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Am J Physiol Lung Cell Mol Physiol 291: L547-L558, 2006; doi:10.1152/ajplung.00546.2005
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Table 1. Diagnostic classification of pulmonary hypertension


Pulmonary arterial hypertension Familial PAH, idiopathic (Primary) PAH
PAH associated with collagen vascular disease, sarcoidosis, congenital left-to-right shunt, portal hypertension, human immunodeficiency infection, appetite-suppressant drugs, pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis, and persistent pulmonary hypertension of the newborn
PH with left heart disease Left-sided atrial, ventricular, or valvular heart disease
PH associated with chronic lung disease or hypoxemia Chronic obstructive pulmonary disease, interstitial lung disease, sleep-disordered breathing, alveolar hypoventilation syndromes, prolonged residence at high altitude
PH due to chronic thrombo-embolic disease


PH, pulmonary hypertension; PAH, pulmonary arterial hypertension. [Adapted from Humbert (50).]





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