Oladunjoye Olubunmi, Piekarski Breanna, Baird Christopher, Banka Puja, Marx Gerald, Del Nido Pedro J, Emani Sitaram M
Department of Cardiovascular Surgery, Boston Children's Hospital, Boston, Mass; Department of Internal Medicine, Reading Hospital, Reading, Pa.
Department of Cardiovascular Surgery, Boston Children's Hospital, Boston, Mass.
J Thorac Cardiovasc Surg. 2020 Jan;159(1):254-264. doi: 10.1016/j.jtcvs.2019.06.120. Epub 2019 Aug 30.
Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. This study aimed to describe the clinical outcomes of biventricular (BiV) repair for DORV.
Patients with DORV, who underwent BiV repair between January 2000 and December 2017 were retrospectively reviewed. Group 1 underwent primary BiV repair, whereas group 2 underwent staged BiV repair over a series of operations. The decision to pursue staged approach included complexity of intracardiac anatomy, age of the patient, and the size and function of the ventricles and the atrioventricular valves. Time-dependent surgical reintervention for LVOTO and mortality were evaluated using Kaplan-Meier survival analysis.
A total of 238 patients with DORV underwent BiV repair at a median age of 6.2 months (range, 1.1 month-27.5 years) (158 in group 1, 80 in group 2). Twenty-two patients (7.8%) required surgical reintervention within 30 days of BiV repair. Overall survival at 5 years was 89.0%. Freedom from LVOTO reoperation at 5 years was 84%. Primary outcomes were not significantly different between groups. CAVC repair and right ventricle to pulmonary artery conduit at BiV repair were associated with higher surgical reintervention (hazard ratio, 2.9 and 1.75, respectively).
Patients with DORV and complex anatomy may undergo staged BiV repair with acceptable outcomes. Although LVOTO is a potential complication in these patients, the rate of surgical reintervention for LVOTO does not differ significantly from patients undergoing primary BiV repair.
右心室双出口（DORV）是一种复杂的心脏畸形，具有多种解剖变异和多种手术修复方法。本研究旨在描述DORV双心室（BiV）修复的临床结果。
回顾性分析2000年1月至2017年12月期间接受BiV修复的DORV患者。第1组接受一期BiV修复，而第2组在一系列手术中接受分期BiV修复。采用分期手术的决定因素包括心内解剖结构的复杂性、患者年龄、心室及房室瓣的大小和功能。使用Kaplan-Meier生存分析评估左心室流出道梗阻（LVOTO）的时间依赖性手术再次干预和死亡率。
共有238例DORV患者接受了BiV修复，中位年龄为6.2个月（范围1.1个月至27.5岁）（第1组158例，第2组80例）。22例患者（7.8%）在BiV修复后30天内需要手术再次干预。5年总生存率为89.0%。5年无LVOTO再次手术率为84%。两组的主要结局无显著差异。BiV修复时的完全性房室通道（CAVC）修复和右心室至肺动脉管道与较高的手术再次干预相关（风险比分别为2.9和1.75）。
解剖结构复杂的DORV患者可接受分期BiV修复，结果可接受。虽然LVOTO是这些患者的潜在并发症，但LVOTO的手术再次干预率与接受一期BiV修复的患者相比无显著差异。
