Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies

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Abstract
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.