Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus
Challenging differential diagnosis, Diagnostic / therapeutic accidents, Rare co-existance of disease or pathology
Takahide Ito, Shu-ichi Fujita, Yumiko Kanzaki, Koichi Sohmiya, Masaaki Hoshiga
Department of Cardiology, Osaka Medical College, Takatsuki, Osaka, Japan
Am J Case Rep 2018; 19:1197-1203
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