Zhu Diqi, Bryant Roosevelt, Heinle Jeffrey, Nihill Michael R
Section of Pediatric Cardiology, Texas Heart Center at Texas Children's Hospital and Baylor College of Medicine, Houston, Texas 77030, USA.
Tex Heart Inst J. 2009;36(6):553-6.
We have reviewed the clinical presentations, courses, and outcomes of 90 patients seen at Texas Children's Hospital from 1983 through 2008 who had an isolated cleft of the mitral valve without some form of endocardial cushion defect. Additional congenital cardiac defects were present in 61 of the 90 patients, 35 of whom had a congenital syndrome. Seven patients had isolated cleft of the mitral valve without other intracardiac defects, and in these 7 there was a progressive increase in the degree of mitral regurgitation during a median time of 26.5 months from diagnosis to surgery. The patients ranged from a gestational age of approximately 32 weeks to 21.9 years of age. No death was observed among the 39 surgical patients, including 32 who had additional cardiac defects. There was a significant reduction in the degree of mitral regurgitation in all patients who underwent surgery. Among the 51 patients who did not have surgery, the degree of regurgitation did not change significantly over the course of 1 to 27 years' observation. Isolated cleft of the mitral valve is an uncommon (but not rare) congenital malformation of the mitral valve that can cause all degrees of mitral regurgitation but can be managed medically or surgically.
我们回顾了1983年至2008年在德克萨斯儿童医院就诊的90例患者的临床表现、病程及转归，这些患者患有单纯二尖瓣裂缺，无某种形式的心内膜垫缺损。90例患者中有61例存在其他先天性心脏缺陷，其中35例患有先天性综合征。7例患者有单纯二尖瓣裂缺，无其他心内缺损，这7例患者从诊断到手术的中位时间为26.5个月，二尖瓣反流程度呈进行性加重。患者的孕周约为32周，年龄从32周到21.9岁不等。39例接受手术的患者中无死亡病例，包括32例有其他心脏缺陷的患者。所有接受手术的患者二尖瓣反流程度均显著降低。在51例未接受手术的患者中，反流程度在1至27年的观察期内无显著变化。单纯二尖瓣裂缺是一种不常见（但并非罕见）的二尖瓣先天性畸形，可导致不同程度的二尖瓣反流，但可通过内科或外科治疗。
