主动脉瓣四叶瓣畸形的外科诊治

目的 提高对主动脉瓣四叶瓣畸形(QAV)的认识,总结术前超声心动图检查的特殊表现,探讨外科治疗的方法及疗效.方法 回顾性分析上海第二军医大学附属长海医院胸心外科2000年1月至2008年12月的766例主动脉瓣手术患者中11例QAV患者的临床资料,其中男9例,女2例,年龄4～55(32±16)岁.结果 本组11例患者占主动脉瓣手术患者的1.4%;其中合并感染性心内膜炎、左上腔静脉、升主动脉瘤、二尖瓣脱垂、三尖瓣关闭不全各1例;术前超声心动图检查明确诊断7例,误诊为单叶畸形1例,误诊为二叶畸形1例,误诊为风湿性改变者2例;术中发现B型7例,A型2例,F型1例,G型1例;11例患者均行主动脉瓣置换术(AVR),同时行主动脉根部拓宽术1例,升主动脉成形术1例,二尖瓣成形术(后瓣矩形切除+"C"成形环术)、三尖瓣成形术(Kay法成形术)各1例.结论 QAV在临床上非常罕见,术前超声心动图检查有助于明确诊断,但有时也存在一定的漏诊率;AVR是治疗QAV的有效措施.

Surgical treatment of congenital quadricuspid aortic valve

Objective To improve the understanding of congenital quadricuspid aortic valve ( QAV), explore its echocardiographic diagnostic value and summarize the methods and outcomes of surgical treatments. Methods The clinical data of 11 QAV patients from January 2000 to December 2008 were retrospectively analyzed. There were 9 males and 2 females with a mean operative age of (32 ± 16) years (range: 4-55 ). Results In 766 patients undergoing aortic valve surgery, 11 were of congenital quadricuspid aortic valve ( 1.4% ); cardiac pathology: infective endocarditis ( n = 1 ), left superior vena cava ( n = 1 ), aortic aneurysm ( n = 1 ), mitral prolapse ( n = 1 ) and tricuspid insufficiency ( n = 1 ). The patients of congenital QAV deformity was diagnosed by echocardiography ( n = 7 ), misdiagnosed as single valve ( n = 1 ), misdiagnosed as bicuspid valve (n = 1 ) and misdiagnosed as rheumatic heart disease ( n =2 ). Type B ( n = 7 ), Type A ( n = 2), Type F ( n = 1 ) and Type G ( n = 1 ). Eleven patients underwent the procedure of aortic valve replacement. And the concomitant procedures were aortic root broadening ( n = 1 ),ascending aortoplasty ( n = 1 ), mitral valvuloplasty ( n = 1 ) and tricuspid valvuloplasty ( n = 1 ).Conclusion Quadricuspid aortic valve is rare in clinical practice. And echocardiography plays an important diagnostic role. Surgical replacement of aortic valve is the first-line therapy for these patients.