Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: a report from the Congenital Diaphragmatic Hernia Study Group

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Background/Purpose Patients with congenital diaphragmatic hernia (CDH) are known to have associated cardiac anomalies. Data from the Congenital Diaphragmatic Hernia Study Group has allowed better definition of the types of heart defects (HDs) and survival for these patients. Methods Since 1995, 2636 patients were enrolled in the Congenital Diaphragmatic Hernia Study Group from 82 centers. Patients with hemodynamically significant HD, excluding patent ductus arteriosus (PDA); patent foramen ovale (PFO); and atrial septal defect (ASD), were selected. Cardiac anatomy and survival data for all patients were reviewed. Results Two hundred eighty (10.6%) patients had significant HDs: ventricular septal defect (VSD); (42.2%), aortic arch obstruction (15%), univentricular anatomy (13.9%), tetralogy of Fallot variants (11.1%), total anomalous pulmonary venous return (3.9%), double outlet right ventricle (RV) (3.2%), pulmonary stenosis (2.5%), transposition of the great arteries (2.5%), and various other defects in 5.7%. Survival for the entire group was 67.1%; survival for patients without HD was 70.2% and for patients with HD was 41.1% ( P < .001). Patients with biventricular cardiac anatomy had a 47% survival, whereas those with univentricular anatomy had a 5% survival ( P < .001). Conclusion Significant HD is associated with 10.6% of CDH. Survival for patients with HD is significantly lower than for patients with normal cardiac anatomy. Patients with CDH and univentricular cardiac anatomy have a poor prognosis.