Korean J Pediatr.  2010 May;53(5):644-647. 10.3345/kjp.2010.53.5.644.

Electrocardiography as an early cardiac screening test in children with mitochondrial disease

• Baik R
• Chae JH
• Lee YM
• Kang HC
• Lee JS
• Kim HD

• KMID: 2042068
• DOI: http://doi.org/10.3345/kjp.2010.53.5.644

Abstract

PURPOSE
To evaluate myocardial conductivity to understand cardiac involvement in patients with mitochondrial disease.
METHODS
We performed retrospective study on fifty-seven nonspecific mitochondrial encephalopathy patients with no clinical cardiac manifestations. The patients were diagnosed with mitochondrial respiratory chain complex defects through biochemical enzyme assays of muscle tissue. We performed standard 12-lead electrocardiography (ECG) on all patients.
RESULTS
ECG abnormalities were observed in 30 patients (52.6%). Prolongation of the QTc interval (>440 ms) was seen in 19 patients (33.3%), widening of the corrected QRS interval in 15 (26.3%), and bundle branch block in four (7.0%). Atrioventricular block, premature atrial contraction and premature ventricular contraction were seen in two patients each (3.5%) and Wolff-Parkinson-White syndrome in one patient (1.8%).
CONCLUSION
Given this finding, we recommend active screening with ECG in patients with mitochondrial disease even in patients without obvious cardiac manifestation.

Keyword

Electrocardiography; Mitochondria; Respiratory chain; Heart; Conductivity; Child

MeSH Terms

Atrial Premature Complexes
Atrioventricular Block
Bundle-Branch Block
Child
Electrocardiography
Electron Transport
Enzyme Assays
Heart
Humans
Mass Screening
Mitochondria
Mitochondrial Diseases
Mitochondrial Encephalomyopathies
Muscles
Retrospective Studies
Ventricular Premature Complexes
Wolff-Parkinson-White Syndrome
Mitochondrial Encephalomyopathies