Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an infrequently diagnosed condition with a high incidence of sudden cardiac death. While the only option for cure is orthotopic cardiac transplantation, the use of an implantable cardiac defibrillator can be life saving. Accordingly, the prompt recognition of ARVC is crucial. Fortunately, a definitive diagnosis of ARVC can often be made by a combination of the clinical history and electrocardiogram alone, as illustrated by the present case. In our patient, who presented with multiple syncopal episodes and in whom ventricular tachycardia was observed shortly after her admission to the emergency department, a lengthy and expensive workup was initiated, which ultimately may have delayed implantation of an implantable cardiac defibrillator. Clinicians should be made aware of the clinical guidelines for dealing with this potentially dangerous condition so that appropriate therapy can be promptly initiated.

s Arrhythmogenic right ventricular cardiomyopathy in a 52-year-old man – clinical presentation mimicking an acute coronary syndrome

“…It should be however remembered that such patients are especially prone to procedure-related complications and reoperations which are not infrequent considering the relatively young age at diagnosis and progressive character of the disease. Increased procedure-related risk is associated with the fact that fibrofatty changes in the right ventricle may interfere with adequate lead positioning, and thin RV walls increase the risk of perforation [ 8 ]. Iatrogenic (lead-related) tricuspid regurgitation further deteriorates the function of the abnormal right ventricle [ 9 ].…”

“…Orthotopic heart transplantation is the only option providing a complete cure. However, due to limited availability it is reserved for a selected small group of patients with end-stage heart failure and drug-resistant ventricular tachyarrhythmias [ 8 ].…”