Key result
Arrhythmogenic cardiomyopathy is a heterogeneous genetic disease with a high risk of life-threatening ventricular arrhythmias, requiring individualized risk stratification especially in pediatric patients.
Why the study?
Diagnosis, evolution, and outcomes of arrhythmogenic cardiomyopathy have been widely reported in adults, but data and debated risk stratification in the pediatric population remain limited.
This review highlights the need for better risk stratification and more data on arrhythmogenic cardiomyopathy in the pediatric population.
Scarce pediatric ACM data limit risk stratification; leaves open validation of new factors before guiding care.
Arrhythmogenic cardiomyopathy (ACM) is a cardiomyopathy characterized by the occurrence of a high risk of life-threatening ventricular arrhythmias and sudden cardiac death even at presentation. Diagnosis, evolution and outcomes in adults have been extensively reported, but little data in pediatric population are available. Risk stratification in this particular setting is still a matter of debate and new risk factors are needed in a model of an ever more "individualized medicine".
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Cicenia et al. (2022) conducted a review in Arrhythmogenic Cardiomyopathy. Arrhythmogenic cardiomyopathy is a heterogeneous genetic disease with a high risk of life-threatening ventricular arrhythmias, requiring individualized risk stratification especially in pediatric patients.