Breakdown of long-term responders to calcium channel blocker (CCB) monotherapy amongst those who are acutely vasoreactive, by type of PAH (data adopted from Sitbon et al. (2004)
a Other includes PAH associated with connective tissue disease, veno-occlusive disease, pulmonary capillary haemangiomatosis, human immunodeficiency virus, portopulmonary hypertension, familial and congenital heart defects.
b Acute vasoreactors were defined by a fall in both mean pulmonary artery pressure and pulmonary vascular resistance>20%.
c Long-term responders were defined as those being in functional class I or II after at least 1 year on CCB monotherapy.