Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.
Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.
World J Pediatr Congenit Heart Surg. 2022 Sep;13(5):664-675. doi: 10.1177/21501351221088030. Epub 2022 May 5.
Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.
一些患有左心发育不全综合征（HLHS）和 HLHS 相关畸形伴导管依赖性体循环的患者，行 Norwood 姑息性手术的风险极高。我们报告了我们全面的管理方法，旨在最大限度地提高生存率和优化供心的利用。
我们回顾了我们目前在单一中心的 83 例 HLHS 和 HLHS 相关畸形新生儿和婴儿的全部经验（2015-2021 年）。标准风险患者（n=62）接受初始 Norwood（第 1 阶段）姑息性手术。高风险患者（除了主要心脏危险因素外，有其他危险因素，n=9）接受初始 Hybrid Stage 1 姑息性手术，包括双侧肺动脉环扎术、动脉导管支架置入术和需要时行房间隔切开术。有主要心脏危险因素的高风险患者（n=9）接受初始联合 Hybrid Stage 1 姑息性手术和搏动性心室辅助装置（VAD）置入术（HYBRID+VAD）桥接移植。3 例患者通过前列腺素桥接移植。
1 年总生存率为 90.4%（75/83）。行初始 Norwood（第 1 阶段）手术的标准风险患者的手术死亡率为 2/62（3.2%）。60 例存活者中：57 例行 Glenn 手术，2 例行双心室修复术，1 例行心脏移植术。无 VAD 初始 Hybrid Stage 1 姑息性手术的高风险患者（除了主要心脏危险因素外，有其他危险因素）的手术死亡率为 0/9：4 例行移植，1 例等待移植，3 例行全面第 2 阶段手术（其中 1 例死亡），1 例行双心室修复术。9 例 HYBRID+VAD 患者中，6 例（67%）成功进行心脏移植并存活至今，3 例（33%）在等待 VAD 期间死亡。VAD 中位支持时间为 134 天（均值=134，范围=56-226）。
HLHS 或 HLHS 相关畸形患者的综合管理方法与 Norwood 术后的手术死亡率为 2/62=3.2%和 1 年生存率为 75/83=90.4%相关。83 例患者中有 9 例（11%）接受 HYBRID+VAD 稳定后等待移植。VAD 可在长时间等待期间促进在等待名单上的生存。
