Objective To evaluate the efficacy of 3-dimensional printing model (3DPM) aiding decision making and surgery rehearsal for the treatment of double outlet right ventricle (DORV) with non-committed ventricular septal defect (NC-VSD). Methods From January 1st, 2012 through December 30th, 2014, 12 patients with DORV and NC-VSD were operated with the aid of “3DPM guidance” to do decision making and surgical technique rehearsal preoperatively. There were 9 males and 3 females at age of 2.9±2.2 years. The “3DPM guidance” consisted of step by step procedures: computerized tomography (CT) scan for the patients, CT based 3DPM rendering, 3DPM exploration, decision making, and surgery rehearsal. During surgery rehearsal, surgeons did patch designing, VSD enlargement planning, muscle bundle resection etc. Eight out of the twelve patients underwent biventricular repair, 4 patients underwent single ventricle repair. Six of the eight biventricular repair patients had intra-ventricular baffle repair, 1 patient had intra-ventricular baffle repair and arterial switch procedure, 1 had modified Nikaidoh procedure. VSD enlargement was performed in all the patients in biventricular repair group. The reasons not to do a biventricular repair included very restrictive VSD, tricuspid attachments across the sub-aortic passway. Results The operation findings correlated well with the 3DPM in all the cases. There was no hospital death, no major complication. One patient had a mild sub-aortic stenosis and he was under close follow-up. There was no late death and reoperation. Surgeons involved were satisfied with the “3DPM guidance”. Conclusions 3-D printing model is an excellent way to help decision making for DORV with NC-VSD and can provide surgery simulation which decrease complication rate and help achieve good outcomes.
目的探讨罕见肺动脉瓣缺如综合征（absent pulmonary valve syndrome，APVS）的外科治疗效果。 方法回顾性分析3例APVS患者在低温体外循环下行根治手术，其中女2例、男1例，年龄分别为9个月、14岁、10岁，其中2例合并右室双出口（double outlet right ventricle，DORV）。3例均采用自体心包片重建肺动脉瓣，1例行肺动脉成形术，2例未处理肺动脉。 结果3例均存活，术中食管超声及术后超声复查肺动脉瓣功能良好，随访8～24个月，均恢复良好、生活质量明显改善，复查心脏彩色超声心动图肺动脉瓣功能良好，无明显反流。 结论APVS是一种少见的先天性心脏病，合并DORV或心内其他结构正常的病例非常罕见，手术治疗可获得良好效果。
ObjectiveTo explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD).MethodsClinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time.ResultsIn the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001).ConclusionThe effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.