Comprehensive Approach to the Management of Patients With Hypoplastic Left Heart Syndrome: Analysis of 100 Consecutive Neonates.

IF 3.6 2区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Annals of Thoracic Surgery Pub Date : 2025-01-01 Epub Date: 2024-05-28 DOI:10.1016/j.athoracsur.2024.05.010

Mark Steven Bleiweis, Jennifer Co-Vu, Joseph Philip, James C Fudge, Himesh V Vyas, Andrew D Pitkin, Gregory M Janelle, Kevin J Sullivan, Curt J DeGroff, Dipankar Gupta, John-Anthony Coppola, Biagio Bill A Pietra, Frederick Jay Fricker, Susana C Cruz Beltrán, Giles J Peek, Jeffrey Phillip Jacobs

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Abstract

Background: We report our comprehensive approach to the management of patients with hypoplastic left heart syndrome (HLHS) and describe our outcomes in 100 consecutive neonates.

Methods: We stratified 100 consecutive neonates (January 1, 2015 to September 1, 2023, inclusive) into 3 pathways. Pathway 1: 77 of 100 (77%) were standard risk and underwent an initial Norwood Stage 1. Pathway 2: 10 of 100 (10%) were high-risk with noncardiac risk factors and underwent an initial Hybrid Stage 1. Pathway 3: 13 of 100 (13%) were high-risk with cardiac risk factors: 10 underwent an initial Hybrid Stage 1 + Ventricular Assist Device insertion (HYBRID+VAD), and 3 were supported with prostaglandin as a planned bridge to primary cardiac transplantation.

Results: The overall 1-year mortality for the entire cohort of 100 patients was 9% (9 of 100). Pathway 1: Operative Mortality in Pathway 1 for the initial Norwood Stage 1 was 2.6% (2 of 77). Of the 75 survivors of Norwood Stage 1, 72 underwent successful Glenn, 2 underwent successful biventricular repair, and 1 underwent successful cardiac transplantation. Pathway 2: Operative Mortality in Pathway 2 for the initial Hybrid Stage 1 without VAD was 10% (1 of 10). Of 9 survivors of Hybrid Stage 1, 4 underwent successful cardiac transplantation, 1 died while awaiting cardiac transplantation, 3 underwent Comprehensive Stage 2 (with 1 Operative Mortality after Comprehensive Stage 2), and 1 underwent successful biventricular repair. Pathway 3: Of 10 patients supported with initial HYBRID+VAD in Pathway 3, 7 (70%) underwent successful cardiac transplantation and are alive today, and 3 (30%) died on VAD while awaiting transplantation. Median VAD support time was 134 days (range, 56-226 days). Of 3 patients who were bridged to transplant with prostaglandin, 2 underwent successful transplantation and 1 died while awaiting transplantation.

Conclusions: A comprehensive approach to the management of patients with HLHS is associated with an Operative Mortality after Norwood of 2.6% (2 of 77) and an overall 1-year mortality of 9% (9 of 100). Ten patients (10%) were stabilized with HYBRID+VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged waiting times.

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综合管理 HLHS 患者的方法：对 100 例连续新生儿的分析。

背景：我们报告了治疗左心发育不全综合征（HLHS）患者的综合方法，并描述了我们对 100 例连续新生儿的治疗结果：将 100 名连续新生儿（2015-2023 年）分为 3 个路径：路径(1)：77/100=77%为标准风险，接受初始诺伍德（第一阶段）治疗。路径(2)：10/100=10%为具有非心脏风险因素的高风险患者，接受初始混合1期治疗。路径(3)：13/100=13%为有心脏风险因素的高危人群：10人接受了最初的混合1期+心室辅助装置植入术（HYBRID+VAD），3人接受了初次移植：结果：一年死亡率=9/100=9%。路径(1)：初始诺伍德（1期）手术死亡率=2/77=2.6%。诺伍德（第一阶段）的 75 名幸存者中，72 人成功接受了格伦手术，2 人成功接受了格伦手术：72人成功接受了格伦手术，2人成功接受了双心室修复手术，1人成功接受了心脏移植手术。路径(2)：最初的混合1期（不含VAD）手术死亡率=1/10=10%。在 9 名混合（第一阶段）幸存者中，4 人成功进行了心脏移植手术：4人成功进行了心脏移植，2人在等待心脏移植期间死亡，3人进行了综合阶段2（1人死亡），1人成功进行了双心室修复。途径（3）：在10例HYBRID+VAD患者中：7/10=70%成功接受了心脏移植手术并存活至今，3/10=30%在等待移植期间死于VAD。中位 VAD 支持时间=134 天（范围=56-226）。(使用前列腺素桥接至移植的三名患者中有两名成功接受了移植，一名在等待移植期间死亡）。结论：采用综合方法治疗 HLHS 患者，诺伍德术后死亡率为 2/77=2.6%，一年总死亡率为 9/100=9%。10/100=10%的患者在等待移植期间使用 HYBRID+VAD 稳定了病情。在漫长的等待时间里，VAD有助于患者在等待名单上存活。

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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来源期刊

Annals of Thoracic Surgery 医学-呼吸系统

CiteScore

6.40

自引率

13.00%

发文量

1235

审稿时长

42 days

期刊介绍： The mission of The Annals of Thoracic Surgery is to promote scholarship in cardiothoracic surgery patient care, clinical practice, research, education, and policy. As the official journal of two of the largest American associations in its specialty, this leading monthly enjoys outstanding editorial leadership and maintains rigorous selection standards. The Annals of Thoracic Surgery features: • Full-length original articles on clinical advances, current surgical methods, and controversial topics and techniques • New Technology articles • Case reports • "How-to-do-it" features • Reviews of current literature • Supplements on symposia • Commentary pieces and correspondence • CME • Online-only case reports, "how-to-do-its", and images in cardiothoracic surgery. An authoritative, clinically oriented, comprehensive resource, The Annals of Thoracic Surgery is committed to providing a place for all thoracic surgeons to relate experiences which will help improve patient care.