Belli E, Lacour-Gayet F, Serraf A, Alkhulaifi A M, Touchot A, Bruniaux J, Planché C
Department of Pediatric Cardiac Surgery, Paris-Sud University, Marie Lannelongue Hospital, Le Plessis-Robinson, France.
Eur J Cardiothorac Surg. 1999 Jul;16(1):14-20. doi: 10.1016/s1010-7940(99)00132-3.
The presence of associated multiple ventricular septal defects (VSDs) increases the risk of the anatomic repair for transposition of the great arteries (TGA). The aim of this study was to define the optimal management of this complex anomaly.
Between January 1988 and December 1998, 45 patients underwent anatomic repair of TGA associated with multiple VSDs. The median age was 50 days and the median weight 4 kg. Eighteen (40%) had undergone previous palliation including 17 pulmonary artery banding procedure (PAB), seven associated with coarctation repair and one isolated coarctation repair. The perimembraneous septum was involved in 24 patients, the trabecular in 43, the inlet in seven and the infundibular in two. Closure of the VSDs included Dacron or pericardial patchs and matress sutures. The initial approach was through right atriotomy which was sufficient in 15 patients. VSDs were closed through right ventriculotomy in 13 patients, through pulmonary artery in six, through the aorta in one and in the remaining (n = 10) combined approaches were used. Only one patient required left apical ventriculotomy.
There were five hospital deaths (11%; 70% CL: 6-18%) including the one early reoperation for residual VSD closure. Five patients had successful early reoperation for secondary PAB for residual VSD. Three late deaths occurred (7%; 70% CL: 3-13%). At the last visit, 95% of survivors were asymptomatic and without any cardiac medication.
Mid-term survival with good quality of life can be achieved following either one or two-stage repair of this complex anomaly. In the presence of VSD closure failure a secondary PAB may be the procedure of choice.
合并多个室间隔缺损（VSD）会增加大动脉转位（TGA）解剖修复的风险。本研究旨在明确这种复杂异常的最佳治疗方法。
1988年1月至1998年12月期间，45例患者接受了合并多个VSD的TGA解剖修复术。中位年龄为50天，中位体重为4千克。18例（40%）曾接受过姑息治疗，其中17例行肺动脉环扎术（PAB），7例合并缩窄修复，1例单纯缩窄修复。24例患者的膜周部间隔受累，43例小梁部受累，7例流入道受累，2例漏斗部受累。VSD的闭合包括使用涤纶或心包补片及褥式缝合。初始入路为右心房切开术，15例患者采用此方法即可。13例患者通过右心室切开术闭合VSD，6例通过肺动脉，1例通过主动脉，其余10例采用联合入路。仅1例患者需要左心尖心室切开术。
有5例医院死亡（11%；70%可信区间：6 - 18%），其中1例因残余VSD闭合而早期再次手术。5例患者因残余VSD成功早期再次行继发性PAB手术。发生3例晚期死亡（7%；70%可信区间：3 - 13%）。在最后一次随访时，95%的幸存者无症状且无需任何心脏药物治疗。
对这种复杂异常进行一期或二期修复后可实现中期生存且生活质量良好。若VSD闭合失败，继发性PAB可能是首选手术。
