A Case Report of Biventricular Arrhythmogenic Cardiomyopathy in a Middle-Aged Female

Arrhythmogenic cardiomyopathy is an inherited disease in which the normal myocardium is replaced by fibroadipose infiltrates. It is increasingly being recognized as a separate entity to arrhythmogenic right ventricular cardiomyopathy though is rarely diagnosed. We report a 47-year-old female who presented to her local emergency department with a history of presyncope while driving. Electrocardiograph revealed inferolateral ST changes and right bundle branch block. A high burden of premature ventricular contractions and non-sustained ventricular tachycardia was seen on telemetry. Echocardiography showed reduced left ventricular systolic function and cardiac magnetic resonance imaging demonstrated extensive fibrosis involving the left ventricle and the septum of the right ventricle. An inherited cardiac disease genetic panel, including desmosomal gene mutations, was non-contributary. Extensive workup for other potential causes of cardiac fibrosis and reduced left ventricle function including cardiac positron emission tomography (PET) was negative. Based on the presentation and these findings, a diagnosis of biventricular arrhythmogenic cardiomyopathy was made. The patient’s condition was complicated by third-degree heart block two weeks after initiation of pharmacological treatment that included amiodarone. An implantable cardiac defibrillator was implanted. She was referred to a tertiary centre specializing in inherited cardiac conditions for familial screening.