TY - JOUR
T1 - Long-term outcomes of childhood left ventricular noncompaction cardiomyopathy results from a national population-based study
AU - National Australian Childhood Cardiomyopathy Study
AU - Shi, William Y.
AU - Moreno-Betancur, Margarita
AU - Nugent, Alan
AU - Cheung, Michael
AU - Colan, Steven
AU - Turner, Christian
AU - Sholler, Gary F.
AU - Robertson, Terry
AU - Justo, Robert
AU - Bullock, Andrew
AU - King, Ingrid
AU - Davis, Andrew M.
AU - Daubeney, Piers E.F.
AU - Weintraub, Robert G.
N1 - Funding Information:
This work was supported by grant 98001 from the Royal Children's Hospital Research Foundation; grants G98M0159, G04M1586, G05M2151, G07M3180 from the National Heart Foundation of Australia; and NACCS grant from the Australia and New Zealand Children's Heart Research Center. Dr Shi is supported by the Royal Australasian College of Surgeons Foundation for Surgery Peter King/Heart Foundation Research Scholarship in addition to the University of Melbourne Viola Edith Reid and the R.G. and A.U. Meade Scholarships. Dr Daubeney's research was supported by the Biomedical Research Unit at the Royal Brompton Hospital. The Murdoch Children's Research Institute is supported by the Victorian Government's Operational Infrastructure Support Program.
Funding Information:
This work was supported by grant 98001 from the Royal Children’s Hospital Research Foundation; grants G98M0159, G04M1586, G05M2151, G07M3180 from the National Heart Foundation of Australia; and NACCS grant from the Australia and New Zealand Children’s Heart Research Center. Dr Shi is supported by the Royal Australasian College of Surgeons Foundation for Surgery Peter King/Heart Foundation Research Scholarship in addition to the University of Melbourne Viola Edith Reid and the R.G. and A.U. Meade Scholarships. Dr Daubeney’s research was supported by the Biomedical Research Unit at the Royal Brompton Hospital. The Murdoch Children’s Research Institute is supported by the Victorian Government’s Operational Infrastructure Support Program.
Publisher Copyright:
© 2018 American Heart Association, Inc.
PY - 2018
Y1 - 2018
N2 - BACKGROUND: Long-term outcomes for childhood left ventricular noncompaction (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 before 10 years of age between 1987 and 1996. Outcomes for subjects with LVNC with a dilated phenotype (LVNC-D) were compared with outcomes for those with dilated cardiomyopathy. 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 individuals. Congestive heart failure was the initial symptom in 24 of 29 subjects (83%), and 27 (93%) had LVNC-D. The median age at diagnosis was 0.3 (interquartile interval, 0.08-1.3) years. The median duration of follow-up was 6.8 (interquartile interval, 0.7-24.0) years for all subjects and 24.7 (interquartile interval, 23.3 - 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% confidence interval [CI], 30-65) at 10 years after diagnosis and 45% (95% CI, 27-63) at 15 years. In competing-risk analysis, 21% of subjects with LVNC were alive with normal left ventricular systolic function, and 31% were alive with abnormal function at 15 years. Propensity-score matching between subjects with LVNC-D and those with dilated cardiomyopathy suggested a lower freedom from death/transplantation at 15 years after diagnosis in the subjects with LVNC-D (LVNC-D, 46% [95% CI, 26-66] versus dilated cardiomyopathy, 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 (hazard ratio, 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 dilated cardiomyopathy.
AB - BACKGROUND: Long-term outcomes for childhood left ventricular noncompaction (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 before 10 years of age between 1987 and 1996. Outcomes for subjects with LVNC with a dilated phenotype (LVNC-D) were compared with outcomes for those with dilated cardiomyopathy. 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 individuals. Congestive heart failure was the initial symptom in 24 of 29 subjects (83%), and 27 (93%) had LVNC-D. The median age at diagnosis was 0.3 (interquartile interval, 0.08-1.3) years. The median duration of follow-up was 6.8 (interquartile interval, 0.7-24.0) years for all subjects and 24.7 (interquartile interval, 23.3 - 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% confidence interval [CI], 30-65) at 10 years after diagnosis and 45% (95% CI, 27-63) at 15 years. In competing-risk analysis, 21% of subjects with LVNC were alive with normal left ventricular systolic function, and 31% were alive with abnormal function at 15 years. Propensity-score matching between subjects with LVNC-D and those with dilated cardiomyopathy suggested a lower freedom from death/transplantation at 15 years after diagnosis in the subjects with LVNC-D (LVNC-D, 46% [95% CI, 26-66] versus dilated cardiomyopathy, 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 (hazard ratio, 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 dilated cardiomyopathy.
KW - Cardiomyopathy
KW - Left ventricular noncompaction
KW - Long-term follow-up
KW - Survival
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U2 - 10.1161/CIRCULATIONAHA.117.032262
DO - 10.1161/CIRCULATIONAHA.117.032262
M3 - Article
C2 - 29514799
AN - SCOPUS:85058882666
SN - 0009-7322
VL - 138
SP - 367
EP - 376
JO - Circulation
JF - Circulation
IS - 4
ER -