Pulmonary hypertension

Pulmonary hypertension is a formidable challenge to the care of children with congenital heart disease, with a burgeoning incidence in the perioperative period. Awareness of this problem, prospective clinical pathway development, and focused expertise are all needed to optimally equip multidisciplinary teams to meet this challenge. Here we present insights into epidemiology, costs, anatomy, pathophysiology, medical diagnostics, and pharmacotherapies involved in treating pulmonary hypertension in pediatric congenital heart disease patients. The emphasis is on identifying patients at risk, choosing appropriate diagnostics, optimizing perioperative outcomes, timely recognition and management of pulmonary hypertensive crisis, the molecular basis of pharmacotherapy, and guidelines for treatment. Anticipatory guidance is also provided for transferring children from hospital to home, and frontiers of research in the field are considered.