Columbia University Vagelos College of Physicians and Surgeons, New York, NY.
Department of Pediatrics, Columbia University Irving Medical Center, New York, NY.
J Pediatr. 2023 Sep;260:113530. doi: 10.1016/j.jpeds.2023.113530. Epub 2023 May 31.
To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making and outcomes based on complexity of CHD and associated conditions.
A retrospective review of patients with CHD and CDH diagnosed by echocardiogram between 01/01/2005 and 07/31/2021. The cohort was divided into 2 groups based on survival at discharge.
Clinically important CHD was diagnosed in 19% (62/326) of CDH patients. There was 90% (18/20) survival in children undergoing surgery for both CHD and CDH as neonates, and 87.5 (22/24) in those undergoing repair initially for CDH alone. A genetic anomaly identified on clinical testing was noted in 16% with no significant association with survival. A higher frequency of other organ system anomalies was noted in nonsurvivors compared with survivors. Nonsurvivors were more likely to have unrepaired CDH (69% vs 0%, P < .001) and unrepaired CHD (88% vs 54%, P < .05), reflecting a decision not to offer surgery.
Survival was excellent in patients who underwent repair of both CHD and CDH. Patients with univentricular physiology have poor survival and this finding should be incorporated into pre and postnatal counseling about eligibility for surgery. In contrast, patients with other complex lesions including transposition of the great arteries have excellent outcomes and survival at 5 years follow-up at a large pediatric and cardiothoracic surgical center.
描述大型医疗中心中先天性膈疝（CDH）患者合并先天性心脏病（CHD）的类型，并基于 CHD 的复杂性和相关合并症评估手术决策和结局。
对 2005 年 1 月 1 日至 2021 年 7 月 31 日期间经超声心动图诊断为 CHD 和 CDH 的患者进行回顾性研究。根据出院时的生存情况，将队列分为两组。
在 CDH 患者中，临床重要的 CHD 诊断率为 19%（62/326）。在新生儿期同时接受 CHD 和 CDH 手术的患儿中，有 90%（18/20）存活，而仅接受 CDH 修复的患儿中，有 87.5%（22/24）存活。在临床检查中发现 16%的患儿存在遗传异常，但与生存率无显著相关性。与幸存者相比，非幸存者其他器官系统异常的发生率更高。非幸存者更有可能存在未修复的 CDH（69% vs 0%，P < 0.001）和未修复的 CHD（88% vs 54%，P < 0.05），这反映了不进行手术的决策。
同时修复 CHD 和 CDH 的患者生存率非常高。具有单心室生理结构的患者生存率较差，这一发现应纳入关于手术资格的产前和产后咨询中。相比之下，具有大动脉转位等其他复杂病变的患者具有极好的结局和生存率，这是在大型儿科和心胸外科中心进行 5 年随访得出的。
