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April 28, 2021Journal of the American Heart AssociationOpen Access

Clinical genetic testing and exome sequencing identified a definitive molecular cause in 48% of previously tested and 21% of previously untested children with cardiomyopathy.

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Why the study?

Practice variations exist despite guideline recommendations for genetic testing in pediatric cardiomyopathy, and robust data on clinical testing practices and diagnostic yield in children are lacking.

Population

152 children with familial or idiopathic cardiomyopathy from 14 institutions in North America

Comparison

Exome sequencing for rare variants in 37 known cardiomyopathy genes across clinical testing subgroups

Design

Multicenter observational cohort study

Key result

Clinical genetic testing and exome sequencing identified a definitive molecular cause in 48% of previously tested and 21% of previously untested children with cardiomyopathy.

Authors

SWStephanie M. WareJWJames D. WilkinsonMTMuhammad Tariq

Discussion

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Overview

Adds diagnostic yield data for genetic testing in pediatric cardiomyopathy; leaves open effects on management and outcomes.

Study Design

Type

Observational (n=152)

Multicenter

Yes

Structured PICO

P
Population
152 children with familial or idiopathic cardiomyopathy enrolled from 14 institutions in North America.
E
Exposure
Exome sequencing assessing rare sequence variants in 37 known cardiomyopathy genes
O
Outcome
Identification of genetic causes of cardiomyopathy and clinical genetic testing practices

Exome sequencing can identify a definitive molecular genetic diagnosis in a substantial proportion of children with cardiomyopathy, supporting the routine use of genetic testing in this population.

Cite This Study

Ware et al. (2021) conducted an observational in Pediatric cardiomyopathy (n=152). Genetic testing and exome sequencing was evaluated on Identification of a molecular cause (positive genetic test result). Clinical genetic testing and exome sequencing identified a definitive molecular cause in 48% of previously tested and 21% of previously untested children with cardiomyopathy.

synapsesocial.com/papers/6a984e3f3de45e4adad7e919https://doi.org/10.1161/jaha.120.017731
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Genetic Basis of Childhood Cardiomyopathy2022 · 65 citations
  2. 2Importance of genetic evaluation and testing in pediatric cardiomyopathy2014 · 51 citations
  3. 3Evaluation of genetic causes of cardiomyopathy in childhood2015
  4. 4Genomics of pediatric cardiomyopathy2025 · 13 citations
  5. 5Genetic testing and family screening in idiopathic pediatric cardiomyopathy: a prospective observational study from a tertiary care center in North India2023 · 1 citations