Abstract
A rare case of a 6-year-old male with idiopathic familial cardiomyopathy manifesting severely restrictive physiology is reported. The patient showed congestive heart failure with dilatation of both atria with a normal ventricular cavity. A square-root configuration was revealed in the ventricular pressure tracings. His elder brother had died of hypertrophic cardiomyopathy at the age of 3 years. Endomyocardial biopsy disclosed marked disorganization of muscle bundles with hypertrophy of the myocytes and interstitial fibrosis. The patient died suddenly during hospitalization. Autopsy revealed diffuse hypertrophy of both the ventricular walls and the ventricular septum with extensive myocardial disorganization and interstitial fibrosis. These advanced myopathic changes in the myocardium may have been related to the restrictive physiology in this case.
| Original language | English |
|---|---|
| Pages (from-to) | 206-210 |
| Number of pages | 5 |
| Journal | Heart and Vessels |
| Volume | 7 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - 1992 Dec |
Keywords
- Hypertrophic cardiomyopathy
- Myocardial disorganization
- Pediatric cardiomyopathy
- Restrictive physiology
- Square-root configuration
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine