Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction

Results From the Pediatric Cardiomyopathy Registry

Pediatric Cardiomyopathy Registry Investigators

Research output: Contribution to journalArticle

37 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)877-884
Number of pages8
JournalJournal of Cardiac Failure
Volume21
Issue number11
DOIs
StatePublished - Nov 1 2015

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Cardiomyopathies
Registries
Pediatrics
Phenotype
Transplantation
Confidence Intervals
National Heart, Lung, and Blood Institute (U.S.)
Dilated Cardiomyopathy
Heart Transplantation
Heart Ventricles

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

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

In: Journal of Cardiac Failure, Vol. 21, No. 11, 01.11.2015, p. 877-884.

Research output: Contribution to journalArticle

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title = "Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry",
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.",
author = "{Pediatric Cardiomyopathy Registry Investigators} and John Jefferies and Wilkinson, {James D.} and Sleeper, {Lynn A.} and Colan, {Steven D.} and Minmin Lu and Elfriede Pahl and Kantor, {Paul F.} and Everitt, {Melanie D.} and Webber, {Steven A.} and Kaufman, {Beth D.} and Lamour, {Jacqueline M.} and Canter, {Charles E.} and Hsu, {Daphne T.} and Addonizio, {Linda J.} and Lipshultz, {Steven E.} and Jeffrey Towbin",
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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

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

PY - 2015/11/1

Y1 - 2015/11/1

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.

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JO - Journal of Cardiac Failure

JF - Journal of Cardiac Failure

SN - 1071-9164

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