Natural history and outcomes in paediatric RASopathy‐associated hypertrophic cardiomyopathy

• Published in: ESC Heart Failure Vol. 11; no. 2; pp. 923 - 936
• Main Authors: Boleti, Olga, Norrish, Gabrielle, Field, Ella, Dady, Kathleen, Summers, Kim, Nepali, Gauri, Bhole, Vinay, Uzun, Orhan, Wong, Amos, Daubeney, Piers E. F., Stuart, Graham, Fernandes, Precylia, McLeod, Karen, Ilina, Maria, Ali, Muhammad Najih Liaqath, Bharucha, Tara, Donne, Grazia Delle, Brown, Elspeth, Linter, Katie, Jones, Caroline B., Searle, Jonathan, Regan, William, Mathur, Sujeev, Boyd, Nicola, Reinhardt, Zdenka, Duignan, Sophie, Prendiville, Terence, Adwani, Satish, Kaski, Juan Pablo
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.04.2024; John Wiley and Sons Inc; Wiley
• Subjects: Age; Arrhythmia; Cardiology; Cardiomyopathy; Cardiomyopathy, Hypertrophic - diagnosis; Child; Death, Sudden, Cardiac; Demographics; Electrocardiography; Genes; Genetic testing; Genetics; Heart Failure; Heart transplants; Humans; Hypertrophic cardiomyopathy; Inherited cardiac conditions; Kinases; Mortality; Noonan; Noonan Syndrome - genetics; Original; Paediatric cardiology; Patients; Pediatrics; Predictors; Proteins; RASopathy; Retrospective Studies; Sudden cardiac death; Variables; Variance analysis; Inherited cardiac conditions; Arrhythmia; Mortality; RASopathy; Noonan; Hypertrophic cardiomyopathy; Genetics; Paediatric cardiology; Sudden cardiac death; Predictors

Aims This study aimed to describe the natural history and predictors of all‐cause mortality and sudden cardiac death (SCD)/equivalent events in children with a RASopathy syndrome and hypertrophic cardiomyopathy (HCM). Methods and results This is a retrospective cohort study from 14 paediatric cardio...