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Inverted U-Shaped Relation Between the Risk of Sudden Cardiac Death and Maximal Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy

O'Mahony, C; Jichi, F; Monserrat, L; Ortiz-Genga, M; Anastasakis, A; Rapezzi, C; Biagini, E; ... Elliott, PM; + view all (2016) Inverted U-Shaped Relation Between the Risk of Sudden Cardiac Death and Maximal Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy. Circulation: Arrhythmia and Electrophysiology , 9 (6) , Article e003818. 10.1161/CIRCEP.115.003818. Green open access

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Abstract

BACKGROUND: Hypertrophic cardiomyopathy is associated with sudden cardiac death (SCD). Some studies have shown an association between risk of sudden death and left ventricular maximal wall thickness (MWT), but there are few data in patients with extreme hypertrophy. The aim of this study was to determine the relation between MWT and the risk of SCD. METHODS AND RESULTS: This is a multicenter, retrospective, longitudinal cohort study of 3673 adult (≥16 years) patients, previously used to develop and validate a risk prediction model for SCD (HCM Risk-SCD [hypertrophic cardiomyopathy risk-SCD]). There was an inverted U-shaped relation between MWT and the estimated 5-year risk of SCD. In patients with MWT≥35 mm (n=47; mean age, 33 years; 81% men), there was a single SCD end point (annual rate, 0.2%; 95% confidence interval, 0.03–1.60) and 3 additional cardiovascular events during a median follow-up of 9.5 years. Compared with patients with MWT≤14 mm, those with MWT≥35 mm did not have a higher risk for SCD (hazard ratio, 0.22; 95% confidence interval, 0.03–1.65), cardiovascular death (hazard ratio, 0.66; 95% confidence interval, 0.26–1.67), or all-cause mortality (hazard ratio, 0.73; 95% confidence interval, 0.32–1.69). CONCLUSIONS: The risk of SCD has a complex, nonlinear relationship to MWT. The pathophysiological mechanisms behind this observation require further study but implantable cardioverter defibrillator implantation should not be guided solely on the severity of left ventricular hypertrophy.

Type: Article
Title: Inverted U-Shaped Relation Between the Risk of Sudden Cardiac Death and Maximal Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy
Open access status: An open access version is available from UCL Discovery
DOI: 10.1161/CIRCEP.115.003818
Publisher version: http://dx.doi.org/10.1161/CIRCEP.115.003818
Language: English
Additional information: Copyright © 2016 by American Heart Association, Inc. All rights reserved.
Keywords: Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, Cardiomyopathy, Hypertrophic, Death, Sudden, Defibrillators, Implantable, Follow-Up Studies, Hypertrophy, Left Ventricular, Myosin Heavy-Chain, Outflow Tract Obstruction, Binding-Protein-C, Task-Force, Practice Guidelines, European-Society, Gene-Mutations, Phenotype, Diagnosis, Genotype
UCL classification: UCL > Provost and Vice Provost Offices
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 Pop Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > Institute of Cardiovascular Science > Clinical Science
UCL > Provost and Vice Provost Offices > UCL BEAMS
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Statistical Science
UCL > Provost and Vice Provost Offices > VP Health
UCL > Provost and Vice Provost Offices > VP Health > Clinical Research Support Centre
UCL > Provost and Vice Provost Offices > VP Health > Clinical Research Support Centre > Joint Research Office
URI: https://discovery.ucl.ac.uk/id/eprint/1493835
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