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QT intervals in metabolic dilated cardiomyopathy
Lindsay M Ryerson1, R Michael Giuffre
1Department of Pediatrics, Faculty of Medicine, University of Calgary, Alberta.
The Canadian Journal of Cardiology
|March 8, 2006
Summary
Pediatric dilated cardiomyopathy patients with prolonged corrected QT (QTc) intervals face higher sudden death risks. Metabolic etiology combined with a long QTc interval significantly increases mortality in these children.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Genetic Metabolic Disorders
Background:
- Pediatric dilated cardiomyopathy presents significant morbidity and mortality challenges.
- A notable subset of patients, particularly within the Hutterite population, exhibit metabolic diseases linked to dilated cardiomyopathy.
Purpose of the Study:
- To determine the prevalence of prolonged corrected QT (QTc) intervals in children diagnosed with dilated cardiomyopathy.
- To investigate the association between prolonged QTc intervals and ventricular dysrhythmia in pediatric dilated cardiomyopathy patients.
Main Methods:
- Retrospective review of 38 pediatric patients with dilated cardiomyopathy.
- Assessment for prolonged QTc intervals and presence of dysrhythmias.
- Analysis of patient data, including etiological factors such as metabolic disease.
Main Results:
- 36% of the pediatric cohort presented with a prolonged QTc interval.
- Survival probability at 50 months was approximately 50% for children with long QTc versus 72% for those with normal QTc.
- 70% of deceased patients had both a metabolic etiology and a prolonged QTc interval.
Conclusions:
- Dilated cardiomyopathy in children is associated with prolonged QTc intervals, elevating the risk of sudden cardiac death.
- The presence of a metabolic etiology for dilated cardiomyopathy further compounds the risk of mortality.
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