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.

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Sickle cell disease (SCD) affects approximately 100,000 Americans and affects 250,000 births each year worldwide. Despite being a genetic hemoglobinopathy, SCD is a diffuse vascular disease and complications such as acute chest syndrome (ACS), sepsis and stroke are the most common causes of death. Many intensivists experience relative discomfort in managing SCD patients. We proposed to develop a protocol of simulation driven learning to increase education surrounding critical care complications of SCD. We piloted this protocol with Internal Medicine housestaff.