ABSTRACT
Congenital diaphragmatic hernia (CDH) is marked by varying degrees of pulmonary hypoplasia, abnormal pulmonary vasculature, and disturbed transition to extra-uterine life. Prenatal ascertainment of severity, using ultrasound and magnetic resonance imaging, has markedly improved the ability of providers to predict risk for extracorporeal membrane oxygenation (ECMO), prolonged hospitalization and mortality. The post-natal management is dependent on the cardio-respiratory adaptation, with a spectrum of disease going from minimal respiratory support to severe hypoxic respiratory failure. Adoption of a standardized protocol of care, including minimization of stimulation, use of pre-ductal saturation for saturation targets, gentle ventilation and a step-wise (less aggressive) approach to pulmonary vasodilators and inotropic support, has led to decreased ECMO use and improved survival. The understanding regarding the pathophysiology of pulmonary hypertension in CDH is constantly evolving and an adequate evaluation of the underlying cardiovascular physiology is critical when choosing therapeutic agents to support cardiac function. The optimal timing for surgical repair of the diaphragmatic defect is still under debate. This chapter addresses the most current knowledge on prenatal evaluation, concepts regarding post-natal targeted cardiovascular management and controversies regarding closure of the diaphragm.
