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The Role of Genetics in Risk Stratification Strategy of Dilated Cardiomyopathy
Anastasia Xintarakou1, Ourania Kariki2, Ioannis Doundoulakis1
1First Cardiology Department, National and Kapodistrian University of Athens, Hippokration General Hospital, 11527 Athens, Greece.
Insights
Dilated cardiomyopathy (DCM) is a complex heart condition. Genetic testing aids in diagnosing DCM and predicting sudden cardiac death (SCD) risk, improving patient management.
Area of Science:
- Cardiology
- Genetics
- Molecular Biology
Background:
- Dilated cardiomyopathy (DCM) affects millions globally, with high mortality and sudden cardiac death (SCD) risk.
- Current assessment methods, like ejection fraction, are insufficient for predicting arrhythmic events.
- Improved SCD risk stratification is crucial for effective patient management.
Purpose of the Study:
- To review current data on the genetic basis of DCM.
- To evaluate the role of genetic testing in DCM patient assessment.
- To explore genetic testing's value in predicting arrhythmic risk.
Main Methods:
- Literature review focusing on genetic substrates of DCM.
- Analysis of studies on genetic testing in DCM diagnosis and risk stratification.
- Synthesis of data on gene mutations and disease prognosis.
Main Results:
- Pathogenic variants are frequently found in DCM patients, irrespective of family history.
- Specific gene mutations correlate with DCM prognosis.
- Genetic testing aids in identifying patients for preventive SCD treatment.
Conclusions:
- Genetic testing is vital for DCM diagnosis and management.
- Understanding the genetic landscape of DCM improves risk stratification.
- Genetic insights facilitate personalized treatment strategies, including defibrillator implantation for SCD prevention.
Abstract:
Dilated cardiomyopathy (DCM) is a heart disorder of diverse etiologies that affects millions of people worldwide, associated with increased mortality rate and high risk of sudden cardiac death. Patients with DCM are characterized by a wide range of clinical and pre-clinical phenotypes which are related with different outcomes. Dominant studies have failed to demonstrate the value of the left ventricular ejection fraction as the only indicator for patients' assessment and arrhythmic events prediction, thus making sudden cardiac death (SCD) risk stratification strategy improvement, more crucial than ever. The multifactorial two-step approach, examining non-invasive and invasive risk factors, represents an alternative process that enhances the accurate diagnosis and the individualization of patients' management. The role of genetic testing, regarding diagnosis and decision making, is of great importance, as pathogenic variants have been detected in several patients either they had a disease relative family history or not. At the same time there are specific genes mutations that have been associated with the prognosis of the disease. The aim of this review is to summarize the latest data regarding the genetic substrate of DCM and the value of genetic testing in patients' assessment and arrhythmic risk evaluation. Undoubtedly, the appropriate application of genetic testing and the thoughtful analysis of the results will contribute to the identification of patients who will receive major benefit from an implantable defibrillator as preventive treatment of SCD.
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