John L Jefferies1, James D Wilkinson2, Lynn A Sleeper3, Steven D Colan4, Minmin Lu3, Elfriede Pahl5, Paul F Kantor6, Melanie D Everitt7, Steven A Webber8, Beth D Kaufman9, Jacqueline M Lamour10, Charles E Canter11, Daphne T Hsu10, Linda J Addonizio12, Steven E Lipshultz13, Jeffrey A Towbin14. 1. Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address: john.jefferies@cchmc.org. 2. Department of Pediatrics, Wayne State University School of Medicine, Detroit, Michigan; Department of Pediatrics, University of Miami Miller School of Medicine and Holtz Children's Hospital, Miami, Florida. 3. New England Research Institutes, Watertown, Massachusetts. 4. Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts. 5. Division of Cardiology, Ann and Robert Lurie Children's Hospital, Chicago, Illionis. 6. Division of Pediatric Cardiology, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada. 7. Division of Pediatric Cardiology, Primary Children's Hospital, Salt Lake City, Utah. 8. Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee. 9. Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. 10. Department of Pediatrics, Montefiore Children's Hospital, Bronx, New York. 11. Division of Pediatric Cardiology, St. Louis Children's Hospital, St. Louis, Missouri. 12. Division of Pediatric Cardiology, Morgan Stanley Children's Hospital, New York, New York. 13. Department of Pediatrics, University of Miami Miller School of Medicine and Holtz Children's Hospital, Miami, Florida; Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit, Michigan. 14. Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Heart Institute, Le Bonheur Children's Hospital, University of Tennessee Health Science Center, Memphis, Tennessee.
Abstract
BACKGROUND: Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children. METHODS AND RESULTS: According to diagnoses from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC. Each LVNC patient was also classified as having an associated echocardiographically diagnosed cardiomyopathy phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, or indeterminate. The time to death or transplantation differed among the phenotypic groups (P = .035). Time to listing for cardiac transplantation significantly differed by phenotype (P < .001), as did time to transplantation (P = .015). The hazard ratio for death/transplantation (with isolated LVNC as the reference group) was 4.26 (95% confidence interval [CI] 0.78-23.3) for HCM, 6.35 (95% CI 1.52-26.6) for DCM, and 5.66 (95% CI 1.04-30.9) for the indeterminate phenotype. Most events occurred in the 1st year after diagnosis. CONCLUSIONS: LVNC is present in at least 5% of children with cardiomyopathy. The specific LVNC-associated cardiomyopathy phenotype predicts the risk of death or transplantation and should inform clinical management.
BACKGROUND: Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children. METHODS AND RESULTS: According to diagnoses from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC. Each LVNCpatient was also classified as having an associated echocardiographically diagnosed cardiomyopathy phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, or indeterminate. The time to death or transplantation differed among the phenotypic groups (P = .035). Time to listing for cardiac transplantation significantly differed by phenotype (P < .001), as did time to transplantation (P = .015). The hazard ratio for death/transplantation (with isolated LVNC as the reference group) was 4.26 (95% confidence interval [CI] 0.78-23.3) for HCM, 6.35 (95% CI 1.52-26.6) for DCM, and 5.66 (95% CI 1.04-30.9) for the indeterminate phenotype. Most events occurred in the 1st year after diagnosis. CONCLUSIONS:LVNC is present in at least 5% of children with cardiomyopathy. The specific LVNC-associated cardiomyopathy phenotype predicts the risk of death or transplantation and should inform clinical management.
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