HAISSAGUERRE, M., ET AL.: Distinctive Response of Arrhythmogenic Right Ventricular Disease to High Dose Isoproterenol.Arrhythmogenic right ventricular disease is a potential cause of ventricular arrhythmias. Diagnosis is important due to the risk of sudden death, particularly as first symptom. Diagnosis is based on the angiographic demonstration of abnormal right ventricular morphology and function, while the sensitivity of noninvasive tests is relatively low. Following a particular observation studied in 1984, we prospectively assessed the diagnostic value of high dose isoproterenol infusion in 44 patients with an angiographically determined arrhythmogenic right ventricle. A continuous infusion of isoproterenol (8–30 µg/min) was administered during a 3-minute period, regardless of the obtained heart rate. In a control group of 50 patients without structural heart disease, isoproterenol induced a monomorphic ventricular tachycardia salvo in only one patient (2%). In patients with an arrhythmogenic right ventricle, isoproterenol induced one or more ventricular tachycardia runs in 39/44 cases (88%): one triplet in three patients, several runs in 23 patients and a sustained ventricular tachycardia in 13 patients. Arrhythmia was polymorphous in 85% of cases, but left bundle branch block morphelogy was the predominant pattern. In conclusion, high dose isoproterenol is a highly sensitive test for the diagnosis of arrhythmogenic right ventricular disease.