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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.
Insights
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.
Objectives:
The present study determined the prevalence of dilated cardiomyopathy together with prolonged corrected QT (QTc) intervals in children. The study also examined whether an association exists between prolonged QTc intervals and ventricular dysrhythmia in a patient cohort with dilated cardiomyopathy.
Background:
The morbidity and mortality for pediatric patients with dilated cardiomyopathy remains high and is a clinical challenge. The patient population includes a significant number of Hutterite patients with metabolic disease associated with dilated cardiomyopathy.
Methods:
Thirty-eight pediatric patients with dilated cardiomyopathy were reviewed for the presence of prolonged QTc and dysrhythmias. Eleven patients had a metabolic etiology for their dilated cardiomyopathy.
Results:
Thirty-six per cent of the patient cohort had a long QTc interval. After 50 months of follow-up, the probability of survival for a child with a long QTc interval was approximately 50%. The probability of survival for a child with a normal QTc interval was 72%. Seventy per cent of the patients who died had a metabolic etiology for their dilated cardiomyopathy and a long QTc.
Conclusions:
Dilated cardiomyopathy may be associated with a prolonged QTc and may increase the patient's risk for sudden death. The presence of a metabolic etiology for dilated cardiomyopathy increases the risk of death.
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