主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

Surgical treatment of congenital quadricuspid aortic valve associated with aortic valve dysfunction

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.

Objective To explore the effect of congenital quadricuspid aortic valve（QAV） on aortic valve function, and to evaluate the long-term outcomes of surgical treatment for QAV associated with aortic valve dys- function. Methods From January 2000 to January 2013, 13 patients with QAV were diagnosed by transtho- racic echocardiography or pathology. Four cases had normal aortic valve function, and 9 cases had aortic valve dysfunction. Among these 9 patients, seven cases had severe aortic valve regurgitation, and two cases had se- vere aortic valve regurgitation combined with stenosis. Moderate mitral regurgitation was in two cases, and moderate to severe tricuspid regurgitation was in one case. Results Four cases with normal aortic valve function continue to follow up. Nine cases with aortic valve dysfunction underwent aortic valve replacement. At the same time, mitral valve plasty was performed in two cases, and tricuspid valve plasty was performed in one case. There was no serious complications or early death in perioperation. At a median follow-up of （6.34±5.17）years, aortic valve function was normal in all patients, and no late death was observed. Conclusion Quadricuspid aortic valve is rare, and the echocardiography plays an important role in diagnosing. Follow-up is enough when aortic valve function is normal, but aortic valve replacement is necessary when aortic valve is dysfunctional. The long-term out- comes of surgical treatment are satisfy.