Tara Bharucha1, Katherine J Lee2, Piers E F Daubeney3, Alan W Nugent4, Christian Turner5, Gary F Sholler5, Terry Robertson6, Robert Justo7, Jim Ramsay8, John B Carlin2, Steven D Colan9, Ingrid King2, Robert G Weintraub10, Andrew M Davis11. 1. University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom. 2. Murdoch Children's Research Institute, Parkville, Victoria, Australia. 3. Imperial College and Royal Brompton Hospital, London, United Kingdom. 4. University of Texas Southwestern Medical Center, Dallas, Texas. 5. Children's Hospital at Westmead, Sydney, Australia. 6. Women's and Children's Hospital, Adelaide, Australia. 7. Mater Children's Hospital, Brisbane, Australia. 8. Princess Margaret Hospital for Children, Perth, Australia. 9. Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts. 10. Murdoch Children's Research Institute, Parkville, Victoria, Australia; Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. 11. Murdoch Children's Research Institute, Parkville, Victoria, Australia; Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia. Electronic address: andrew.davis@rch.org.au.
Abstract
BACKGROUND: Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear. OBJECTIVES: This study sought to determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM. METHODS: The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were <10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis. RESULTS: Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p=0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM. CONCLUSIONS: Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.
BACKGROUND:Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear. OBJECTIVES: This study sought to determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM. METHODS: The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were <10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis. RESULTS: Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p=0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM. CONCLUSIONS: Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.
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Authors: Melanie D Everitt; James D Wilkinson; Ling Shi; Jeffrey A Towbin; Steven D Colan; Paul F Kantor; Charles E Canter; Steven A Webber; Daphne T Hsu; Elfriede Pahl; Linda J Addonizio; Debra A Dodd; John L Jefferies; Joseph W Rossano; Brian Feingold; Stephanie M Ware; Teresa M Lee; Justin Godown; Kathleen E Simpson; Lynn A Sleeper; Jason D Czachor; Hiedy Razoky; Ashley Hill; Joslyn Westphal; Kimberly M Molina; Steven E Lipshultz Journal: Prog Pediatr Cardiol Date: 2019-03-07