TABLE 1.

Updated clinical classification of group 1 pulmonary arterial hypertension (PAH) adapted from the 6th World Symposium on Pulmonary Hypertension

1.1 Idiopathic PAH 1.2 Heritable PAH 1.3 Drug- and toxin-induced PAH 1.4 PAH associated with:     1.4.1 connective tissue disease     1.4.2 HIV infection     1.4.3 portal hypertension     1.4.4 congenital heart disease     1.4.5 schistosomiasis 1.5 PAH long-term responders to calcium channel blockers 1.6 PAH with overt features of venous/capillaries (PVOD/PCH) involvement 1.7 Persistent pulmonary hypertension of the newborn syndrome

PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis.