Abstract
The therapeutic approach to congenital diaphragmatic hernia (CDH) has shifted from one of immediate repair to management of pulmonary hypertension, physiologic stabilization, and delayed surgical repair. Lung hypoplasia, remodeled pulmonary vasculature, and ventricular dysfunction all contribute to the high morbidity and mortality associated with CDH. In addition, genetic syndromes associated with CDH can increase the incidence of serious anomalies and hence impact survival. Prenatal and postnatal management of infants with CDH is challenging in the best of circumstances and need multidisciplinary teams for optimal outcomes. However, advances using ultrasound and fetal MRI can predict prognosis and survival and plan for postnatal management. Survival rates for patients with CDH have increased for the past decade with better management at resuscitation; implementation of gentle ventilation strategies; and medical management of pulmonary hypertension, physiologic stabilization, and extracorporeal membrane oxygenation. However, follow-up of these infants for long-term morbidities is essential for optimal outcomes after discharge.