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Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension

Abstract

Backround 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.

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Figure 1: Transverse CT angiogram demonstrating a large mass almost totally occluding the right main pulmonary artery with extension into the interlobar artery (thick arrows)
Figure 2: Perfusion scan (posterior view)
Figure 3: Pulmonary angiogram
Figure 4

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Kerr, K. Pulmonary artery sarcoma masquerading as chronic thromboembolic pulmonary hypertension. Nat Rev Cardiol 2, 108–112 (2005). https://doi.org/10.1038/ncpcardio0118

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  • DOI: https://doi.org/10.1038/ncpcardio0118

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