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2015 ; 21
(11
): 877-84
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
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.