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Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy
A T Yetman1, R M Hamilton, L N Benson
1Department of Pediatrics, The Hospital for Sick Children, Ontario, Toronto, Canada.
Insights
Sudden death in children with hypertrophic cardiomyopathy (HCM) can be predicted by specific ECG findings, ventricular tachycardia, and coronary artery abnormalities. Early identification of these risk factors is crucial for timely intervention.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Genetics
- Sudden Cardiac Death Research
Background:
- Hypertrophic cardiomyopathy (HCM) in children poses a significant risk of sudden death, which is challenging to predict.
- Identifying high-risk factors in pediatric HCM is essential for improving patient outcomes and guiding therapeutic strategies.
Purpose of the Study:
- To identify clinical, angiographic, and echocardiographic predictors of survival in pediatric patients with isolated hypertrophic cardiomyopathy.
- To establish factors associated with reduced survival and increased risk of sudden death in children diagnosed with HCM.
Main Methods:
- Retrospective review of 99 pediatric patients diagnosed with HCM between 1958 and 1997.
- Analysis of clinical data, coronary angiography (n=62), and echocardiography (n=83) to assess survival predictors.
- Application of Cox's proportionate hazard modeling to evaluate the impact of clinical characteristics on sudden death events.
Main Results:
- Increased corrected QT interval (QTc) dispersion on ECG (RR 1.61), ventricular tachycardia (VT) on ambulatory ECG (RR 3.75), and myocardial bridging of the LAD coronary artery (RR 12.0) were significant predictors of reduced survival.
- A total of 18 out of 97 patients experienced death or resuscitated sudden death, with a sudden death rate of 2.7%/year after age 8.
- Male sex (71 subjects) and a family history of HCM (37/97 patients) were noted, with a median age at diagnosis of 5.0 years.
Conclusions:
- Detailed electrocardiogram (ECG) assessments, including ambulatory ECG monitoring, are vital for risk stratification in pediatric HCM.
- Coronary angiography revealing myocardial bridging is a strong indicator of increased risk for sudden death in children with HCM.
- Integrating these diagnostic tools can significantly aid in identifying children with HCM who are at high risk for sudden cardiac events.
Objectives:
We sought to determine clinical, angiographic, and echocardiographic predictors of survival in children with isolated hypertrophic cardiomyopathy (HCM) in a large pediatric centre.
Background:
Sudden death is a catastrophic outcome of HCM in childhood but has been difficult to predict. Current therapies might provide for improved outcome if factors identifying high risk can be identified.
Methods:
Records of 99 patients diagnosed with HCM from 1958 to 1997 at <18 yr were reviewed for clinical, angiographic (n = 62) and echocardiographic (n = 83) predictors of survival outcome. The effects of clinical characteristics on sudden death (including resuscitated sudden death) were individually tested in Cox's proportionate hazard modeling.
Results:
Seventy-one subjects were male. Median age at diagnosis was 5.0 yr with a medical follow-up interval of 4.8 yr. Thirty-seven of 97 patients had a family history of HCM. Ambulatory electrocardiograms (ECG) in 78 patients demonstrated supraventricular tachycardia in 16 and ventricular tachycardia in 21. Death or resuscitated sudden death occurred in 18 patients. Sudden death rate was 2.7%/yr after age 8 yr. Cox's proportionate survival modeling revealed increased corrected QT interval (QTc) dispersion on ECG (relative risk [RR] 1.61 per 20 ms increment, p < 0.0003), ventricular tachycardia (VT) on ambulatory ECG (RR 3.75, p < 0.006) and myocardial bridging of the LAD coronary (RR 12.0, p < 0.003) to be associated with reduced time to death or resuscitated sudden death.
Conclusions:
Detailed assessment of ECGs, ambulatory ECGs, and coronary angiography can assist in identifying which children with HCM are at risk for sudden death.