Pulmonary Hypertension Associated with Congenital Heart Disease: A Practical Review for the Pediatric Cardiologist

Pulmonary hypertension (PH) is a complex condition with a broad range of etiologies that result in a common outcome—elevated pulmonary arterial pressure. For the pediatric cardiologist, this entity provides a manifest demonstration of the interrelationship of cardiac and pulmonary physiology. A thoughtful approach must be applied to each patient with PH in order to achieve a thorough understanding of the nature of disease and to proceed with individualized therapy in an appropriate and timely manner. This article aims to provide a clinically relevant, practical review of current literature related to PH in patients with congenital heart disease, including the causes, diagnostic considerations, and an overview of management.