Extract

A 60-year-old woman with heart failure and no family history of cardiomyopathy/sudden death was referred to our hospital. Echocardiography revealed large pericardial effusion and thinning of akinetic right ventricular free wall (PanelsA and B). Serum biochemistry showed low level of free thyroxine with elevation of thyroid-stimulating hormone level, elevated creatinine kinase level, and positive anti-mitochondrial antibody (AMA) (M2; 13.4 U/mL). Although supplementation of thyroid hormone and administration of diuretics were started, her condition was progressively deteriorated. Repeated echocardiography revealed reduction of left ventricular ejection fraction (40%), and right heart catheterization showed atrialization of right ventricular pressure pattern (Panel C), seen in Uhl’s anomaly. Two weeks after her admission, she died from cardiogenic shock despite inotropic/mechanical supports, and an autopsy was performed. Macroscopic examinations showed right ventricle-dominant fibrofatty replacement (Panel D), similar to arrhythmogenic right ventricular cardiomyopathy (ARVC). In histology, there was massive infiltration of lymphocytes with various degrees of fibrofatty replacement in right and left ventricles (PanelsE and F). Immunostaining with anti-plakophilin-2 antibody was positive without abnormality (Panel E, inset). Primary biliary cirrhosis (PBC) and Hashimoto’s thyroiditis were histologically confirmed (Panel G). Taken together, the final diagnosis of AMA-positive lymphocytic myocarditis with Hashimoto’s thyroiditis and PBC was made. !!...!!

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