title: Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias.
abstract: In 24 subjects presenting with apparently idiopathic ventricular arrhythmias, a final diagnosis of arrhythmogenic right ventricular cardiomyopathy was formulated following global evaluation of the clinical, cross-sectional echocardiography and angiographic findings, and the observation of myocardial atrophy with fibrous-fatty substitution in right ventricular endomyocardial biopsy. All patients had good effort tolerance, and a normal cardiac silhouette. Ventricular arrhythmias with a left bundle branch block pattern were present in 23 cases (sustained ventricular tachycardia, nonsustained ventricular tachycardia, ventricular couplets, and ventricular premature complexes); 1 patient experienced an episode of ventricular fibrillation. A nearly constant electrocardiographic feature was T wave negativity in the right precordial leads. Cross-sectional echocardiography and hemodynamic studies showed that right ventricular impairment consisted only of localized structural and dynamic abnormalities; in a few cases the left ventricle was segmentally involved. Familial occurrence was present in 29% of the cases. No case of sudden death was observed during follow-up. These findings confirm that the concealed form of arrhythmogenic right ventricular cardiomyopathy is a cause of so-called "idiopathic" ventricular arrhythmias in subjects with apparently "normal hearts". Echocardiographic and angiographic investigations may lead to the correct diagnosis.
authors.affiliation: Department of Cardiology, University of Padua Medical School, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Nava
authors.forename: G
authors.initials: G
authors.lastname: Thiene
authors.forename: B
authors.initials: B
authors.lastname: Canciani
authors.forename: B
authors.initials: B
authors.lastname: Martini
authors.forename: L
authors.initials: L
authors.lastname: Daliento
authors.forename: G
authors.initials: G
authors.lastname: Buja
authors.forename: G
authors.initials: G
authors.lastname: Fasoli
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000209:etiology*; D002037:Bundle-Branch Block / Q000175:diagnosis / Q000209:etiology; D006328:Cardiac Catheterization; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D017023:Coronary Angiography; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000209:etiology