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Updated: Feb 13, 2026
Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Long-Term Outcomes of Childhood Left Ventricular Noncompaction Cardiomyopathy: Results From a National
William Y Shi1,2,3,4, Margarita Moreno-Betancur3,4, Alan W Nugent5
1Department of Cardiac Surgery (W.Y.S.).
Insights
Childhood left ventricular noncompaction (LVNC) predominantly presents as a dilated heart condition in infancy. Long-term outcomes for these children are poorer compared to those with dilated cardiomyopathy.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Genetics of Heart Disease
Background:
- Childhood left ventricular noncompaction (LVNC) is a rare congenital heart defect with uncertain long-term prognosis.
- Early diagnosis and understanding of LVNC are crucial for effective management and improved patient outcomes.
Purpose of the Study:
- To investigate the long-term outcomes of children diagnosed with left ventricular noncompaction (LVNC).
- To compare the outcomes of LVNC patients with a dilated phenotype (LVNC-D) against those with dilated cardiomyopathy.
Main Methods:
- Utilized data from the National Australian Childhood Cardiomyopathy Study (1987-1996).
- Compared outcomes of LVNC patients with dilated cardiomyopathy using propensity-score analysis for risk adjustment.
- Employed competing-risk analysis and Cox regression to assess mortality and transplantation risks.
Main Results:
- LVNC affected 9.2% of cardiomyopathy cases, with a median age at diagnosis of 0.3 years.
- Congestive heart failure was the primary symptom in 83% of LVNC cases, with 93% exhibiting a dilated phenotype (LVNC-D).
- At 15 years post-diagnosis, freedom from death or transplantation was 45% for LVNC, and LVNC-D showed a significantly increased risk of death or transplantation (HR 2.3, P=0.0012).
Conclusions:
- Symptomatic children with LVNC typically present with a dilated phenotype in early infancy.
- Long-term outcomes for childhood LVNC, particularly the dilated phenotype, are worse than for matched cases of dilated cardiomyopathy.
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.
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