Congenital diaphragmatic hernia: pathophysiology and pharmacologic support.

The incidence of death from congenital diaphragmatic hernia appears to be unchanged in recent years despite advances in resuscitation, transport, and ventilatory support. Bilateral lung hypoplasia and abnormal pulmonary vascular reactivity as developmental consequences of the defect appear to play a major role in the continued high mortality rate. Recent advances in pharmacologic support have further elucidated the mechanisms of ventilatory failure in these patients and may represent means of improving survival in the future.