Why the study?
Uncertainties regarding pathogenesis, classification, diagnostic criteria, and risk stratification continue to fuel controversy surrounding noncompaction cardiomyopathy.
This review highlights the importance of genetic testing and advanced cardiac imaging for the risk stratification and clinical management of noncompaction cardiomyopathy.
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May support genetic-imaging stratification in noncompaction cardiomyopathy; leaves open prospective validation before practice change.
Noncompaction cardiomyopathy is a heterogeneous and complex entity characterized by hypertrabeculation, typically of the left ventricle. Uncertainties regarding pathogenesis, classification as primary genetic or unclassified cardiomyopathy, diagnostic criteria, and risk stratification have contributed to fuel the discussion surrounding this disorder. Meanwhile, noncompaction phenotype is thought to be the morphological expression of different underlying pathophysiological mechanisms, genetics, and pathologies. Recent studies suggest that distinguishing genetic from nongenetic causes allows risk stratification and may support clinical management and counselling of patients and their relatives. Additionally, advanced cardiac imaging techniques have demonstrated a complementary role in outcome prediction. The purpose of this review is to provide a brief comprehensive review of this controversial entity.
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Santos et al. (2020) studied this question.
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