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.