Surgical Approaches in Congenital Heart Disease With Congenital Diaphragmatic Hernia: A Multiinstitutional Analysis

Abstract

Background: Surgical decision making for congenital heart disease (CHD) with concomitant congenital diaphragmatic hernia (CDH) remains a notable challenge. This study analyzed the relationship between surgical timing and outcomes for patients with both CDH and CHD (CDH + CHD).

Methods: A retrospective analysis of patients with CDH + CHD was performed using data from the multiinstitutional CDH Study Group registry (2007-2022). CDH was categorized by defect size (A-D, smallest to largest) and cardiac anomalies stratified by Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category. Multivariable regression models and Loess smoothing analyses were used, focusing on patient mortality as a function of defect size and surgery timing.

Results: Among 9261 patients with CDH, 1886 had CDH + CHD, and 209 (11.1%) underwent both cardiac and diaphragm repair. A total of 94.3% (n = 197) underwent diaphragmatic repair before the cardiac operation, and STAT category distribution was as follows: 1, 30.1%; 2, 27.3%; 3, 15.8%; 4, 20.1%; and 5, 6.7%. Overall mortality was 23.4%. Multilevel mix-effects logistic regression identified extracorporeal life support as a significant predictor of in-hospital mortality (odds ratio, 5.74; P = .001). When stratified by STAT category, patient mortality correlated with CDH Study Group stage. Median time between operations was 46 days (survivors, 51; nonsurvivors, 39; P = .20) and varied by STAT category. Survival after cardiac operations was greatest 30 to 80 days after CDH repair.

Conclusions: CHD operation outcomes are influenced by CDH size or severity and STAT category risk. CDH repair is almost universally completed before a CHD operation, with the most selected timing for CHD operations occurring 30 to 80 days after CDH repair. These data provide insight into current practice and evidence to guide surgical decision making strategies for patients with CDH + CHD.