Background-The prognosis of patients with right ventricular (RV) arrhythmias remains uncertain. This study prospectively evaluated the prognostic value of RV and left ventricular (LV) involvement assessed by radionuclide angiography (RNA) as predictors for sudden death. Methods and Results-Patients (nϭ188) with severe arrhythmias originating from the RV were followed up for a mean of 45Ϯ34 months. Data on clinical presentation, resting and stress ECG, signal-averaged ECG, 24-hour Holter

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... ng, and programmed stimulation were collected along with RNA. Patients were classified as group I (nϭ82) with normal RNA or group II (nϭ106) with an abnormal RV suggestive of arrhythmogenic RV cardiomyopathy, classified as diffuse or localized disease, with or without associated LV abnormalities. During follow-up, 14 patients died suddenly, all in group II. None of the clinical and electrical data were predictive of death. An abnormal RNA study was a highly predictive factor for death (PϽ0.005), as well as the presence of LV abnormalities (PϽ0.01). Conclusions-The present study confirms that arrhythmogenic RV cardiomyopathy is a severe disease with a high risk for cardiac death. Evidence of RV abnormalities in patients presenting with RV arrhythmias is highly predictive for sudden death, as is its association with LV involvement. (Circulation. 2001;103:1972-1976.)