KEY POINTS

• The severity of congenital diaphragmatic hernia (CDH) can be estimated prenatally using observed-to-expected lung–head ratios (by ultrasound) and total fetal lung volumes (by magnetic resonance imaging), as well as fetal liver position.
• Infants with CDH require intensive cardiopulmonary support after birth, including immediate endotracheal intubation and “gentle ventilation,” as well as judicious fluid and inotropic support.
• Pulmonary hypertension, as assessed by echocardiography, may require the use of pulmonary vasodilators and other medical adjuncts (e.g., prostaglandin E1, milrinone) or, in severe cases, extracorporeal life support, if available.
• Open surgical repair of the diaphragmatic defect should usually be delayed until physiologic stability has been achieved, but a failure to perform surgery within the first two weeks of life should prompt a team discussion of priorities with the family.
• Infants with CDH should undergo long-term, multidisciplinary surveillance that includes standardized neurodevelopmental testing, especially if they are considered high risk (i.e., needing pulmonary support at 30 days, needing a patch repair or requiring extracorporeal life support).