Pulmonary arterial hypertension frequently occurs secondary to common cardi
opulmonary diseases, and more rarely as a primary condition. Observations i
n animals and man have suggested genetic influences underlying the suscepti
bility to pulmonary hypertension. A gene underlying many cases of familial
and apparently sporadic cases of primary pulmonary hypertension has now bee
n identified, and a genetic approach to hypoxia-induced pulmonary hypertens
ion promises to reveal new insights into the unique nature of the pulmonary
circulation. The exploration of potential candidate genes influencing susc
eptibility and the identification of new genes promise new therapeutic appr
oaches to the treatment of pulmonary hypertension.