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Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
G. Norrish, A. Cleary, E. Field, E. Cervi, O. Boleti, L. Ziółkowska, I. Olivotto, D. Khraiche, G. Limongelli, A. Anastasakis, R. Weintraub, E. Biagini, L. Ragni, T. Prendiville, S. Duignan, K. McLeod, M. Ilina, A. Fernandez, C. Marrone, R....
Language English Country United States
Document type Journal Article, Research Support, Non-U.S. Gov't
Grant support
British Heart Foundation - United Kingdom
NLK
Free Medical Journals
from 1983 to 1 year ago
Open Access Digital Library
from 1998-01-01
- MeSH
- Defibrillators, Implantable * adverse effects MeSH
- Child MeSH
- Cardiomyopathy, Hypertrophic * diagnosis epidemiology therapy MeSH
- Humans MeSH
- Death, Sudden, Cardiac prevention & control MeSH
- Heart Failure * epidemiology MeSH
- Heart Transplantation * adverse effects MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
BACKGROUND: Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES: The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS: Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS: At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS: Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages.
Aalborg University Hospital Aalborg Denmark
Aarhus University Hospital Aarhus Denmark
Alder Hey Children's Hospital Liverpool United Kingdom
Bambino Gesu Hospital Rome Italy
Birmingham Children's Hospital Birmingham United Kingdom
Bristol Royal Hospital for Children Bristol United Kingdom
Cardiology Unit St Orsola Hospital IRCCS Azienda Ospedalierao Universitaria di Bologna Bologna Italy
Cardiothoracovascular Department University of Trieste Trieste Italy
Careggi University Hospital Florence Italy
Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital London United Kingdom
Complexo Hospitalario Universitario A Coruña INIBIC CIBERCV La Coruña Spain
Department of Pediatric Cardiology Charite Universitatsmedizin Berlin Berlin Germany
DZHK partner site Berlin Berlin Germany
Evelina Children's Hospital London United Kingdom
Fondazione Toscana G Monasterio Massa Pisa Italy
Fundación Favaloro University Hospital Buenos Aires Argentina
Ghent University Hospital Ghent Belgium
Glenfield Hospital Leicester United Kingdom
Hospital General Universitario Gregorio Marañón Madrid Spain
Hospital Saint Joseph Marseille France
Hospital Universitario Puerta de Hierro Majadahonda Madrid Spain
Institute of Cardiovascular Sciences University College London London United Kingdom
John Radcliffe Hospital Oxford United Kingdom
Kochi Medical School Hospital Kochi Japan
Leeds General Infirmary Leeds United Kingdom
Leiden University Medical Center Leiden the Netherlands
Mater Dei Hospital Msida Malta
Necker Enfants Malades Hospital Paris France
Onassis Cardiac surgery Center Athens Greece
Our Lady's Children's Hospital Dublin Ireland
Papa Giovanni XXIII Hospital Bergamo Italy
Rio Hortega University Hospital Valladolid Spain
Royal Brompton and Harefield NHS Trust London United Kingdom
Royal Hospital for Children Glasgow United Kingdom
Sant Joan de Deu Barcelona Spain
Southampton General Hospital Southampton United Kingdom
The Children's Memorial Health Institute Warsaw Poland
The Freeman Hospital Newcastle United Kingdom
The Royal Children's Hospital Melbourne Victoria Australia
University Hospital La Paz Madrid Spain
University Hospital Motol Prague Czech Republic
University Hospital of Wales Cardiff United Kingdom
References provided by Crossref.org
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