Norrish, G;
Field, E;
Kaski, JP;
(2021)
Childhood Hypertrophic Cardiomyopathy: A Disease of the Cardiac Sarcomere.
Frontiers in Pediatrics
, 9
, Article 708679. 10.3389/fped.2021.708679.
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Abstract
Hypertrophic cardiomyopathy is the second most common cause of cardiomyopathy presenting during childhood and whilst its underlying aetiology is variable, the majority of disease is caused by sarcomeric protein gene variants. Sarcomeric disease can present at any age with highly variable disease phenotype, progression and outcomes. The majority have good childhood-outcomes with reported 5-year survival rates above 80%. However, childhood onset disease is associated with considerable life-long morbidity and mortality, including a higher SCD rate during childhood than seen in adults. Management is currently focused on relieving symptoms and preventing disease-related complications, but the possibility of future disease-modifying therapies offers an exciting opportunity to modulate disease expression and outcomes in these young patients.
| Type: | Article |
|---|---|
| Title: | Childhood Hypertrophic Cardiomyopathy: A Disease of the Cardiac Sarcomere |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3389/fped.2021.708679 |
| Publisher version: | https://doi.org/10.3389/fped.2021.708679 |
| Language: | English |
| Additional information: | This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Pediatrics, paediatric, hypertrophic cardiomyopathy, sarcomere, sudden death, progression, LATE GADOLINIUM ENHANCEMENT, LONG-TERM OUTCOMES, RISK-FACTORS, PEDIATRIC CARDIOMYOPATHY, OBSTRUCTIVE CARDIOMYOPATHY, CLINICAL-SIGNIFICANCE, NATURAL-HISTORY, SEPTAL MYECTOMY, GENETIC-BASIS, SUDDEN-DEATH |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10132463 |
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