Effect of Bosentan on Exercise Capacity and Quality of Life in Adults With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease With and Without Down's Syndrome
Effect of Bosentan on Exercise Capacity and Quality of Life in Adults With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease With and Without Down's Syndrome
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DOI：
10.1016/j.amjcard.2009.01.021
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发表时间：
2009-05-01
影响因子：
2.8
通讯作者：
Mulder, Barbara J. M.
中科院分区：
文献类型：
--
作者：
Duffels, Marielle G. J.;Vis, Jeroen C.;Mulder, Barbara J. M.
肺动脉高压与由体肺分流引起的先天性心脏病相关，与发病率和死亡率的高风险相关。在这项回顾性研究中，在58例先天性心脏病相关肺动脉高压成人患者（>18岁）（包括唐氏综合征患者）中评价了波生坦对运动能力和生活质量（QoL）的长期治疗效果。在基线和随访期间，使用实验室检查、6分钟步行试验、QoL问卷和多普勒超声心动图对所有患者进行评价。分别分析了无唐氏综合征患者（n = 30）和唐氏综合征患者（n = 28）的治疗疗效。所有接受波生坦治疗的患者的中位随访时间为22个月（范围3 - 36）。在无唐氏综合征的患者中，平均6分钟步行距离从427 +/- 97米增加到461 +/- 104米（p
Pulmonary arterial hypertension associated with congenital heart disease caused by systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In this retrospective study, the longer term treatment effect of bosentan on exercise capacity and quality of life (QoL) were evaluated in 58 adult patients (>18 years) with pulmonary arterial hypertension associated with congenital heart disease, including patients with Down's syndrome. All patients were evaluated at baseline and during follow-up using laboratory tests, 6-minute walk test, QoL questionnaires, and Doppler echocardiography. Treatment efficacy was analyzed separately for patients without (n = 30) and with Down's syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Down's syndrome, mean 6-minute walk distance increased from 427 +/- 97 to 461 +/- 104 m (p