Li Shoujun, Ma Kai, Hu Shengshou, Hua Zhongdong, Yang Keming, Yan Jun, Chen Qiuming
Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
Pediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, People's Republic of China.
J Thorac Cardiovasc Surg. 2014 Sep;148(3):817-24. doi: 10.1016/j.jtcvs.2014.06.020. Epub 2014 Jun 13.
The study objective was to report the outcomes of biventricular repair in patients with double outlet right ventricle.
Patients with double outlet right ventricle who underwent biventricular repair at Fuwai Hospital from January 2005 to December 2012 were included. Patients were excluded if double outlet right ventricle was combined with atrioventricular septal defect, heterotaxy syndrome, atrioventricular discordance, or univentricular physiology.
A total of 380 consecutive patients with a mean age of 1.9 ± 2.1 years (range, 1 month to 6 years) were included. Varied types of biventricular repair were customized individually. Follow-up was 90.4% complete, and the mean follow-up time was 3.4 ± 3.9 years. There were 17 (4.5%) early deaths and 7 (2.1%) late deaths. Preoperative pulmonary hypertension was the only risk factor for early mortality. Postoperative significant left ventricular outflow tract obstruction was present in 9 survivors. Patients with noncommitted ventricular septal defect had a longer crossclamp time, longer cardiopulmonary bypass time, and higher incidence of postdischarge left ventricular outflow tract obstruction. There were 4 reoperations, all of which were caused by subaortic left ventricular outflow tract obstruction. All of the pressure gradients were decreased to less than 20 mm Hg after the modified Konno procedure with an uneventful postoperative course.
Optimal results of varied types of biventricular repair for double outlet right ventricle have been acquired. Although noncommitted ventricular septal defect is technically difficult, the outcomes of patients are favorable. Late-onset left ventricular outflow tract obstruction is the main reason for reoperation but can be successfully relieved by the modified Konno procedure.
本研究旨在报告右心室双出口患者双心室修复的结果。
纳入2005年1月至2012年12月在阜外医院接受双心室修复的右心室双出口患者。若右心室双出口合并房室间隔缺损、内脏异位综合征、房室不一致或单心室生理情况，则将患者排除。
共纳入380例连续患者，平均年龄为1.9±2.1岁（范围为1个月至6岁）。根据个体情况定制了不同类型的双心室修复术。随访完成率为90.4%，平均随访时间为3.4±3.9年。有17例（4.5%）早期死亡和7例（2.1%）晚期死亡。术前肺动脉高压是早期死亡的唯一危险因素。9例幸存者术后出现明显的左心室流出道梗阻。无室间隔缺损患者的主动脉阻断时间更长、体外循环时间更长，出院后左心室流出道梗阻的发生率更高。有4例再次手术，均由主动脉下左心室流出道梗阻引起。在改良Konno手术后，所有压力阶差均降至20 mmHg以下，术后过程平稳。
已获得右心室双出口不同类型双心室修复的最佳结果。尽管无室间隔缺损在技术上具有挑战性，但患者的预后良好。晚期左心室流出道梗阻是再次手术的主要原因，但可通过改良Konno手术成功缓解。
