Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias

doi:10.1016/0167-5273(92)90177-5

International Journal of Cardiology

Volume 35, Issue 2, May 1992, Pages 195-206

https://doi.org/10.1016/0167-5273(92)90177-5 Get rights and content

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.

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^☆: Supported by the Research Project “Juvenile Sudden Death” Veneto Region, Venice and by the Research Project “FATMA”, National Council for Research, Rome, Italy.

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Original studyClinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias☆

Abstract

J Am Coll Cardiol

Int J Cardiol

Am Heart J

Am J Cardiol

J Electrocardiol

J Am Coll Cardiol

Am J Med

Am J Cardiol

Am J Cardiol

Am J Cardiol

Am J Cardiol

Am Heart J

Right ventricular dysplasia. A report of 24 adult cases

Circulation

Right ventricular cardiomyopathy and sudden death in young people

N Engl J Med

Juvenile sudden death and effort ventricular tachycardias in a family with right ventricular cardiomyopathy

Int J Cardiol

Echocardiographic and histologic evaluation of the right ventricle in ventricular tachycardias of left bundle branch block morphology without overt cardiac abnormality

Am J Cardiol

Right ventricular tachycardia: clinical and electrophysiologic characteristics

Circulation

Ventricular tachycardia of left bundle configuration in patients with isolated right ventricular dilatation. Clinical and electrophysiological features

Br Heart J

Ventricular tachycardia in a young population without overt heart disease

Circulation

Nonischemic ventricular tachycardia. Clinical course and long-term follow-up in patients without overt heart disease

Circulation

Accelerated idioventricular rhythm of infundibular origin in patients with a concealed form of arrhythmogenic right ventricular dysplasia

Br Heart J

Contribution of cross-sectional echocardiography to the diagnosis of right ventricular dysplasia at the asymptomatic stage

Eur Heart J

Diagnostic angiocardiographique des dysplasies ventriculaires droites

Arch Mal Coeur

Biopsia endomiocardica nella cardiomiopatia aritmogena del ventricolo, destro

Cardiologia

Original study
Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias☆