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Heart Vessels. 2014 Nov;29(6):873-7. doi: 10.1007/s00380-013-0446-7. Epub 2013 Nov 26.

Rapidly progressive symptom development of pulmonary arterial hypertension: a case report of Trousseau syndrome.

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  • 1Department of Cardiology, Toki Municipal General Hospital, 703-24 Tokiguchi, Tokitsu-cho, Toki, Gifu, 509-5193, Japan.


Trousseau syndrome is most commonly defined as a hypercoagulability syndrome associated with mucin-producing adenocarcinoma. We report here a rare case of Trousseau syndrome presenting as pulmonary arterial hypertension. The patient complained of cough and increasing exertional dyspnea. Rapidly progressive symptom development of pulmonary arterial hypertension accompanied by right heart failure was observed, and the patient died on hospital day 2. An autopsy revealed Krukenberg tumors on both ovaries and a signet-ring cell gastric carcinoma. In the lungs there was tumor embolism with signet-ring cells to some extent, but the peripheral pulmonary arteries were occupied primarily by pulmonary embolism with platelets, fibroblasts, and fibrotic organized thrombi.

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