Long-term outcomes of childhood left ventricular non-compaction cardiomyopathy: results from a national population-based study

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Title: Long-term outcomes of childhood left ventricular non-compaction cardiomyopathy: results from a national population-based study
Author(s): Shi, WY
Moreno-Betancur, M
Nugent, AW
Cheung, M
Colan, S
Turner, C
Sholler, GF
Robertson, T
Justo, R
Bullock, A
King, I
Davis, AM
Daubeney, PEF
Weintraub, RG
For the National Australian Childhood Cardiomyopathy Study
Item Type: Journal Article
Abstract: Background -Long-term outcomes for childhood left ventricular non-compaction (LVNC) are uncertain. We examined late outcomes for children with LVNC enrolled in a national population-based study. Methods -The National Australian Childhood Cardiomyopathy Study includes all children in Australia with primary cardiomyopathy diagnosed <10 years of age between 1987 and 1996. Outcomes for LVNC subjects with a dilated phenotype (LVNC-D) were compared to those with dilated cardiomyopathy (DCM). Propensity-score analysis was used for risk factor adjustment. Results -There were 29 subjects with LVNC (9.2% of all cardiomyopathy subjects) with a mean annual incidence of newly diagnosed cases of 0.11 per 100,000 at-risk persons. Congestive heart failure was the initial symptom in 24 (83%) of 29 subjects, and 27 (93%) had a dilated phenotype (LVNC-D). The median age at diagnosis was 0.3 (interquartile interval 0.08 - 1.3) years of age. The median (interquartile interval) duration of follow-up was 6.8 (0.7-14.1) years for all subjects and 24.7 (23.3 - 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% CI 30 - 65%) at 10 years after diagnosis and 45% (95% CI 27-63%) at 15 years. By competing risk analysis, 21% of LVNC subjects were alive with normal LV systolic function and 31% were alive with abnormal function at 15 years. Propensity-score matching between LVNC-D and DCM subjects suggested a lower freedom from death/transplantation at 15 years after diagnosis in the LVNC-D subjects (LVNC-D: 46% (95% CI 26-66%) vs. DCM: 70% (95% CI 42-97%), p=0.08). Using propensity-score inverse probability of treatment weighted Cox regression, we found evidence that LVNC-D was associated with a greater risk of death or transplantation (HR 2.3, 95% CI 1.4-3.8, p=0.0012). Conclusions -Symptomatic children with LVNC usually present in early infancy with a predominant dilated phenotype. Long-term outcomes are worse than for matched children with DCM.
Publication Date: 24-Jul-2018
Date of Acceptance: 22-Feb-2018
URI: http://hdl.handle.net/10044/1/57929
DOI: https://dx.doi.org/10.1161/CIRCULATIONAHA.117.032262
ISSN: 0009-7322
Publisher: American Heart Association
Start Page: 138
End Page: 367
Journal / Book Title: Circulation
Volume: 138
Copyright Statement: ©2018 American Heart Association, Inc. All rights reserved.
Keywords: Science & Technology
Life Sciences & Biomedicine
Cardiac & Cardiovascular Systems
Peripheral Vascular Disease
Cardiovascular System & Cardiology
left ventricular noncompaction
long-term Follow-up
left ventricular non-compaction
long-term follow-up
National Australian Childhood Cardiomyopathy Study
left ventricular non-compaction
long-term follow-up
1103 Clinical Sciences
1102 Cardiovascular Medicine And Haematology
1117 Public Health And Health Services
Cardiovascular System & Hematology
Publication Status: Published
Conference Place: United States
Online Publication Date: 2018-03-07
Appears in Collections:National Heart and Lung Institute
Faculty of Medicine

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