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

Int J Cardiol. 1992 May;35(2):195-206; discussion 207-9. doi: 10.1016/0167-5273(92)90177-5.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Arrhythmias, Cardiac / diagnosis
  • Arrhythmias, Cardiac / etiology*
  • Bundle-Branch Block / diagnosis
  • Bundle-Branch Block / etiology
  • Cardiac Catheterization
  • Cardiomyopathies / complications
  • Cardiomyopathies / diagnosis*
  • Child
  • Child, Preschool
  • Coronary Angiography
  • Echocardiography
  • Electrocardiography
  • Female
  • Heart Ventricles / pathology
  • Humans
  • Male
  • Middle Aged
  • Myocardium / pathology
  • Ventricular Fibrillation / diagnosis
  • Ventricular Fibrillation / etiology