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@article{Shi:2018:10.1161/CIRCULATIONAHA.117.032262,
author = {Shi, WY and Moreno-Betancur, M and Nugent, AW and Cheung, M and Colan, S and Turner, C and Sholler, GF and Robertson, T and Justo, R and Bullock, A and King, I and Davis, AM and Daubeney, PEF and Weintraub, RG and for, the National Australian Childhood Cardiomyopathy Study},
doi = {10.1161/CIRCULATIONAHA.117.032262},
journal = {Circulation},
pages = {138--367},
title = {Long-term outcomes of childhood left ventricular non-compaction cardiomyopathy: results from a national population-based study},
url = {http://dx.doi.org/10.1161/CIRCULATIONAHA.117.032262},
volume = {138},
year = {2018}
}

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TY  - JOUR
AB  - 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 usu
AU  - Shi,WY
AU  - Moreno-Betancur,M
AU  - Nugent,AW
AU  - Cheung,M
AU  - Colan,S
AU  - Turner,C
AU  - Sholler,GF
AU  - Robertson,T
AU  - Justo,R
AU  - Bullock,A
AU  - King,I
AU  - Davis,AM
AU  - Daubeney,PEF
AU  - Weintraub,RG
AU  - for,the National Australian Childhood Cardiomyopathy Study
DO  - 10.1161/CIRCULATIONAHA.117.032262
EP  - 367
PY  - 2018///
SN  - 0009-7322
SP  - 138
TI  - Long-term outcomes of childhood left ventricular non-compaction cardiomyopathy: results from a national population-based study
T2  - Circulation
UR  - http://dx.doi.org/10.1161/CIRCULATIONAHA.117.032262
UR  - https://www.ncbi.nlm.nih.gov/pubmed/29514799
UR  - http://hdl.handle.net/10044/1/57929
VL  - 138
ER  -

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