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Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy
G. Norrish, T. Ding, E. Field, E. Cervi, 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. Fernández, C. Marrone, R. Bökenkamp, A....
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, práce podpořená grantem
Grantová podpora
FS/16/72/32270
British Heart Foundation - United Kingdom
MR/T024062/1
Medical Research Council - United Kingdom
Department of Health - United Kingdom
NLK
Free Medical Journals
od 2008 do Před 1 rokem
Open Access Digital Library
od 2008-04-01
- MeSH
- defibrilátory implantabilní * škodlivé účinky MeSH
- dítě MeSH
- dospělí MeSH
- hodnocení rizik MeSH
- hypertrofická kardiomyopatie * komplikace diagnostické zobrazování MeSH
- hypertrofie levé komory srdeční komplikace diagnostické zobrazování MeSH
- lidé MeSH
- náhlá srdeční smrt epidemiologie etiologie MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- srdeční komory diagnostické zobrazování MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
BACKGROUND: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. METHODS: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). RESULTS: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. CONCLUSIONS: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.
Aalborg University Hospital Denmark
Aarhus University Hospital Denmark
Alder Hey Children's Hospital Liverpool United Kingdom
Bambino Gesu Hospital Rome Italy
Birmingham Children's Hospital United Kingdom
Bristol Royal Hospital for Children United Kingdom
Cardiology Unit S Orsola Malpighi Hospital IRCCS Azienda Ospedalierao Universitaria di Bologna Italy
Careggi University Hopsital Florence Italy
Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital London United Kingdom
Complexo Hospitalario Universitario A Coruna INIBIC CIBERCV Spain
Department of Pediatric Cardiology Charite Universitatsmedizin Berlin Germany
Department of Statistical Science University College London United Kingdom
Evelina Children's Hospital London United Kingdom
Fondazione Toscana G Monasterio Massa Pisa Italy
Fundación Favaloro University Hospital Buenos Aires Argentina
German Centre for Cardiovascular Research Partner Site Berlin Germany
Ghent University Hospital Belgium
Heart Muscle Disease Registry Trieste University of Trieste Italy
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 United Kingdom
John Radcliffe Hospital Oxford
Kochi Medical School Hospital Japan
Leeds General Infirmary United Kingdom
Leiden University Medical Center the Netherlands
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
Rio Hortega University Hospital Valladolid Spain
Royal Brompton and Harefield NHS Trust London United Kingdom
Royal Children's Hospital Melbourne Australia
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
University Hospital La Paz Madrid Spain
University Hospital Motol Prague Czech Republic
University Hospital of Wales Cardiff
Citace poskytuje Crossref.org
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