Long-term tolerability of phosphodiesterase-5 inhibitors in pulmonary hypertension of sickle cell disease.

Abstract

Sickle cell disease-related pulmonary hypertension (SCD-PH) is a complex disorder with multifactorial contributory mechanisms. Previous trials have evaluated the efficacy of pulmonary arterial hypertension (PAH) therapies in SCD-PH with mixed results. We hypothesized that a subset of patients with right heart catheterization (RHC) confirmed disease may benefit from PAH therapy. We performed a retrospective chart review of patients with SCD-PH diagnosed by RHC who were treated with phosphodiesterase 5 inhibitor (PDE5-I) therapy for ≥4months between 2008 and 2019 at two institutions. Thirty-six patients were included in the analysis. The median age (IQR) upon PDE5-I initiation was 47.5years (35-51.5years); 58% were female and twenty-nine (81%) had HbSS disease. Of these, 53% of patients had a history of acute chest syndrome, 42% had a history of venous thromboembolism, and 38% had imaging consistent with chronic thromboembolic PH. Patients were treated for a median duration of 25months (IQR 13-60months). Use of PDE5-I was associated with a significant improvement in symptoms as assessed by NYHA Class (P=.002). In SCD patients with PH defined by RHC, PDE5-I therapy was tolerated long-term and may improve physical activity.