Lethal heart failure with anti-mitochondrial antibody: an arrhythmogenic right ventricular cardiomyopathy mimetic

<span class="paragraphSection">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 (<span style="font-style:italic;">Panel</span><span style="font-style:italic;">s</span><span style="font-style:italic;">A</span> and <span style="font-style:italic;">B</span>). 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 (<span style="font-style:italic;">Panel C</span>), 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 (<span style="font-style:italic;">Panel D</span>), similar to arrhythmogenic right ventricular cardiomyopathy (ARVC). In histology, there was massive infiltration of lymphocy...
Source: European Heart Journal - Category: Cardiology Source Type: research