Anesthetic management of children with pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is associated with significant perioperative risk for major complications, including pulmonary hypertensive crisis and cardiac arrest. Several mechanisms of hemodynamic deterioration, including acute increases in pulmonary vascular resistance (PVR), alterations of ventricular contractility and function and coronary hypoperfusion can contribute to morbidity. Anesthetic drugs exert a variety of effects on PVR, some of which are beneficial and some undesirable. The goals of balanced and cautious anesthetic management are to provide adequate anesthesia and analgesia for the surgical procedure while minimizing increases in PVR and depression of myocardial function. The development of specific pulmonary vasodilators has led to significant advances in medical therapy of PAH that can be incorporated in anesthetic management. It is important that anesthesiologists caring for children with PAH be aware of the increased risk, understand the pathophysiology of PAH, form an appropriate anesthetic management plan and be prepared to treat a pulmonary hypertensive crisis.