TY - JOUR
T1 - Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction
T2 - Results From the Pediatric Cardiomyopathy Registry
AU - Pediatric Cardiomyopathy Registry Investigators
AU - Jefferies, John L.
AU - Wilkinson, James D.
AU - Sleeper, Lynn A.
AU - Colan, Steven D.
AU - Lu, Minmin
AU - Pahl, Elfriede
AU - Kantor, Paul F.
AU - Everitt, Melanie D.
AU - Webber, Steven A.
AU - Kaufman, Beth D.
AU - Lamour, Jacqueline M.
AU - Canter, Charles E.
AU - Hsu, Daphne T.
AU - Addonizio, Linda J.
AU - Lipshultz, Steven E.
AU - Towbin, Jeffrey A.
N1 - Funding Information:
Funding: National Heart, Lung, and Blood Institute (NHLBI; grants R01 HL53392 to S.E.L. and R01 HL087000 to J.A.T.) and the Children's Cardiomyopathy Foundation (CCF; to S.E.L.). No NHLBI or CCF representatives were involved in the drafting of this manuscript. The contents of this publication are solely the responsibility of the authors and do not necessarily represent the official views of the NHLBI or CCF.
Funding Information:
The authors thank the participating centers for subject recruitment and follow-up data collection. They also thank the Children's Cardiomyopathy Foundation for their ongoing support of the Pediatric Cardiomyopathy Registry's research efforts.
Publisher Copyright:
© 2015 Elsevier Inc.
PY - 2015/11
Y1 - 2015/11
N2 - 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.
AB - 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.
KW - Cardiomyopathy
KW - heart failure
KW - pediatrics
UR - http://www.scopus.com/inward/record.url?scp=84946473891&partnerID=8YFLogxK
U2 - 10.1016/j.cardfail.2015.06.381
DO - 10.1016/j.cardfail.2015.06.381
M3 - Article
C2 - 26164213
AN - SCOPUS:84946473891
SN - 1071-9164
VL - 21
SP - 877
EP - 884
JO - Journal of Cardiac Failure
JF - Journal of Cardiac Failure
IS - 11
ER -