Research: Congenital Heart Disease With Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes

J Pediatr

. 2023 May 31;113530.

 doi: 10.1016/j.jpeds.2023.113530. Online ahead of print.

Congenital Heart Disease With Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes

Latoya A Stewart 1Rebecca R Hernan 2Christopher Mardy 3Eunice Hahn 3Wendy K Chung 2Emile A Bacha 4Ganga Krishnamurthy 2Vincent P Duron 5Usha S Krishnan 6

Affiliations expand


Objectives: 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.

Study design: 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 two groups based on survival at discharge.

Results: 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 non-survivors compared with survivors. Non-survivors were more likely to have unrepaired CDH (69% vs 0%, p<0.001) and unrepaired CHD (88% vs 54%, p<0.05), reflecting a decision not to offer surgery.

Conclusions: 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 post-natal 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 of age with follow-up at a large pediatric and cardiothoracic surgical center.

Keywords: cardiac and thoracic surgery; genetics; outcomes; prenatal counseling.

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