Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids)
Por:
Norrish G, Ding T, Field E, Ziólkowska L, Olivotto I, Limongelli G, Anastasakis A, Weintraub R, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernández A, Bökenkamp R, Baban A, Kubuš P, Daubeney PEF, Sarquella-Brugada G, César-Díaz S, Marrone C, Bhole V, Medrano C, Uzun O, Brown E, Gran F, Castro FJ, Stuart G, Vignati G, Barriales-Villa R, Guereta LG, Adwani S, Linter K, Bharucha T, Garcia-Pavia P, Rasmussen TB, Calcagnino MM, Jones CB, De Wilde H, Toru-Kubo J, Felice T, Mogensen J, Mathur S, Reinhardt Z, O'Mahony C, Elliott PM, Omar RZ and Kaski JP
Publicada:
1 sep 2019
Categoría:
Cardiology and cardiovascular medicine
Resumen:
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.
Filiaciones:
Norrish G:
Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital, London, United Kingdom
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Ding T:
Department of Statistical Science, University College London, London, United Kingdom
Field E:
Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital, London, United Kingdom
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Ziólkowska L:
Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland
Olivotto I:
Cardiothoracovascular Department, Careggi University Hospital, Florence, Italy
Limongelli G:
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Department of Cardiothoracic Sciences, Monaldi Hospital, Naples, Italy
Anastasakis A:
Department of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece
Weintraub R:
Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia
Department of Clinical Sciences, The Murdoch Children's Research Institute, Parkville, Australia
Department of Medical and Health Sciences, University of Melbourne, Melbourne, Australia
Biagini E:
Department of Cardiology, S. Orsola-Malpighi Hospital, Bologna, Italy
Ragni L:
Department of Cardiology, S. Orsola-Malpighi Hospital, Bologna, Italy
Prendiville T:
The Children's Heart Centre, Our Lady's Children's Hospital, Dublin, Ireland
Duignan S:
The Children's Heart Centre, Our Lady's Children's Hospital, Dublin, Ireland
McLeod K:
Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, United Kingdom
Ilina M:
Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, United Kingdom
Fernández A:
Department of Ambulatory Cardiology, Favaloro Foundation University Hospital, Buenos Aires, Argentina
Bökenkamp R:
Department of Paediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands
Baban A:
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Department of Paediatric Cardiology and Cardiac Surgery, Bambino Gesu Hospital, Rome, Italy
Kubuš P:
Children's Heart Centre, University Hospital Motol, Prague, Czech Republic
Daubeney PEF:
Department of Paediatric Cardiology, Royal Brompton and Harefield NHS Trust, London, United Kingdom
Sarquella-Brugada G:
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain
Medical Sciences Department, School of Medicine, University of Girona, Girona, Spain
César-Díaz S:
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Arrhythmia and Inherited Cardiac Diseases Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain
Marrone C:
Department of Paediatric Cardiology, Papa Giovanni XXIII Hospital, Bergamo, Italy
Bhole V:
The Heart Unit, Birmingham Children's Hospital, Birmingham, United Kingdom
Medrano C:
Department of Paediatric Cardiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Uzun O:
Children's Heart Unit, University Hospital of Wales, Cardiff, United Kingdom
Brown E:
Department of Paediatric Cardiology, Leeds General Infirmary, Leeds, United Kingdom
Gran F:
Paediatric Cardiology Department, Val d'Hebron University Hospital, Barcelona, Spain
Castro FJ:
Department of Cardiology, University Hospital Virgen de la Arrixaca, Murcia, Spain
Stuart G:
Department of Paediatric Cardiology, Bristol Royal Hospital for Children, Bristol, United Kingdom
Vignati G:
Paediatric Cardiology Unit, Niguarda Hospital, Milan, Italy
Barriales-Villa R:
Department of Cardiology, Complexo Hospitalario Universitario A Coruña, Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares, A Coruña, Spain
Guereta LG:
Department of Cardiology, University Hospital La Paz, Madrid, Spain
Adwani S:
Department of Paediatric Cardiology, John Radcliffe Hospital, Oxford, United Kingdom
Linter K:
Department of Paediatric Cardiology, Glenfield Hospital, Leicester, United Kingdom
Bharucha T:
Department of Paediatric Cardiology, Southampton General Hospital, Southampton, United Kingdom
Garcia-Pavia P:
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares, Madrid, Spain
Department of Cardiology, University Francisco de Vitoria, Pozuelo de Alarcon, Spain
Rasmussen TB:
Department of cardiology, Aarhus University Hospital, Aarhus, Denmark
Calcagnino MM:
Department of Cardiology, University Hospitals Parma, Parma, Italy
Cardiology Unit, IRCCS Ospedale Maggiore Policlinico, Milan, Italy
Jones CB:
Department of Cardiology, Alder Hey Children's Hospital, Liverpool, United Kingdom
De Wilde H:
Department of Paediatric Cardiology, Ghent University Hospital, Ghent, Belgium
Toru-Kubo J:
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan
Felice T:
Department of Paediatric Cardiology, Mater Dei Hospital, Msida, Malta
Mogensen J:
Department of Cardiology, Odense University Hospital, Odense, Denmark
Mathur S:
Children's Heart Service, Evelina Children's Hospital, London, United Kingdom
Reinhardt Z:
Department of Paediatric Cardiology, The Freeman Hospital, Newcastle, United Kingdom
O'Mahony C:
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
St Bartholomew's Centre for Inherited Cardiovascular Diseases, Barts Heart Centre, St Bartholomew's Hospital, West Smithfield, London, United Kingdom
Elliott PM:
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
St Bartholomew's Centre for Inherited Cardiovascular Diseases, Barts Heart Centre, St Bartholomew's Hospital, West Smithfield, London, United Kingdom
Omar RZ:
Department of Statistical Science, University College London, London, United Kingdom
Kaski JP:
Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital, London, United Kingdom
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
European Reference Network for Rare and Complex Diseases of the Heart, Amsterdam, the Netherlands
Green Submitted, Bronze
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