Several case-reports and small series suggest a causal relationship between
human immunodeficiency virus (HIV) infection and pulmonary hypertension. W
e report on a HIV seropositive man with a high and stable CD4 lymphocyte co
unt (+/- 600/mm(3)) who developed severe pulmonary hypertension, not attrib
utable to other known causes. This case report underscores the fact that th
e degree of immunosuppression secondary to the HIV-infection seems to be of
little relevance in the pathophysiology of the syndrome. HIV-infected pati
ents with dyspnoea, not related to pulmonary infection, with exercise intol
erance, syncope or precordial pain should receive an electrocardiogram and
echocardiographic assessment. The exact pathogenetic mechanism of this rapi
dly progressive disease and whether anti-viral therapy should be promoted i
s still under investigation.