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08 October 2018 : Case report  Japan

Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus

Challenging differential diagnosis, Diagnostic / therapeutic accidents, Rare coexistence of disease or pathology

Takahide Ito1ABDEF*, Shu-ichi Fujita1BD, Yumiko Kanzaki1BD, Koichi Sohmiya1D, Masaaki Hoshiga1D

DOI: 10.12659/AJCR.910861

Am J Case Rep 2018; 19:1197-1203


BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Intracardiac thrombosis is one of the major cardiac complications in EGPA that may cause thromboembolism.

CASE REPORT: A 46-year-old male presenting with intermittent chest pain and numbness of the lower extremities was admitted to our center. His case was complicated by multiple brain infarcts and endocardial thrombosis in the left ventricle. A condition of restrictive cardiomyopathy was also found. After a thorough workup, he was diagnosed with antineutrophil cytoplasmic antibody (ANCA) positive EGPA. Interestingly, the thrombus was accompanied by a pressure gradient producing mid-ventricular obstruction. The patient improved reasonably with immunosuppression and anticoagulation treatment, in addition to heart failure treatment, and had a concomitant regression of the thrombus and reduction of the pressure gradient.

CONCLUSIONS: For an EGPA patient complicated by intraventricular obstruction caused by endocardial thrombosis, which could accelerate the release of the thrombus into the systemic circulation resulting in a life-threating condition, timely and aggressive measures against cardioembolic complications should be considered.

Keywords: Cardiomyopathy, Restrictive, Churg-Strauss syndrome, Echocardiography, Embolism and Thrombosis

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923