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Published on: June 20, 2014
Differences between genetic dilated cardiomyopathy and myocarditis in children presenting with severe cardiac
Ferran Gran1, Andrea Fidalgo2, Paola Dolader2
1Paediatric Cardiology Unit, Hospital Universitari Vall d'Hebron, Vall d'Hebron Barcelona Hospital Campus, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain. fgran@vhebron.net.
Differentiating acute myocarditis from genetic cardiomyopathy in children is challenging. Some genetic cardiomyopathies mimic inflammatory conditions, complicating diagnosis even with endomyocardial biopsies.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
Background:
- Acute myocarditis presents as severe heart failure in children.
- Distinguishing myocarditis from genetic cardiomyopathy is clinically difficult.
- Parvovirus B19 is a primary cause of pediatric myocarditis.
Purpose of the Study:
- Identify clinical presentation patterns differentiating pediatric myocarditis from genetic cardiomyopathy.
- Assess invasive and non-invasive measures for differential diagnosis.
- Evaluate diagnostic utility of endomyocardial biopsy in these conditions.
Main Methods:
- Retrospective descriptive study of pediatric patients (0-16 years) with new-onset heart failure (LVEF < 35%).
- Patients underwent endomyocardial biopsy (EMB) between April 2007 and December 2020.
- Classification into Group 1 (myocarditis, n=18) and Group 2 (genetic cardiomyopathy, n=9).
Main Results:
- Myocarditis cases showed more fulminant/acute presentation (77.8% vs 33.3%), higher cardiac enzyme elevation (p=0.011), lower LV dimension z-score (2.2 vs 5.4), and increased wall thickness (88.8% vs 33.3%).
- Myocarditis group had more frequent edema on EMB.
- Notably, 77.8% of genetic cardiomyopathy patients exhibited inflammation on EMB, meeting criteria for inflammatory cardiomyopathy.
Conclusions:
- Differentiating pediatric myocarditis from genetic cardiomyopathy is challenging, even with endomyocardial biopsy.
- Some genetic cardiomyopathies fulfill diagnostic criteria for inflammatory cardiomyopathy.
- Developing predictive models using invasive/non-invasive measures is crucial for accurate diagnosis.
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