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Endomyocardial biopsy in infants and children with cardiomyopathy
R Narayan1, S Menahem, C W Chow
1Department of Cardiology, Royal Children's Hospital, Parkville, Victoria, Australia.
Insights
Endomyocardial biopsy in children with cardiomyopathy can diagnose rare conditions like hemochromatosis and myocarditis. While risky, this procedure offers valuable diagnostic and prognostic insights for serious heart disorders.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
Background:
- Cardiomyopathy in infants and children presents diagnostic challenges.
- Endomyocardial biopsy (EMB) is an invasive procedure with potential diagnostic yield.
Purpose of the Study:
- To evaluate the diagnostic utility and safety of EMB in pediatric cardiomyopathy.
- To assess the impact of EMB on diagnosis, treatment, and prognosis.
Main Methods:
- Retrospective review of 21 symptomatic infants and children undergoing EMB.
- Analysis of clinical presentation, biopsy findings, and patient outcomes.
Main Results:
- Diagnoses included hemochromatosis, Adriamycin cardiomyopathy, lymphocytic myocarditis, endocardial fibroelastosis, endomyocardial fibrosis, and mitochondrial abnormalities.
- 11 patients had normal or nonspecific findings.
- Two cases of myocardial perforation occurred, with successful resuscitation.
Conclusions:
- EMB can provide crucial diagnostic information in selected pediatric cardiomyopathy cases.
- Despite risks and a low positive yield, EMB may be indicated for serious cardiac conditions.
- Findings can guide treatment and offer prognostic value in pediatric heart disease.
Abstract:
We reviewed our experience of endomyocardial biopsy performed on 21 symptomatic infants and children with cardiomyopathy. Clinical congestive cardiomyopathy was noted in 18 patients, 2 had hypertrophic cardiomyopathy, and 1 a restrictive cardiomyopathy. The biopsy findings led to a diagnosis of hemochromatosis in one patient, Adriamycin cardiomyopathy in another, and lymphocytic myocarditis in a third. Five patients had features of endocardial fibroelastosis, one endomyocardial fibrosis, and a further one, a mitochrondrial abnormality. In 11 patients normal or nonspecific features were seen. There were 2 myocardial perforations, both patients being successfully resuscitated. Endomyocardial biopsy, although occasionally hazardous, may sometimes provide valuable information which may lead to a diagnosis, facilitate treatment, and be of prognostic value. Despite the low positive yield, it may still be indicated in selected patients, in view of the seriousness and often poor prognosis of this disorder: 5 of our study group subsequently died.