Stephens Elizabeth H, Tingo Jennifer, Najjar Marc, Yilmaz Betul, Levasseur Stéphanie, Dayton Jeffrey D, Mosca Ralph S, Chai Paul, Quaegebeur Jan M, Bacha Emile A
1 Division of Cardiac, Thoracic, and Vascular Surgery, Columbia University Medical Center, New York, NY, USA.
2 Division of Cardiology, St Christopher's Hospital for Children, Philadelphia, PA, USA.
World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):189-195. doi: 10.1177/2150135116682719.
Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention.
We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014). A two-patch repair was used in all patients to correct the CAVC defect. Tetralogy of Fallot repair included transannular patch in 11 (65%) patients and valve-sparing in 6 (35%) patients.
The average age at surgery was 72 ± 122 weeks, 40% were male, and 80% had trisomy 21. Mean echo follow-up was 3.0 ± 3.0 years. There were no in-hospital or late mortalities. The rate of reoperation was 20%. At the latest follow-up, moderate left atrioventricular valve regurgitation was present in three (15%) patients and mild stenosis present in seven (35%) patients. One (5%) patient had moderate right ventricular outflow tract (RVOT) obstruction. The valve-sparing population was smaller at the time of surgery than the non-valve-sparing cohort (body surface area: 0.28 ± 0.04 vs 0.42 ± 0.11, P = .002) and less likely to have had a previous shunt (0% vs 64%, P = .01). Among the valve-sparing patients (six), at the latest follow-up, moderate pulmonary insufficiency was present in two (33%) patients.
Repair of CAVC concomitant with TOF can be performed with low mortality and acceptable perioperative morbidity. Management of the RVOT remains a challenge for the long term.
法洛四联症（TOF）合并完全性房室通道（CAVC）的修复手术是一项具有挑战性的手术，目前越来越多地在婴儿期进行完整的一期修复。以往的研究主要关注围手术期结果；然而，中期瓣膜功能、心室功能和残余梗阻情况很少受到关注。
我们回顾性分析了20例2005年1月至2014年12月期间接受CAVC/TOF修复手术的患者。所有患者均采用双片修补术纠正CAVC缺损。法洛四联症修复手术中，11例（65%）患者采用跨环补片，6例（35%）患者采用保留瓣膜术。
手术平均年龄为72±122周，40%为男性，80%患有21三体综合征。平均超声心动图随访时间为3.0±3.0年。无院内死亡或晚期死亡病例。再次手术率为20%。在最近一次随访时，3例（15%）患者出现中度左房室瓣反流，7例（35%）患者出现轻度狭窄。1例（5%）患者出现中度右心室流出道（RVOT）梗阻。手术时保留瓣膜组的患者比非保留瓣膜组患者体型小（体表面积：0.28±0.04 vs 0.42±0.11，P = 0.002），且较少有既往分流史（0% vs 64%，P = 0.01）。在保留瓣膜的患者（6例）中，最近一次随访时，2例（33%）患者出现中度肺动脉瓣关闭不全。
CAVC合并TOF的修复手术死亡率低，围手术期发病率可接受。RVOT的管理仍是长期面临的挑战。
