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Clinical profile of concealed form of arrhythmogenic right ventricular cardiomyopathy presenting with apparently idiopathic ventricular arrhythmias
- Source :
- International journal of cardiology. 35(2)
- Publication Year :
- 1992
-
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.
- Subjects :
- Adult
Male
medicine.medical_specialty
Cardiac Catheterization
Adolescent
Heart Ventricles
Bundle-Branch Block
Cardiomyopathy
Hemodynamics
Ventricular tachycardia
Coronary Angiography
Sudden death
Right ventricular cardiomyopathy
Electrocardiography
Internal medicine
medicine
Humans
cardiovascular diseases
Child
Left bundle branch block
business.industry
Myocardium
Arrhythmias, Cardiac
Middle Aged
medicine.disease
medicine.anatomical_structure
Ventricle
Echocardiography
Child, Preschool
Ventricular fibrillation
Ventricular Fibrillation
cardiovascular system
Cardiology
Female
Cardiology and Cardiovascular Medicine
business
Cardiomyopathies
Subjects
Details
- ISSN :
- 01675273
- Volume :
- 35
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- International journal of cardiology
- Accession number :
- edsair.doi.dedup.....431b69411b73153d4aa91df12731c479