Pulmonary Hypertension Associated with Congenital Heart Disease

Pulmonary hypertension in patients with congenital heart disease is associated with significant mortality, morbidity and health services utilization. The predominant subtype of pulmonary hypertension in these patients is pulmonary arterial hypertension (PAH). PAH associated with congenital heart disease (PAH-CHD) comprises up to one-third of all PAH cases globally and is most commonly associated with anatomically simple shunt lesions. A myriad of clinical phenotypes of PAH-CHD are seen across the spectrum of shunt size, location and directionality. A conceptual framework to categorize these patients based on pathophysiology is described. Contemporary data regarding the management of the varied phenotypes are reviewed, and a novel algorithm to guide decision-making with shunt closure in patients with PAH-CHD is provided. Further data spanning the spectrum of basic, translational and clinical science are much needed to further inform the management of this highly complex and heterogeneous population.