Murguia L Felipe, Ossa-Galvis Maria, Gaitonde Mansi, Fares Munes, Gaur Lasya, Andersen Nicholas, Ikemba Catherine
Heart Center, Children's Health, Division of Cardiology, Department of Pediatrics, UT Southwestern Medical Center, 1935 Medical District Dr, Dallas, TX, 75235, USA.
Heart Center, Children's Health, Division of Pediatric Cardiothoracic Surgery, Department of Cardiovascular and Thoracic Surgery, UT Southwestern Medical Center, 1935 Medical District Dr, Dallas, TX, 75235, USA.
Pediatr Cardiol. 2025 Aug 21. doi: 10.1007/s00246-025-03994-3.
Surgical advances have recently expanded the boundaries of feasibility for biventricular repair (BVR) in patients with complex congenital heart disease (CHD). In particular, diagnoses historically considered prohibitive for BVR-such as unbalanced atrioventricular septal defects (uAVSD), double-outlet right ventricles (DORV) with remote ventricular septal defects (VSD), atrioventricular valve straddle (AVVS), and heterotaxy-are now increasingly being managed with successful two-ventricle strategies. To determine whether fetal echocardiographic features once viewed as contraindications to BVR remain valid in the current surgical era, we retrospectively reviewed all patients evaluated by our institutional BVR committee from June 2023 to February 2025 with prenatally diagnosed uAVSD, DORV, or DORV-AVSD. Among the 23 patients selected for BVR, 16 had available fetal echocardiograms. Features historically associated with poor BVR candidacy were common, including RV outflow tract obstruction (56%), ventricular hypoplasia (44%), heterotaxy (25%), AVVS (13%), remote VSDs (25%), and pulmonary venous anomalies (19%). Despite these findings, all patients were managed with BVR strategies, with the majority achieving complete or staged biventricular circulation at the time of publication. Notably, patients deemed unsuitable for BVR were significantly older at time of referral, highlighting the potential role of fetal cardiology in early identification and timely referral. These findings suggest that many prenatal features long considered exclusionary are no longer barriers to BVR, underscoring the need to modernize fetal counseling frameworks, reflecting contemporary surgical capabilities. As the landscape evolves, fetal cardiologists must adapt their counseling strategies to offer families accurate prognostic information and renewed hope.
外科手术的进展最近扩大了复杂先天性心脏病（CHD）患者双心室修复（BVR）的可行性范围。特别是，历史上被认为对BVR具有禁忌性的诊断——如不平衡型房室间隔缺损（uAVSD）、伴有远离室间隔缺损（VSD）的右心室双出口（DORV）、房室瓣跨立（AVVS）和心脏异位——现在越来越多地采用成功的双心室策略进行治疗。为了确定曾经被视为BVR禁忌证的胎儿超声心动图特征在当前手术时代是否仍然有效，我们回顾性分析了2023年6月至2025年2月期间由我们机构的BVR委员会评估的所有产前诊断为uAVSD、DORV或DORV-AVSD的患者。在选定进行BVR的23例患者中，16例有可用的胎儿超声心动图。历史上与BVR候选不佳相关的特征很常见，包括右心室流出道梗阻（56%）、心室发育不全（44%）、心脏异位（25%）、AVVS（13%）、远离VSD（25%）和肺静脉异常（19%）。尽管有这些发现，但所有患者均采用BVR策略进行治疗，大多数患者在发表时实现了完全或分期双心室循环。值得注意的是，被认为不适合BVR的患者在转诊时年龄明显较大，这突出了胎儿心脏病学在早期识别和及时转诊中的潜在作用。这些发现表明，许多长期以来被视为排除标准的产前特征不再是BVR的障碍，强调了更新胎儿咨询框架的必要性，以反映当代的手术能力。随着情况的发展，胎儿心脏病学家必须调整他们的咨询策略，为家庭提供准确的预后信息和新的希望。
