Perspective:

The 2010 Task Force criteria for the diagnosis of ARVC consist of: 1) “genotype-based” criteria, such as a specific mutation, a positive family history of ARVC or sudden cardiac death, and of 2) “phenotype-based” criteria such as structural abnormalities on echo or CMR, fibrofatty myocardial replacement, electrocardiogram abnormalities, and ventricular tachycardia (VT). The goal of the Task Force criteria was to maximize sensitivity for the diagnosis of this disorder given the incomplete expression, especially in the early phases of the cardiomyopathy.

In the current study of patients with a definite diagnosis of ARVC, the authors show that phenotypic expression as assessed by CMR has an excellent negative predictive value, i.e. patients with a completely normal CMR have an excellent prognosis over an average 5-year follow-up, and that LV involvement by itself or with RV involvement confers a particularly high risk of arrhythmic events. Outcome of patients with isolated RV involvement, on the other hand, could be predicted with the use of the previously described ARVC risk score composed of the following elements: age at diagnosis, gender, presence of cardiac syncope, number of inverted T waves in precordial and inferior leads, maximum 24-hour premature ventricular contraction count, history of nonsustained VT, and the RV ejection fraction (ARVC risk calculator). Additional research might help find predictors of arrhythmic events in patients with ARVC and LV involvement.