Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry.

摘要: Abstract Background Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation the left ventricle. The LVNC phenotype may occur in isolation or with other phenotypes. Prognosis incompletely children. Methods and Results According to diagnoses from National Heart, Lung, Blood Institute–funded Pediatric Cardiomyopathy Registry 1990 2008, 155 3,219 children (4.8%) had LVNC. Each patient was also classified as having an associated echocardiographically diagnosed phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, indeterminate. time death transplantation differed among phenotypic groups ( P  = .035). Time listing for cardiac significantly .015). hazard ratio death/transplantation (with isolated reference group) 4.26 (95% confidence interval [CI] 0.78–23.3) HCM, 6.35 CI 1.52–26.6) DCM, 5.66 1.04–30.9) indeterminate phenotype. Most events occurred 1st year after diagnosis. Conclusions present at least 5% cardiomyopathy. specific LVNC-associated predicts risk should inform clinical management.