Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy.

Marit Kristine SmedsrudMonica ChivulescuMarianne Inngjerdingen ForsåAnna Isotta Castrini Eivind Westrum Aabel Christine Rootwelt-Norberg Martin Prøven Bogsrud Thor Edvardsen Nina Eide HasselbergAndreas FrühKristina Hermann Haugaa

Published in: European heart journal (2022)

In a paediatric ARVC cohort, there was a high incidence of severe cardiac events and half of them occurred in children ≤12 years of age. The ARVC penetrance in genotype positive paediatric relatives was 18%. These findings of a high-malignant phenotype in childhood-onset ARVC indicate a need for ARVC family screening at younger age than currently recommended.

Keyphrases

intensive care unit
emergency department
young adults
early life
risk factors
left ventricular
childhood cancer
early onset