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

Objectives 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. Methods 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 ≥4 months between 2008 and 2019 at two institutions. Results Thirty‐six patients were included in the analysis. The median age (IQR) upon PDE5‐I initiation was 47.5 years (35‐51.5 years); 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 25 months (IQR 13‐60 months). Use of PDE5‐I was associated with a significant improvement in symptoms as assessed by NYHA Class (P = .002). Conclusions In SCD patients with PH defined by RHC, PDE5‐I therapy was tolerated long‐term and may improve physical activity.