关于bosentan治疗先天性心脏病合并肺动脉高压成年患者的首次开放性、前瞻性、多中心研究

作     者：Schulze-Neick I. Gilbert N. Ewert R. 任付先 

作者机构：Abteilung fü r Angeborene Herzfehler Deutsches Herzzentrum Berlin Augustenburger Platz 1 13353 Berlin Germany Dr. 

出 版 物：《世界核心医学期刊文摘(心脏病学分册)》 (Digest of the World Core Medical Journals(Cardiology))

年 卷 期：2006年第2期

页      面：31-31页

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：肺动脉 特发性肺动脉高压 先天性心脏病 心脏先天畸形 bosentan 成年患者 

摘      要：Background: Endothelin receptor antagonism has been introduced as an effective oral therapy of patients with idiopathic pulmonary arterial hypertension. In view of the pathophysiologic and histologic similarities between idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease(PAH-CHD), there is a rationale for treating these patients with the oral dual(ETA/ETB) endothelin receptor antagonist bosentan. Methods: Thirty-three patients with PAH-CHD(43± 14 years, 23 with Eisenmenger syndrome) were treated with bosentan for a mean of 2.1± 0.5 years. Efficacy was assessed by a panel of tests, including New York Heart Association functional class, 6-minute walking distance, and echocardiographic and hemodynamic parameters. Results: Mean 6-minute walking distance increased from 362± 105 to 434± 68 m(P=.001). New York Heart Association class also improved significantly(3.1 to 2.4, P=.0001). This was associated with slight trends in improvements of transcutaneous oxygen saturation(86% ± 7% to 88% ± 7% , P=.13) and maximum oxygen uptake(13.2± 4.0 to 14.9± 2.5, P=.18). Right ventricular systolic pressure measured by echocardiographic decreased from 111± 32 to 106± 22 mm Hg(P=.001). Bosentan treatment was well tolerated by all patients. Conclusions: Long-term bosentan treatment in adult patients with PAH-CHD was well tolerated and improved functional status as well as exercise capacity. These findings have to be corroborated by controlled studies that are presently ongoing.