Table 2 Clinical classification of PAH-CHD [15]

A. Eisenmenger Syndrome

Large congenital systemic-to-pulmonary shunt leading to pulmonary vasculopathy, increased PVR and reversal of shunt. Cyanosis and erythrocytosis present.

B. PAH associated with a predominant systemic-to-pulmonary shunt

Moderate-large shunt with mild-moderately increased pulmonary vascular resistance. Patient is not cyanotic at rest. Defect may be correctable or non-correctable.

C. PAH associated with a small defect

Significantly elevated pulmonary vascular resistance in the presence of a small defect (ASD < 2 cm diameter, VSD < 1 cm diameter). Often behaves similarly to idiopathic pulmonary arterial hypertension.

D. PAH associated with a repaired defect

PAH persists after closure or develops/recurs following closure.