Characteristics, prognosis and treatment of the ventricular arrhythmias of right ventricular dysplasia

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Abstract
We studied 58 cases of arrhythmogenic right ventricular dysplasia (ARVD). Sustained monomorphic ventricular tachycardia (VT) was present in 50 patients, ventricular fibrillation (VF) in three (two also having VT), and non-sustained VT in the remaining seven. Different morphologies of VT were documented in 24 of the 50 patients with sustained VT. They had a left bundle branch block pattern in 96% of cases, without extreme deviation of the QRS axis, and a QRS relatively narrow and ample. These sustained VTs were triggered by provocative techniques. Holter recordings showed frequent ventricular extrasystoles in the great majority of cases. They were polymorphic in 78%, with runs of VT in 59% of patients. Spontaneous onset of VT occurred during exercise in 60% of cases, preceded by a sinus rate increase when recording was available. This is more frequent in angiographically localized forms of ARVD than in diffuse forms, and tends to disappear during follow-up. Only four cardiac deaths occurred after a follow-up of 8·8 ± 7·2 years: three by acute heart failure, and only one by recurrent VF. Spontaneous disappearance of VT which became non-inducible was seen in four cases. Single antiarrhythmic drug therapy was judged satisfactory in 21 cases, and combined therapy in 19 other cases. Surgery or fulguration was performed in 17 cases, with 14 successes (nine of them with combined antiarrhythmic therapy). Despite a frequent lack of control of VT by antiarrhythmic drugs, the follow-up of ARVD seems good in patients with sustained VT. Some arguments favour the concept of a diffuse and progressive disease.

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