鲁登巴赫综合征54例临床分析

Clinical analysis of 54 cases of Lutembacher’s syndrome

目的:分析鲁登巴赫综合征(LS)的发病、超声特点和伴发病变,总结诊断及治疗经验。方法:回顾性分析武汉亚洲心脏病医院2008至2015年,诊治的鲁登巴赫综合征患者54例的临床症状、超声特点及诊治方法。结果:全组LS中,51例为经典性LS,3例为获得性LS,均合并不同程度肺动脉高压,绝大部分合并不同程度的三尖瓣关闭不全,少数合并主动脉瓣狭窄和关闭不全,亦有极少数合并永存左位上腔静脉和感染性心内膜炎。①全组病例有50例经超声心动图诊断,4例右心导管提示,2例术中诊断。②全组病例37例行外科治疗,1例行介入治疗,均无为手术期死亡,无严重心力衰竭、肺动脉高压危象及恶性心律失常发生。结论:LS的超声图像特征明显,超声心动图对其有特异性诊断价值;LS应及早手术或者介入治疗,修补或者封堵房间隔缺损同时有效解除或者减轻二尖瓣狭窄,探查并处理合并的三尖瓣关闭不全,可以取得满意的手术效果。

Objective: Aanalysis Lutembacher’s syndrome about the pathogenesis, ultrasonic features and complications, and to summarize the experience in diagnosis and treatment of Lutembacher’s syndrome. Methods: 54 patients with Lutembacher’s syndrome treated in Wuhan Asian Heart Hospital from 2008 to 2015 were analyzed retrospectively. Results: 51 cases were classic Lutembacher’s syndrome,3 cases were acquired Lutembacher’s syndrome, with different degree of pulmonary hypertension, most of them were complicated with tricuspid insufficiency, and a few cases were complicated with aortic stenosis and insufficiency. There are also very few patients with persistent left superior vena cava and infective endocarditis. ②50 cases were diagnosed by echocardiography, 4 cases were diagnosed by right cardiac catheterization and 2 cases were diagnosed intraoperatively. ③37 cases underwent surgical treatment and 1 case underwent interventional therapy, none of them died of operation, no severe heart failure, pulmonary hypertension crisis and malignant arrhythmia occurred. Conclusions: the sonographic features of Lutembacher’s syndrome are obvious, and echocardiography has specific diagnostic value. Early operation or interventional treatment should be performed to repair or block atrial septal defect and to relieve or alleviate mitral stenosis effectively. Exploration and treatment of combined tricuspid insufficiency can achieve satisfactory surgical result.