A systematic review of pediatric arrhythmogenic right ventricular cardiomyopathy found ventricular tachycardia to be the most common sign, though validated diagnostic tools are currently lacking.
Systematic Review
What are the diagnostic, risk stratification, and prognostic features of Arrhythmogenic Right Ventricular Cardiomyopathy in children?
ARVC in children lacks a gold standard for diagnosis and validated risk stratification models, highlighting the need for further research to ensure early detection and prompt intervention.
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.
Moisă et al. (Fri,) conducted a systematic review in Arrhythmogenic Right Ventricular Cardiomyopathy. A systematic review of pediatric arrhythmogenic right ventricular cardiomyopathy found ventricular tachycardia to be the most common sign, though validated diagnostic tools are currently lacking.