Is Atrial Vulnerability Increased In Arrhythmogenic Right Ventricular Cardiomyopathy?
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle dis- ease, often familial, that is characterized pathologically by fibro-fatty replacement of the right ventricular (RV) myocardium and clinically by ventricular arrhyth- mias of RV origin leading to sudden death, mostly in young people and athletes [1–8]. The progressive loss of the RV myocardium has been considered the result of a genetically determined dystrophic process, an inflammatory myocardial injury and death, or a programmed cell death (“apoptosis”) [1–6,9]. The most fre- quent clinical features of ARVC consist of ventricular arrhythmias with left bun- dle branch block morphology ranging from isolated premature ventricular beats to sustained ventricular tachycardia or ventricular fibrillation [1–7, 10]. Other common clinical manifestations of the disease include global and/or regional dysfunction and structural alterations of the right ventricle or both ventricles, and ECG depolarization/repolarization changes, mostly localized to right precor- dial leads ; supraventricular arrhythmias (SVA) -mostly atrial fibrillation and flutter- represent a less frequent clinical feature [12–15]. Whatever the etiopatho- genetic mechanisms involved, there is definitive clinico-pathological evidence that ARVC is a progressive heart muscle disease . Clinico-pathological investi- gations and long-term follow-up data from clinical studies indicate that ARVC with time may lead to more diffuse RV changes and left ventricular involvement culminating in heart failure [6,16,17]. Therefore, the natural history of ARVC is a function of both the electrical instability of diseased ventricular myocardium, which can precipitate “arrhythmic” cardiac arrest any time during the disease course, and the progressive myocardial loss that results in right or biventricular dysfunction and heart failure.
KeywordsRight Ventricular Atrial Flutter Atrial Tachycardia Arrhythmogenic Right Ventricular Cardiomyopathy Ventricular Cardiomyopathy
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