Noninvasive risk stratification in arrhythmogenic right ventricular cardiomyopathy

Abstract

The natural history of arrhythmogenic right ventricular cardiomyopathy is determined by the electrical instability of the dystrophic myocardium, which can precipitate arrhythmic cardiac arrest any time during the course of the disease and by the progressive myocardial loss that results in ventricular dysfunction and heart failure. Sudden death accounts for the majority of the fatal events but its occurrence is mostly unpredictable. There are no prospective and controlled studies assessing clinical markers that can predict the occurrence of life-threatening ventricular arrhythmias. However, the noninvasive risk profile, which emerges from retrospective analysis of clinical and pathologic series, is characterized by history of syncope, physical exercise, spontaneous ventricular tachycardia or ventricular fibrillation, right ventricular dysfunction, left ventricular involvement, right precordial negative T wave, right bundle branch block, QT-QRS dispersion, right precordial ST-segment elevation and late potentials. At present only QRS dispersion, history of syncope and right and/or left ventricular abnormalities at radionuclide angiography proved to be independent noninvasive predictors of sudden death.

Figure 1

Distribution of QT dispersions in three ARVC groups: group I is composed of 20 patients who died suddenly, group II of 20 patients with sustained ventricular tachycardia, and group III of 20 patients with no sustained ventricular tachycardia. Mean values are indicated by a horizontal line (from Turrini et al., ¹⁸ modified).

Figure 2

Distribution of QRS dispersions in three ARVC groups: group I is composed of 20 patients who died suddenly, group II 20 of patients with sustained ventricular tachycardia, and group III of 20 patients with no sustained ventricular tachycardia. Mean values are indicated by horizontal line (from Turrini et al, ¹⁸ modified).