Key result
Sotalol, spironolactone, and S-ICD prevent recurrent syncope over 2.5 years in pediatric ARVC.
Why the study?
Arrhythmogenic right ventricular cardiomyopathy is rarely diagnosed in childhood and lacks pediatric-specific diagnostic criteria.
Case Report (n=1)
May guide individualized pediatric ARVC management; leaves open need for prospective validation before broader adoption.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia , and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. ( Level of Difficulty: Beginner. )
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Roudijk et al. (2020) conducted a case report in Arrhythmogenic Right Ventricular Cardiomyopathy (n=1). Sotalol, spironolactone, and subcutaneous implantable cardioverter-defibrillator (S-ICD) was evaluated on Clinical status, syncope recurrence, and ICD interventions. Treatment with sotalol, spironolactone, and an S-ICD prevented recurrent syncope and required no device interventions over 2.5 years in a 9-year-old girl with arrhythmogenic right ventricular cardiomyopathy.
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