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Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review
Stefana Maria Moisa1,2, Elena Lia Spoiala1, Eliza Cinteza3,4
1Pediatrics Department, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) in children requires early diagnosis due to sudden cardiac death risks. Validated diagnostic and risk tools are crucial for prompt intervention and improved outcomes in pediatric ARVC patients.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited condition causing fibroadipocytic tissue replacement of the myocardium.
- Early diagnosis is critical in pediatric populations due to the elevated risk of sudden cardiac death.
Purpose of the Study:
- To review the current literature on the diagnosis, risk stratification, and prognosis of ARVC in children.
- To highlight the need for validated tools for early detection and management in the pediatric population.
Main Methods:
- Literature review of case reports and studies focusing on pediatric ARVC.
- Analysis of diagnostic criteria, presenting symptoms, and risk stratification challenges.
Main Results:
- Ventricular tachycardia is the most common sign in pediatric ARVC cases, often presenting as syncope or cardiac arrest.
- No gold standard for diagnosing ARVC in children currently exists; genetic analysis shows promise for asymptomatic cases.
- Validated risk stratification models for pediatric ARVC are lacking, necessitating further research.
Conclusions:
- ARVC significantly impacts survival and clinical outcomes in children, despite its rarity.
- Development and validation of diagnostic and risk stratification tools are essential for early detection and timely therapeutic intervention in pediatric ARVC.
Abstract:
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest. Currently, there is no gold standard for diagnosing ARVC in children. Nevertheless, genetic analysis may provide a proper diagnosis tool for asymptomatic cases. Although risk stratification is recommended in patients with ARVC, a validated prediction model for risk stratification in children is still lacking; thus, it is a matter of further research. In consequence, even though ARVC is a relatively rare condition in children, it negatively impacts the survival and clinical outcomes of the patients. Therefore, appropriate and validated diagnostic and risk stratification tools are crucial for the early detection of children with ARVC, ensuring a prompt therapeutic intervention.
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