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

被引:7
作者
Cramer-Bour, Cassondra [1 ]
Ruhl, Amy Parker [2 ,3 ]
Nouraie, Seyed Mehdi [4 ]
Emeh, Robert O. [5 ]
Ruopp, Nicole F. [6 ]
Thein, Swee Lay [7 ]
Weir, Nargues A. [3 ]
Klings, Elizabeth S. [1 ,8 ]
机构
[1] Boston Univ, Sch Med, Dept Med, Boston, MA 02118 USA
[2] NIAID, Lab Malaria & Vector Res, NIH, 9000 Rockville Pike, Bethesda, MD 20892 USA
[3] NHLBI, Pulm Branch, Bldg 10, Bethesda, MD 20892 USA
[4] Univ Pittsburgh, Dept Med, Div Pulm Asthma & Crit Care Med, Pittsburgh, PA USA
[5] Howard Univ, Coll Med, Washington, DC USA
[6] Tufts Med Ctr, Div Pulm Crit Care & Sleep Med, Boston, MA 02111 USA
[7] NHLBI, Sickle Cell Branch, Bethesda, MD USA
[8] Boston Univ, Sch Med, Pulm Ctr, Boston, MA USA
基金
美国国家卫生研究院;
关键词
phosphodiesterase; 5; inhibitor; pulmonary arterial hypertension; sickle cell disease; SILDENAFIL; MORTALITY; ADULTS;
D O I
10.1111/ejh.13612
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:54 / 62
页数:9
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