The Prevalence and Impact of Congenital Diaphragmatic Hernia Among Patients Undergoing Surgery for Congenital Heart Disease

Semin Thorac Cardiovasc Surg. 2019 Spring;31(1):69-77. doi: 10.1053/j.semtcvs.2018.09.014. Epub 2018 Sep 27.


There has not been a multicenter investigation to elucidate the prevalence and impact of congenital diaphragmatic hernia (CDH) on children undergoing cardiac surgery. We investigated the prevalence of CDH across congenital cardiac diagnostic and procedural groups and its impact on outcomes. The STS Congenital Heart Surgery Database was queried to identify children undergoing cardiac surgery who also had CDH. Baseline perioperative characteristics and postoperative outcomes were compared between groups. Subgroup analyses were performed based on case complexity as determined by STAT Categories. Overall 426 of 157,419 (0.27%) pediatric patients undergoing an index cardiac operation had a diagnosis of CDH including 89 neonates (0.25% of all neonatal index operations), 217 infants (0.39%), and 120 children (0.18%). The frequency of concomitant CDH varied across diagnostic groups and, in neonates and infants, was highest for tetralogy of Fallot (0.45%, n = 41), coarctation (0.39%, n = 27) and complete atrioventricular septal defects (0.31%, n = 19). For neonates and infants outcomes were generally worse in CDH vs no-CDH patients across the various procedural strata. For example, STAT ≥3 operative mortality was 34.4% vs 10.3% ( P < 0.001) for neonates, and 24.2% vs 5.3% ( P < 0.001) for infants with vs without CDH, respectively. In older children, outcomes disparities were less clear cut with no significant differences in mortality, and variable differences for complication and length of stay outcomes. CDH occurs in a small percentage of patients born with congenital heart disease. Concomitant diagnosis of CDH portends increased morbidity and mortality in infants and neonates undergoing cardiac surgery.

Keywords: Congenital diaphragmatic hernia; Congenital heart disease; Pediatrics.

Publication types

  • Multicenter Study

MeSH terms

  • Age Factors
  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / mortality
  • Child
  • Child, Preschool
  • Databases, Factual
  • Health Status
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery*
  • Hernias, Diaphragmatic, Congenital / diagnosis
  • Hernias, Diaphragmatic, Congenital / epidemiology*
  • Hernias, Diaphragmatic, Congenital / mortality
  • Hospital Mortality
  • Humans
  • Infant
  • Infant Mortality
  • Infant, Newborn
  • Length of Stay
  • North America / epidemiology
  • Postoperative Complications / mortality
  • Prevalence
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome