Pulmonary Hypertension in Congenital Heart Disease

Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient’s hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology , diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations.