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- Title
Long‐term tolerability of phosphodiesterase‐5 inhibitors in pulmonary hypertension of sickle cell disease.
- Authors
Cramer‐Bour, Cassondra; Ruhl, Amy Parker; Nouraie, Seyed Mehdi; Emeh, Robert O.; Ruopp, Nicole F.; Thein, Swee Lay; Weir, Nargues A.; Klings, Elizabeth S.
- Abstract
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.
- Subjects
SICKLE cell anemia; PHOSPHODIESTERASE-5 inhibitors; PULMONARY hypertension; PHOSPHODIESTERASE inhibitors; THROMBOEMBOLISM; CARDIAC catheterization
- Publication
European Journal of Haematology, 2021, Vol 107, Issue 1, p54
- ISSN
0902-4441
- Publication type
Article
- DOI
10.1111/ejh.13612