Arrhythmogenic right ventricular cardiomyopathy is a predominantly inherited structural disease with a heterogeneous set of implicated genetic defects. For those patients not identified by targeted population screening programs, ventricular tachycardia can be their first presentation. We report a case of a female from the genetically isolated Hutterite colonies who presented with recurrent ventricular tachycardia. She was found to be homozygous for a truncated desmocollin-2 gene, with both severe right ventricular dysfunction and left ventricular involvement. Her medical management was complicated by the finding of concomitant pre-term pregnancy. Management options for arrhythmia suppression and treatment are reviewed taking into account her decompensated biventricular dysfunction and possible fetal effects.
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