Journal
EUROPEAN JOURNAL OF HAEMATOLOGY
Volume 107, Issue 1, Pages 54-62Publisher
WILEY
DOI: 10.1111/ejh.13612
Keywords
phosphodiesterase 5 inhibitor; pulmonary arterial hypertension; sickle cell disease
Categories
Funding
- Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health
- Division of Intramural Research, National Heart, Lung, and Blood Institute, National Institutes of Health
- Division of Intramural Research for NIAID
- NIH
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This study retrospectively reviewed 36 patients with SCD-PH treated with PDE5-I therapy, showing a significant improvement in symptoms as assessed by NYHA Class with long-term tolerability of PDE5-I therapy.
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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