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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.


CONCLUSION
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.