Olga D Boleti, Sotirios Roussos, Gabrielle Norrish, Ella Field, Stephanie Oates, Jennifer Tollit, Gauri Nepali, Vinay Bhole, Orhan Uzun, Piers E F Daubeney, Graham A Stuart, Precylia Fernandes, Karen McLeod, Maria Ilina, Muhammad Najih Ali Liaqath, Tara Bharucha, Grazia Delle Donne, Elspeth Brown, Katie Linter, Bernadette Khodaghalian, Caroline Jones, Jonathan Searle, Sujeev Mathur, Nicola Boyd, Zdenka Reindhardt, Sophie Duignan, Terence Prendiville, Satish Adwani, Martin Zenker, Cordula Maria Wolf, Juan Pablo Kaski
BACKGROUND: RASopathies account for nearly 20% of cases of childhood hypertrophic cardiomyopathy (HCM). Sudden cardiac death (SCD) occurs in patients with RASopathy-associated HCM, but the risk factors for SCD have not been systematically evaluated. AIM: To validate the HCM Risk-Kids SCD risk prediction model in children with RASopathy-associated HCM and investigate potential specific SCD predictors in this population. METHODS: Validation of HCM Risk-Kids was performed in a retrospective cohort of 169 patients with a RASopathy-associated HCM from 15 international paediatric cardiology centres...
September 28, 2023: International Journal of Cardiology