Early Outcomes for Management of Atrioventricular Septal Defect-Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons' Society Study.

Connor P Callahan, Madison B Argo, Brian W McCrindle, David J Barron, Anusha Jegatheeswaran, Osami Honjo, Anastasios C Polimenakos, Joseph W Turek, Robert J Dabal, James K Kirklin, William M DeCampli, Pirooz Eghtesady, David M Overman
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Abstract

Background: We sought to determine the management and early outcomes of complete atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort.

Methods: Of 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching. Secondly, for patients with AVSD-TOF, we compared staged (n = 16) versus primary (n = 24) repair by assessing the following: patient characteristics, progression of atrioventricular valve (AVV) regurgitation, and time-related reoperation and survival.

Results: Five-year survival was similar between matched AVSD-TOF and isolated AVSD groups (80% vs 81%, P = .9). Compared with primary repair patients, staged patients had smaller pulmonary valve annulus Z-score measured at first presentation (-2.2 vs -2.9, P = .006). All staged patients (12 Blalock-Thomas-Taussig shunts, 3 right-ventricular-outflow-tract stents, 1 ductal stent) survived to complete repair. Freedom from AVSD-related reoperation five years post-AVSD-TOF repair was 57% after staged versus 90% after primary repair (P < .05) and left AVV reoperations were the most frequent reintervention. Survival five years after AVSD-TOF repair was 80% (63% after staged vs 90% after primary repair; P = .08).

Conclusions: Patients undergoing AVSD-TOF repair have similar survival compared with matched isolated AVSD patients. Although approximately half of AVSD-TOF patients had initial palliation and all survived to complete repair, staged repair patients had lower survival and a higher reintervention rate compared with primary repair patients. The decision to pursue staged versus primary repair for future babies with AVSD-TOF remains challenging and should be chosen based on individual circumstances.

过去十年中处理房室隔缺损-法洛四联症的早期疗效:先天性心脏病外科医生协会的一项研究。
背景:我们试图确定当代多中心队列中完全性房室间隔缺损-法洛四联症(AVSD-TOF)的管理和早期预后:在先天性心脏病外科医生协会 AVSD 队列(2012 年 1 月至 2021 年 5 月)的 739 名参与者中,有 40 人患有 AVSD-TOF。我们首先使用倾向匹配法比较了AVSD-TOF患者与孤立AVSD患者的生存率差异。其次,对于 AVSD-TOF 患者,我们通过评估患者特征、房室瓣(AVV)反流进展以及与时间相关的再手术和生存率,比较了分期修复(16 例)和初次修复(24 例):结果:匹配的 AVSD-TOF 组和孤立的 AVSD 组的五年生存率相似(80% vs 81%,P = .9)。与初次修复患者相比,分期患者初次就诊时测得的肺动脉瓣环Z-分数较小(-2.2 vs -2.9,P = .006)。所有分期患者(12 例 Blalock-Thomas-Taussig 分流、3 例右室流出道支架、1 例导管支架)均存活至完全修复。AVSD-TOF修复术后五年内,分期手术与AVSD相关再手术的成功率分别为57%和90%(P P = .08):结论:与匹配的孤立 AVSD 患者相比,接受 AVSD-TOF 修复术的患者存活率相似。结论:与匹配的孤立性 AVSD 患者相比,接受 AVSD-TOF 修复术的患者存活率相似。虽然约有一半的 AVSD-TOF 患者最初病情得到缓解,并且所有患者都存活到了完全修复,但与初次修复患者相比,分期修复患者的存活率较低,再次干预率较高。对于未来的 AVSD-TOF 婴儿来说,决定进行分期修复还是初次修复仍具有挑战性,应根据个人情况进行选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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