A 32-year-old woman had asymptomatic HIV infection diagnosed with primary pulmonary hypertension simultaneously. She presented after a three-month rapid progression of symptoms and hemodynamic change. Physical examination and laboratory findings were compatible with pulmonary hypertension. No sensible cause could be found for the pulmonary hypertension except the HIV seropositivity; therefore, HIV-associated primary pulmonary hypertension was diagnosed. She was treated with diltiazem and oral anti-coagulation. After four months, her functional status improved from a NYHA functional class of II to I and improved in right venticular function. Since HIV is epidemic, the authors recommend HIV testing in cases of primary pulmonary hypertension.

Keywords: HIV, Primary pulmonary hypertension