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Congenital Heart Disease with Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes
Latoya A Stewart1, Rebecca R Hernan2, Christopher Mardy3
1Columbia University Vagelos College of Physicians and Surgeons, New York, NY.
Insights
Congenital heart disease (CHD) affects 19% of congenital diaphragmatic hernia (CDH) patients. Surgical repair of both conditions leads to excellent survival, guiding future treatment and counseling.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Neonatal Medicine
Background:
- Congenital diaphragmatic hernia (CDH) is a serious condition often associated with congenital heart disease (CHD).
- Understanding the spectrum of CHD in CDH patients is crucial for surgical decision-making and prognostication.
- Outcomes can vary significantly based on the complexity of CHD and associated anomalies.
Purpose of the Study:
- To characterize the types of CHD in a cohort of CDH patients.
- To evaluate surgical strategies and outcomes in relation to CHD complexity and co-occurring conditions.
- To inform pre- and postnatal counseling for families affected by CDH and CHD.
Main Methods:
- Retrospective review of patients diagnosed with both CHD and CDH between January 1, 2005, and July 31, 2021.
- Echocardiogram data used for diagnosis.
- Cohort divided into survival and non-survival groups at discharge for comparative analysis.
Main Results:
- Clinically significant CHD was present in 19% of CDH patients.
- Survival was high (90%) for neonates undergoing simultaneous repair of CHD and CDH.
- Nonsurvivors more frequently had unrepaired CDH (69%) and CHD (88%), indicating surgical decision impacts outcomes.
Conclusions:
- Simultaneous surgical repair of CHD and CDH in neonates yields excellent survival rates.
- Patients with univentricular physiology have poor survival, necessitating careful counseling.
- Complex lesions like transposition of the great arteries have favorable outcomes with timely intervention.
Objective:
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 2 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 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.
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 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.
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