Clinical characteristics of various arrhythmogenic cardiomyopathy phenotypes in the pediatric population: a systematic review and meta-analysis

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a progressive disease with a high risk of sudden cardiac death (SCD), which is very rare in the pediatric population. Aim. To study the clinical course, outcomes and prognosis in various ACM phenotypes in pediatric patients.Material and methods. During the initial selection, 144 publications were found in the PubMed database. From the initially identified results, an analysis of 7 works was carried out.Results. Positive family history for ACM was revealed in 90% of cases. Every second patient had symptoms of the disease (49,2% (95% confidence interval (CI): 19,3-79,1)). Frequent clinical manifestations of ACM were palpitations, heart failure symptoms, and syncope. The incidence of SCD and sudden cardiac arrest was 7,1% (95% CI: 1,7-12,4) and 5,1% (95% CI: 1,5-8,7), respectively. Implantation of an implantable-cardioverter defibrillator was performed in 40% of cases. Conclusion. We did not reveal any specificity of clinical signs depending on ACM phenotype. However, an earlier onset and an unfavorable course are characteristic of non-classical ACM types. ACM is characterized by a high risk of SCD, so it is extremely important to make a timely diagnosis.

Keywords

• arrhythmogenic cardiomyopathy
• sudden cardiac death
• implantable cardioverter-defibrillator
• clinical features
• children