Long-Term Outcomes of Childhood Left Ventricular Noncompaction Cardiomyopathy
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- William Y. Shi
- Department of Cardiac Surgery (W.Y.S.)
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- Margarita Moreno-Betancur
- Murdoch Children’s Research Institute, Melbourne, Australia (W.Y.S., M.M.-B., I.K., A.M.D., R.G.W.).
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- Alan W. Nugent
- Department of Pediatrics, University of Texas Southwestern, Dallas (A.W.N.).
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- Michael Cheung
- Department of Cardiology, Royal Children’s Hospital (M.C., A.M.D., R.G.W.)
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- Steven Colan
- Department of Cardiology, Boston Children’s Hospital, MA (S.C.).
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- Christian Turner
- Department of Cardiology, Children’s Hospital at Westmead, Sydney, Australia (C.T.).
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- Gary F. Sholler
- Department of Cardiology, Women’s and Children’s Hospital, Adelaide, Australia (G.F.S.).
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- Terry Robertson
- Department of Cardiology, Mater Children’s Hospital, Brisbane, Australia (T.R., R.J.).
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- Robert Justo
- Department of Cardiology, Mater Children’s Hospital, Brisbane, Australia (T.R., R.J.).
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- Andrew Bullock
- Department of Cardiology, Princess Margaret Hospital, Perth, Australia (A.B.).
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- Ingrid King
- Murdoch Children’s Research Institute, Melbourne, Australia (W.Y.S., M.M.-B., I.K., A.M.D., R.G.W.).
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- Andrew M. Davis
- Department of Cardiology, Royal Children’s Hospital (M.C., A.M.D., R.G.W.)
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- Piers E.F. Daubeney
- Department of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom (P.E.F.D.).
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- Robert G. Weintraub
- Department of Cardiology, Royal Children’s Hospital (M.C., A.M.D., R.G.W.)
Bibliographic Information
- Other Title
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- Results From a National Population-Based Study
Abstract
<jats:sec> <jats:title>Background:</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods:</jats:title> <jats:p>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.</jats:p> </jats:sec> <jats:sec> <jats:title>Results:</jats:title> <jats:p> 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]; <jats:italic>P</jats:italic> =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; <jats:italic>P</jats:italic> =0.0012). </jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions:</jats:title> <jats:p>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.</jats:p> </jats:sec>
Journal
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- Circulation
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Circulation 138 (4), 367-376, 2018-07-24
Ovid Technologies (Wolters Kluwer Health)
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Details 詳細情報について
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- CRID
- 1363107370799004800
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- ISSN
- 15244539
- 00097322
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- Data Source
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- Crossref