Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension.

BACKGROUND: A 40-year-old woman presented with dyspnea in the fifth month of pregnancy followed by a sudden onset of pleuritic chest pain 2 weeks postpartum. She was diagnosed as having pulmonary embolism by CT angiography and anticoagulated with heparin followed by warfarin. Despite 6 months of warfarin therapy she remained symptomatic. An echocardiogram demonstrated pulmonary hypertension, and a repeat CT angiogram demonstrated no change in the pulmonary artery filling defects. INVESTIGATIONS: Chest radiography, ventilation-perfusion scan, echocardiogram, right-heart catheterization, pulmonary angiography and pulmonary artery exploration. DIAGNOSIS: High-grade intimal sarcoma of the pulmonary artery. TREATMENT: Pulmonary endarterectomy.