Single Ventricle Physiology May Not Preclude Cardiac Repair in the Setting of Congenital Diaphragmatic Hernia

Objective: Management for congenital heart disease (CHD) with congenital diaphragmatic hernia (CDH), particularly in single ventricle physiology, is challenging, with limited studies showing poor outcomes. This investigation evaluated outcomes for these complex patients. Methods: This retrospective cohort study (2007-2022) from the CDH Study Group analyzed mortality in patients with CDH and single ventricle physiology, using standard statistical methods and Cox regression for estimation. Results: A total of 9,261 CDH patients were identified, including 1,886 with CDH + CHD and 131 with single ventricle physiology. The median gestational age was 38 weeks and birthweight was 2.8 kg. Of 131 patients, 34 (26.0%) had chromosomal anomalies, 20/131 (15.3%) received extracorporeal life support (ECLS), 53 underwent diaphragm repair (A: 5/53 [9.4%], B: 16/53 [30%], C: 23/53 [43.4%], D: 9/53 {17%}), and 21 received cardiac palliation (STAT 2: 1/21 [4.8%], STAT 4: 8/21 [38.1%], STAT 5: 12/21 [57.1%]). Mortality was 71.0% (93/131), highest (74/77, 96.2%) in patients not receiving diaphragm or cardiac repair, and lowest (5/20, 25.5%) in those receiving both. Mortality increased with defect size, reaching 20.0% (1/5) for STAT 4 and 57.1% (4/7) for STAT 5 in type C defects, and 100.0% for STAT 4 in type D defects, with no type D patients undergoing STAT 5. Unadjusted analyses found Apgar scores, length of stay, diaphragmatic repair, CDHSG stage, and cardiac palliation (all P < .05) associated with mortality, while Cox regression identified ECLS (hazard ratio [HR] = 10.83) and chromosomal anomalies (HR = 19.08) as significant mortality factors. Conclusions: This study underscores the variability in mortality outcomes for patients with single ventricle physiology and CDH, emphasizing the crucial role of surgical management and identifying key mortality predictors to optimize patient outcomes.