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Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center
Samuel T Brescia1, Joseph W Rossano, Ricardo Pignatelli
1Texas Children's Hospital/Section of Pediatric Cardiology, 6621 Fannin, Houston, TX 77030, USA. jjkim@texaschildrens.org
Circulation
|May 2, 2013
Summary
Left ventricular noncompaction (LVNC) in children has high mortality, often linked to arrhythmias and cardiac dysfunction. Normal cardiac function and dimensions in pediatric LVNC patients indicate a low risk for sudden cardiac death.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Genetics
Background:
- Left ventricular noncompaction (LVNC) is a rare cardiomyopathy.
- It is characterized by excessive left ventricular trabeculation, myocardial dysfunction, and high mortality.
- Clinical presentations of LVNC are diverse, including arrhythmias and sudden cardiac death.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of children diagnosed with isolated left ventricular noncompaction.
- To identify factors associated with mortality and sudden cardiac death in this pediatric population.
Main Methods:
- Retrospective review of pediatric patients diagnosed with isolated left ventricular noncompaction (LVNC) between January 1990 and January 2009.
- Exclusion of patients with congenital cardiac lesions.
- Analysis of clinical data, including cardiac function, ECG abnormalities, arrhythmias, and mortality.
Main Results:
- 242 children were diagnosed with isolated LVNC.
- Mortality was 12.8%, and 5.4% received a transplant.
- Cardiac dysfunction (62%) and arrhythmias (33.1%) were strongly associated with increased mortality.
- ECG abnormalities were present in 87%, with repolarization abnormalities linked to higher mortality.
- Sudden cardiac death occurred in 6.2% of patients, primarily those with abnormal cardiac dimensions or dysfunction.
Conclusions:
- Left ventricular noncompaction (LVNC) carries a high mortality rate in children.
- Cardiac dysfunction and ventricular arrhythmias are significant predictors of mortality in pediatric LVNC.
- Children with normal cardiac dimensions and function without preceding arrhythmias have a low risk of sudden death.
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