Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies.
OBJECTIVE
We report an unusual case of primary pulmonary hypertension and review the pertinent literature.


METHODS
A 25-year-old Caucasian woman presented with progressive dyspnea and was found to have pulmonary hypertension. Antiphospholipid antibodies were present. The patient had a prolonged hospital course, was unresponsive to therapy, and died suddenly.


RESULTS
Postmortem examination revealed a large thrombus affecting the right main pulmonary artery, with plexogenic arteriopathy bilaterally.


CONCLUSIONS
This appears to be the first reported case of primary pulmonary hypertension complicated by thrombosis of a main pulmonary artery in association with antiphospholipid antibodies.
Luchi
M E
ME
Department of Medicine, St. Luke's/Roosevelt Hospital, Columbia University, New York, New York 10019.
Asherson
R A
RA
Lahita
R G
RG
D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications*; D001157:Arterial Occlusive Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000209:etiology; D011651:Pulmonary Artery / Q000473:pathology; D013927:Thrombosis / Q000473:pathology