Chapter 40 - Idiopathic Pulmonary Hypertension

Summary

Idiopathic pulmonary arterial hypertension is a subset of pulmonary hypertension in which progressive narrowing of pulmonary vasculature leads to an increase in pulmonary vascular resistance and eventual right ventricular failure. Survival and quality of life have significantly improved with the advent of targeted therapies that promote pulmonary vasodilation and improve right ventricular function. Children with pulmonary hypertension have a 20-fold higher incidence of perioperative cardiac arrest compared to the general pediatric population. A well-balanced, hemodynamically stable anesthetic that aims to avoid increases in pulmonary vascular resistance and decreases in ventricular function or coronary perfusion is crucial in preventing a pulmonary hypertensive crisis. The anesthetist must anticipate, rapidly recognize, and treat impending signs of pulmonary hypertensive crisis in order to safely anesthetize a child with pulmonary hypertension.