We performed a longitudinal study on 369 consecutive patients with at least one criterion for ARVC. Abnormal CMR was defined by the presence of one of the following: increased right ventricular (RV) volumes, reduced RV ejection fraction, RV regional wall motion abnormalities, myocardial fatty infiltration, and myocardial fibrosis. The end-point was a composite of cardiac death, sustained ventricular tachycardia, ventricular fibrillation, and appropriate ICD discharge.
Twenty patients met the composite end-point over a mean follow-up of 4.3 ¡À 1.5 years. An abnormal CMR was an independent predictor of outcomes (p < 0.001). The presence of multiple abnormalities heralded a particular high risk of events (HR 23.0, 95 % CI 5.7-93.2, p < 0.001 for 2 abnormalities; HR 35.8, 95 % CI 9.7-132.6, p < 0.001 for 3 or more abnormalities). The positive predictive value of an abnormal CMR study was 21.0 % for an adverse event, whilst the negative predictive value of a normal CMR study was 98.8 % over the follow-up period.
CMR provides important prognostic information in patients under evaluation for ARVC. A normal study portends a good prognosis. Conversely, the presence of multiple abnormalities identifies a high risk group of patients who may benefit from ICD implantation.