PH may be the result of left ventricular dysfunction (LVD) or isolated pulmonary vasculature abnormality. Our objective was to determine if the cause of PH affects survival in patients with sarcoidosis with persistent dyspnea.

Patients with sarcoidosis with persistent dyspnea despite immunosuppressive therapy underwent right-sided heart catheterization. Patients with a pulmonary artery occluding pressure (PAO) ≥ 15 mm Hg were defined as having LVD. Patients were classified based on hemodynamics as no PH (pulmonary artery mean pressure [PAmean] < 25 mm Hg), PH without LVD (PAmean ≥ 25 mm Hg and PAO < 15 mm Hg), and PH with LVD (PH/LVD) (PAmean ≥ 25 mm Hg and PAO ≥ 15 mm Hg).

One hundred thirty patients were studied at one institution: 50 (38.5%) patients had PH without LVD, whereas 20 (15.4%) had PH/LVD. All patients had their diagnostic procedure at least 18 months prior to analysis. The hazard ratio (HR) for death in PH without LVD vs no PH was 10.39 (95% CI, 2.99-13.78; P < .0001). The HR for dying for PH without LVD vs PH/LVD was 3.14 (95% CI, 1.01-5.62; P < .05). The presence of stage 4 chest roentgenograms and the need for supplemental oxygen were different between the groups. In a Cox proportional hazards model, independent predictors for survival were stage 4 chest roentgenogram (P < .005) and hemodynamic group (P < .02).

PH without LVD was associated with increased mortality. Proper characterization of patients required hemodynamic evaluation, as 29% of sarcoidosis-associated PH was due to LVD.