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10.1016/j.cardfail.2015.06.381

http://scihub22266oqcxt.onion/10.1016/j.cardfail.2015.06.381
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suck abstract from ncbi


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pmid26164213
      J+Card+Fail 2015 ; 21 (11 ): 877-84
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  • Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry #MMPMID26164213
  • Jefferies JL ; Wilkinson JD ; Sleeper LA ; Colan SD ; Lu M ; Pahl E ; Kantor PF ; Everitt MD ; Webber SA ; Kaufman BD ; Lamour JM ; Canter CE ; Hsu DT ; Addonizio LJ ; Lipshultz SE ; Towbin JA
  • J Card Fail 2015[Nov]; 21 (11 ): 877-84 PMID26164213 show ga
  • 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.
  • |*Phenotype [MESH]
  • |*Registries [MESH]
  • |Canada [MESH]
  • |Cardiomyopathies/*genetics/*mortality/physiopathology [MESH]
  • |Cause of Death [MESH]
  • |Child [MESH]
  • |Child, Preschool [MESH]
  • |Female [MESH]
  • |Genetic Predisposition to Disease/epidemiology [MESH]
  • |Humans [MESH]
  • |Incidence [MESH]
  • |Isolated Noncompaction of the Ventricular Myocardium/*genetics/*mortality/physiopathology [MESH]
  • |Kaplan-Meier Estimate [MESH]
  • |Male [MESH]
  • |Multivariate Analysis [MESH]
  • |Pediatrics [MESH]
  • |Predictive Value of Tests [MESH]
  • |Prognosis [MESH]
  • |Proportional Hazards Models [MESH]
  • |Retrospective Studies [MESH]
  • |Survival Analysis [MESH]


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