完全性心内膜垫缺损并发右室流出道狭窄的外科治疗

Surgical treatment for patients with total endocardial cushion defect and right ventricular outflow tract obstruction

目的：对近10年来26例完全性心内膜垫缺损（TECD）并发右室流出道狭窄（PS）患者进行了外科手术并进行了随访，以了解不同手术方式的效果。方法： 2003年11月-2012年12月共有26例TECD＋各种PS患者接受了外科手术，男10例，女16例，年龄3个月-24（8±7）岁，体质量6-52（22±14） kg。手术方法有双心室矫治、单心室矫治和姑息手术。随访依据门诊复查结果（包括病史、体格检查、心电图、X线胸片和超声心动图检查）及电话随访。结果： 26例患儿双心室矫治12例（46%），单心室矫治9例（35%），姑息手术5例（19%）。近期死亡9例（35%），住院生存患儿17例，随访期限为术后5-84（平均12.8）个月。晚期死亡1例（4%）。结论： TECD并发各种类型的PS，手术复杂，手术近期死亡率高，但中远期生存率较好。在同等条件下，双心室矫治较单心室矫治及姑息手术远期预后更好。

AIM：To investigate the postoperative early and mid-term effect of different surgical methods for patients with total endocardial cushion defect and right ventricular outflow tract obstruction. METHODS： Twenty-six patients with total endocardial cushion defect with various right ventricular outflow tract stenosis underwent surgery from November 2003 to December 2012, of whom ten were males and 16 females, aged 3 months-24 years old （7.9±6.7） years and weighing 6-52（22.3±14.5） kg. Surgical approaches carried out were biventricular correction, single ventricle correction and palliative surgery. Follow-up included out-patient review （case history, physical examination, electrocardiogram, chest X-ray and echocardiography） and telephone survey. RESULTS： Among the 26 cases, 12（46.2%） underwent biventricular correction, 9（34.6%） single ventricle correction and five （19.2%） palliative surgery. Nine cases （34.6%） of early death occurred and the other 17 cases survived during hospitalization. The follow-up period after surgery was 5-84 months （mean 12.8 months） and one case （3.8%） of late death occurred. CONCLUSION： Patients with total endocardial cushion defect and various types of right ventricular outflow tract obstruction have a low natural survival rate. The surgical procedure is complex and has a very high early death rate for atrioventricular valve regurgitation of any degree, regardless of the surgical approach. However, surgical procedures have a relatively better long-term survival rate. Compared with single ventricle correction and palliative surgical treatment, biventricular correction has a relatively better long-term prognosis.