Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids)
Language English Country United States Media print
Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't
Grant support
FS/16/72/32270
British Heart Foundation - United Kingdom
Department of Health - United Kingdom
PubMed
31411652
PubMed Central
PMC6694401
DOI
10.1001/jamacardio.2019.2861
PII: 2747608
Knihovny.cz E-resources
- MeSH
- Child MeSH
- Risk Assessment methods MeSH
- Cardiomyopathy, Hypertrophic complications mortality MeSH
- Incidence MeSH
- Humans MeSH
- Survival Rate trends MeSH
- Adolescent MeSH
- Death, Sudden, Cardiac epidemiology etiology MeSH
- Follow-Up Studies MeSH
- Prognosis MeSH
- Retrospective Studies MeSH
- Risk Factors MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Europe epidemiology MeSH
IMPORTANCE: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. OBJECTIVE: To develop and validate an SCD risk prediction model that provides individualized risk estimates. DESIGN, SETTING, AND PARTICIPANTS: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. EXPOSURES: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. MAIN OUTCOMES AND MEASURES: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). RESULTS: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. CONCLUSIONS AND RELEVANCE: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.
Cardiology Unit IRCCS Ospedale Maggiore Policlinico Milan Italy
Cardiothoracovascular Department Careggi University Hospital Florence Italy
Children's Heart Centre University Hospital Motol Prague Czech Republic
Children's Heart Service Evelina Children's Hospital London United Kingdom
Children's Heart Unit University Hospital of Wales Cardiff United Kingdom
Department of Ambulatory Cardiology Favaloro Foundation University Hospital Buenos Aires Argentina
Department of Cardiology Aarhus University Hospital Aarhus Denmark
Department of Cardiology Alder Hey Children's Hospital Liverpool United Kingdom
Department of Cardiology and Geriatrics Kochi Medical School Kochi University Kochi Japan
Department of Cardiology Odense University Hospital Odense Denmark
Department of Cardiology Onassis Cardiac Surgery Center Athens Greece
Department of Cardiology S Orsola Malpighi Hospital Bologna Italy
Department of Cardiology The Children's Memorial Health Institute Warsaw Poland
Department of Cardiology The Royal Children's Hospital Melbourne Australia
Department of Cardiology University Francisco de Vitoria Pozuelo de Alarcon Spain
Department of Cardiology University Hospital La Paz Madrid Spain
Department of Cardiology University Hospital Virgen de la Arrixaca Murcia Spain
Department of Cardiology University Hospitals Parma Parma Italy
Department of Cardiothoracic Sciences Monaldi Hospital Naples Italy
Department of Clinical Sciences The Murdoch Children's Research Institute Parkville Australia
Department of Medical and Health Sciences University of Melbourne Melbourne Australia
Department of Paediatric Cardiology and Cardiac Surgery Bambino Gesu Hospital Rome Italy
Department of Paediatric Cardiology Bristol Royal Hospital for Children Bristol United Kingdom
Department of Paediatric Cardiology Ghent University Hospital Ghent Belgium
Department of Paediatric Cardiology Glenfield Hospital Leicester United Kingdom
Department of Paediatric Cardiology Hospital General Universitario Gregorio Marañón Madrid Spain
Department of Paediatric Cardiology John Radcliffe Hospital Oxford United Kingdom
Department of Paediatric Cardiology Leeds General Infirmary Leeds United Kingdom
Department of Paediatric Cardiology Leiden University Medical Center Leiden the Netherlands
Department of Paediatric Cardiology Mater Dei Hospital Msida Malta
Department of Paediatric Cardiology Papa Giovanni XXIII Hospital Bergamo Italy
Department of Paediatric Cardiology Royal Brompton and Harefield NHS Trust London United Kingdom
Department of Paediatric Cardiology Royal Hospital for Children Glasgow United Kingdom
Department of Paediatric Cardiology Southampton General Hospital Southampton United Kingdom
Department of Paediatric Cardiology The Freeman Hospital Newcastle United Kingdom
Department of Statistical Science University College London London United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart Amsterdam the Netherlands
Institute of Cardiovascular Sciences University College London London United Kingdom
Medical Sciences Department School of Medicine University of Girona Girona Spain
Paediatric Cardiology Department Val d'Hebron University Hospital Barcelona Spain
Paediatric Cardiology Unit Niguarda Hospital Milan Italy
The Children's Heart Centre Our Lady's Children's Hospital Dublin Ireland
The Heart Unit Birmingham Children's Hospital Birmingham United Kingdom
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