先天性主动脉瓣下狭窄的外科治疗

目的探讨先天性主动脉瓣下狭窄（congenital subvalvular aortic stenosis，CSAS）的临床特点及手术疗效。方法全组32例，男16例，女16例。年龄4～57岁，平均年龄31岁；纤维隔膜型24例，纤维肌束型6例，弥漫型2例；手术在体外循环下进行，单纯纤维隔膜切除24例，纤维隔膜切除并室间隔肌肉切除术7例，Kono术1例。主动脉瓣机械瓣置换术6例，主动脉瓣成形3例，主动脉瓣二尖瓣机械瓣替换1例，同期矫治其它合并畸形。结果术后无早期死亡。全部患者术后瓣下压差较术前显著下降，术前、后LVEDD，EF差异无显著性。全部病例随访，随访时间1～60个月。长期生存者，心功能恢复至Ⅰ级31例，Ⅱ级1例。结论CSAS一旦确诊，应尽早手术。手术关键要根据病理解剖特点彻底疏通左室流出道。

Surgical treatment of congenital subvalvular aortic stenosis

Objective To investigate the clinical features and surgical results of congenital subvalvular aortic stenosis.Methods 32 patients with congenital subvalvular aortic stenosis were treated surgically. Among them, there were 16 male patients and 16 female patients. The mean age was 31 years old (ranging from 4 to 57 in age). 24 patients had fibro membrane typical stenosis, 6 patients had fibromuscular band typical stenosis and 2 patients had diffuse typical stenosis. The operation was performed under extracorporeal circulation, Simple membranectomy was performed in 24 cases, membranectomy +myectomy was performed in 7 cases , and 1 patient was performed with Kono's operation. 6 patients were provided with aortic valve replacement. Valvulop1asty of aortic were performed in 3 patient, 1 patient was provided with aortic and mital valve replacement. Corrective therapies for other endocardial abnormality were performed during the same period.Results There were no cases of death in early phase of this group. There was significant difference between the pressure under the value before the operation and after operation. There was no significant difference in left ventricular end-diastolic diameter(LVD) or left ventricular ejection fraction (EF)(all P>0.05). All cases were followed up after surgery from 1 to 60 months. During the period the cardiac function was restored to classⅠin 31patients, and to classⅡin 1 patient.Conclusion Once congenital subvalvular aortic stenosis is fully diagnosed, operation shall be as carly as possible. The key to the operation is that left ventricular outflow tract should be thoroughly dredged according to the pathology and anatoruical characteristic.