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Updated: Oct 4, 2025
Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk
Barbara Cardoso1, Aamir Jeewa1, Sunghoon Minn1
1Division of Cardiology, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Left ventricular noncompaction (LVNC) in children is linked to outcomes. Baseline left ventricular dilation and systolic dysfunction predict the risk of death or heart transplantation (HTx) in pediatric LVNC patients.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Genetics
Background:
- Left ventricular noncompaction (LVNC) presents with significant genetic and phenotypic variability, impacting patient outcomes.
- Understanding risk factors for adverse events is crucial for managing pediatric LVNC cases.
Purpose of the Study:
- To identify risk factors for death or heart transplantation (HTx) in a cohort of pediatric patients diagnosed with LVNC.
- To analyze the association between baseline cardiac function and adverse outcomes in LVNC.
Main Methods:
- Retrospective review of pediatric patients (<18 years) diagnosed with LVNC (isolated or dilated phenotype) or dilated cardiomyopathy (DCM) between 2001 and 2018.
- Statistical analysis including descriptive statistics, multivariate analysis, and time-to-event analysis to determine predictors of death or HTx.
Main Results:
- The study included 188 patients: 34 with isolated LVNC (I-LVNC), 37 with dilated LVNC (D-LVNC), and 117 with DCM.
- Overall incidence of death or HTx was 43.6%. 10-year survival free from death/HTx was 88.6% for I-LVNC, 47% for D-LVNC, and 42.3% for DCM.
- Multivariable analysis identified baseline left ventricular ejection fraction (LVEF) and LV end-diastolic diameter (LVEDD) z-score as significant predictors of death or HTx.
Conclusions:
- Baseline left ventricular dilation and systolic dysfunction are independently associated with an increased risk of progression to death or heart transplantation in pediatric LVNC patients.
- Early identification of LV dilation (LVEDD z-score > 4) and systolic dysfunction is critical for risk stratification and management in pediatric LVNC.
Background:
Left ventricular noncompaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplantation (HTx) in a paediatric LVNC cohort.
Methods:
We reviewed patients < 18 years of age (2001-2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC), and at least mildly reduced left ventricular ejection fraction (EF). Patients with dilated cardiomyopathy (DCM) were included as control subjects. Descriptive statistics, multivariate analysis, and time-to-event analysis were used.
Results:
We included 188 patients, 34 (18%) with I-LVNC, 37 (20%) with D-LVNC, and 117 (62%) with DCM. Overall median age at diagnosis was 1.08 years (interquartile range [IQR] 0.22-10.65) and median follow-up was 1.4 years (IQR 0.2-5.2) years. I-LVNC patients' median baseline LVEF was 47%, compared with 33% for D-LVNC, and 21% for DCM (P < 0.0001); 62% of I-LVNC patients developed moderate to severe LV dysfunction during follow-up. The incidence of death or transplantation was 43.6% in the overall cohort. Freedom from death or HTx at 10 years after diagnosis was 88.6% (95% CI 76%-100%) for I-LVNC, 47% (95% CI 29%-65%) for D-LVNC, and 42.3% (95% CI 33%-52%) for DCM. On multivariable analysis, baseline LVEF and LV end-diastolic diameter (LVEDD) z-score were associated with death or transplantation. Patients with a baseline LVEDD z-score > 4 and moderate to severe LV dysfunction had a transplantation-free survival of 38%.
Conclusions:
Baseline LV dilation and systolic dysfunction were independently associated with progression to death or HTx in LVNC patients.
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