Sudden cardiac death in childhood RASopathy-associated hypertrophic cardiomyopathy: Validation of the HCM risk-kids model and predictors of events

• Published in: International journal of cardiology Vol. 393; p. 131405
• Main Authors: Boleti, Olga D., Roussos, Sotirios, Norrish, Gabrielle, Field, Ella, Oates, Stephanie, Tollit, Jennifer, Nepali, Gauri, Bhole, Vinay, Uzun, Orhan, Daubeney, Piers E.F., Stuart, Graham A., Fernandes, Precylia, McLeod, Karen, Ilina, Maria, Liaqath, Muhammad Najih Ali, Bharucha, Tara, Delle Donne, Grazia, Brown, Elspeth, Linter, Katie, Khodaghalian, Bernadette, Jones, Caroline, Searle, Jonathan, Mathur, Sujeev, Boyd, Nicola, Reindhardt, Zdenka, Duignan, Sophie, Prendiville, Terence, Adwani, Satish, Zenker, Martin, Wolf, Cordula Maria, Kaski, Juan Pablo
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.12.2023
• Subjects: Cardiomyopathy, Hypertrophic - complications; Cardiomyopathy, Hypertrophic - diagnosis; Child; Child, Preschool; Death, Sudden, Cardiac - epidemiology; Death, Sudden, Cardiac - etiology; Humans; Hypertrophic cardiomyopathy; Infant; Noonan syndrome; Pediatrics; RASopathies; Retrospective Studies; Risk Assessment; Risk Factors; Sudden cardiac death; Syncope; NSML; Pediatrics; LP; RV; NS; LV; RASopathies; CFCS; LVOT; RVOT; P; MLVWT; ANOVA; MACE; Noonan syndrome; LVOTO; NYHA; CCF; NSVT; NS-LAH; ICD; IQR; Sudden cardiac death; RVOTO; RA; CS; CV; HCM; LA; SCD; Hypertrophic cardiomyopathy; CHD; VT; LVH

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. To validate the HCM Risk-Kids SCD risk prediction model in chi...