Surgical approaches in Congenital Heart Disease with Congenital Diaphragmatic Hernia: A multi-institutional 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 CDH+CHD.

Methods: A retrospective analysis of patients with CDH+CHD was performed using data from the multi-institutional CDH study group registry (2007-2022). CDH was categorized by defect size (A-D, smallest-largest) and cardiac anomalies stratified by STAT category. Multivariable regression models and Loess smoothing analyses were utilized, focusing on patient mortality as a function of defect size and surgery timing.

Results: Among 9,261 CDH patients, 1,886 had CDH+CHD, and 209 (11.1%) underwent both cardiac and diaphragm repair. 94.3% (n=197) underwent diaphragmatic repair prior to cardiac operation, and STAT category distribution was: STAT1 (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 (OR=5.74, p=0.001). When stratified by STAT category, patient mortality correlated with CDHSG stage. Median time between operations was 46 days (survivors 51, non-survivors 39, (p=0.20)) and varied by STAT category. Survival following cardiac operations was greatest 30-80 days following CDH repair.

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