Victor Nauffal1, Peter Marstrand2, Larry Han3, Victoria N Parikh4, Adam S Helms5, Jodie Ingles6,7, Daniel Jacoby8, Neal K Lakdawala1, Sunil Kapur1, Michelle Michels9, Anjali T Owens10, Euan A Ashley11, Alexandre C Pereira12, Joseph W Rossano13, Sara Saberi5, Christopher Semsarian7,14, James S Ware15,16, Samuel G Wittekind17,18, Sharlene Day10, Iacopo Olivotto19, Carolyn Y Ho1. 1. Department of Medicine, Brigham and Women's Hospital, Cardiovascular Medicine Division, 75 Francis Street, Boston, MA 02115, USA. 2. Department of Cardiology, Herlev-Gentofte Hospital, University Hospital of Copenhagen, Gentofte Hospitalsvej 1, Hellerup 2900, Denmark. 3. Department of Biostatistics, Harvard T.H. Chan School of Public Health, 677 Huntington Ave, Boston, MA 02115, USA. 4. Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, 291 Campus Drive, Stanford, CA 94305, USA. 5. Department of Medicine, Cardiovascular Medicine Division, University of Michigan, 1500 E Medical Center Dr, Ann Arbor, MI 48109, USA. 6. Department of Cardiology, Cardio Genomics Program at Centenary Institute, The University of Sydney, Royal Prince Alfred Hospital, Missenden Rd, Sydney NSW 2050, Australia. 7. Department of Cardiology, Royal Prince Alfred Hospital, Missenden Rd, Sydney NSW 2050, Australia. 8. Department of Internal Medicine, Section of Cardiovascular Medicine, Yale University, 20 York St, New Haven, CT 06510, USA. 9. Department of Cardiology, Thoraxcenter, Erasmus, Dr. Molewaterplein 40, Rotterdam 3015 GD, the Netherlands. 10. Division of Cardiovascular Medicine, Department of Medicine, Center for Inherited Cardiovascular Disease, University of Pennsylvania Perelman School of Medicine, 3400 Spruce St, Philadelphia, PA 19104, USA. 11. Division of Cardiovascular Medicine, Department of Medicine, Stanford Center for Inherited Cardiovascular Disease, Stanford University School of Medicine, 291 Campus Drive, Stanford, CA 94305, USA. 12. Department of Cardiology, Heart Institute (InCor), University of Sao Paulo Medical School, Av. Dr. Enéas Carvalho de Aguiar, 44 - Cerqueira César, São Paulo - SP, 05403-900, Brazil. 13. Department of Pediatrics, Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA. 14. Department of Cardiology, Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Australia. 15. Department of Medicine, National Heart & Lung Institute & MRC London Institute of Medical Sciences, Imperial College London, Du Cane Rd, London W12 0NN, UK. 16. Division of Cardiovascular Medicine, Department of Medicine, Royal Brompton & Harefield Hospitals, Sydney St, London SW3 6NP, UK. 17. Department of Pediatrics, University of Cincinnati College of Medicine, 3230 Eden Ave, Cincinnati, OH 45267, USA. 18. The Heart Institute, Cincinnati Children's, 3333 Burnet Ave, Cincinnati, OH 45229, USA. 19. Department of Experimental and Clinical Medicine, Careggi University Hospital, Largo Giovanni Alessandro Brambilla, 3, 50134 Firenze FI, Italy.
Abstract
AIMS: Risk stratification algorithms for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) and regional differences in clinical practice have evolved over time. We sought to compare primary prevention implantable cardioverter defibrillator (ICD) implantation rates and associated clinical outcomes in US vs. non-US tertiary HCM centres within the international Sarcomeric Human Cardiomyopathy Registry. METHODS AND RESULTS: We included patients with HCM enrolled from eight US sites (n = 2650) and five non-US (n = 2660) sites and used multivariable Cox-proportional hazards models to compare outcomes between sites. Primary prevention ICD implantation rates in US sites were two-fold higher than non-US sites (hazard ratio (HR) 2.27 [1.89-2.74]), including in individuals deemed at high 5-year SCD risk (≥6%) based on the HCM risk-SCD score (HR 3.27 [1.76-6.05]). US ICD recipients also had fewer traditional SCD risk factors. Among ICD recipients, rates of appropriate ICD therapy were significantly lower in US vs. non-US sites (HR 0.52 [0.28-0.97]). No significant difference was identified in the incidence of SCD/resuscitated cardiac arrest among non-recipients of ICDs in US vs. non-US sites (HR 1.21 [0.74-1.97]). CONCLUSION: Primary prevention ICDs are implanted more frequently in patients with HCM in US vs. non-US sites across the spectrum of SCD risk. There was a lower rate of appropriate ICD therapy in US sites, consistent with a lower-risk population, and no significant difference in SCD in US vs. non-US patients who did not receive an ICD. Further studies are needed to understand what drives malignant arrhythmias, optimize ICD allocation, and examine the impact of different ICD utilization strategies on long-term outcomes in HCM. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: Risk stratification algorithms for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) and regional differences in clinical practice have evolved over time. We sought to compare primary prevention implantable cardioverter defibrillator (ICD) implantation rates and associated clinical outcomes in US vs. non-US tertiary HCM centres within the international Sarcomeric Human Cardiomyopathy Registry. METHODS AND RESULTS: We included patients with HCM enrolled from eight US sites (n = 2650) and five non-US (n = 2660) sites and used multivariable Cox-proportional hazards models to compare outcomes between sites. Primary prevention ICD implantation rates in US sites were two-fold higher than non-US sites (hazard ratio (HR) 2.27 [1.89-2.74]), including in individuals deemed at high 5-year SCD risk (≥6%) based on the HCM risk-SCD score (HR 3.27 [1.76-6.05]). US ICD recipients also had fewer traditional SCD risk factors. Among ICD recipients, rates of appropriate ICD therapy were significantly lower in US vs. non-US sites (HR 0.52 [0.28-0.97]). No significant difference was identified in the incidence of SCD/resuscitated cardiac arrest among non-recipients of ICDs in US vs. non-US sites (HR 1.21 [0.74-1.97]). CONCLUSION: Primary prevention ICDs are implanted more frequently in patients with HCM in US vs. non-US sites across the spectrum of SCD risk. There was a lower rate of appropriate ICD therapy in US sites, consistent with a lower-risk population, and no significant difference in SCD in US vs. non-US patients who did not receive an ICD. Further studies are needed to understand what drives malignant arrhythmias, optimize ICD allocation, and examine the impact of different ICD utilization strategies on long-term outcomes in HCM. Published on behalf of the European Society of Cardiology. All rights reserved.
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Authors: Carolyn Y Ho; Sharlene M Day; Euan A Ashley; Michelle Michels; Alexandre C Pereira; Daniel Jacoby; Allison L Cirino; Jonathan C Fox; Neal K Lakdawala; James S Ware; Colleen A Caleshu; Adam S Helms; Steven D Colan; Francesca Girolami; Franco Cecchi; Christine E Seidman; Gautam Sajeev; James Signorovitch; Eric M Green; Iacopo Olivotto Journal: Circulation Date: 2018-08-23 Impact factor: 29.690