BibTex format
@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}
}
RIS format (EndNote, RefMan)
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 -