Advanced Search
Korean J Pediatr 2010 May;53(5) :644-647.
Electrocardiography as an early cardiac screening test in children with mitochondrial disease
Ran Baik (Baik R), Jung Hyun Chae (Chae JH), Young Mock Lee (Lee YM), Hoon Chul Kang (Kang HC), Joon Soo Lee (Lee JS), Heung Dong Kim (Kim HD)
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
Corresponding Author: Young Mock Lee ,Email:
Copyright © 2010 by The Korean Pediatric Society
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.
Keywords: Electrocardiography | Mitochondria | Respiratory chain | Heart | Conductivity | Child
PDF Links  PDF Links
Full text via DOI  Full text via DOI
  via Pubmed
Full text via PMC  Full text via PMC
via Pubreader  via PubReader
Download Citation  Download Citation
Supplementary Material  Supplementary Material
The use of complementary and alternative medicine in children with common neurologic diseases  2016 July;59(8)
Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy  2014 May;57(5)
Early postictal electroencephalography and correlation with clinical findings in children with febrile seizures  2013 December;56(12)
Risk factors of ocular involvement in children with mitochondrial respiratory chain complex defect  2010 December;53(12)
Register for e-submission
Register here to access the e-submission system of Korean J Pediatr for authors and reviewers.
Manuscript Submission
To submit a manuscript, please visit the Korean J Pediatr e-submission management system at, read the Instructions for Authors, and log into the Korean J Pediatr e-submission system. For assistance with manuscript submission, please contact:
Free archive
Anyone may access any past or current articles without logging in.
Korean Pediatric Society Office
#1606, Seocho World Officetel, 19 Seoun-ro, Seocho-gu, Seoul 137-070, Korea
TEL : +82-2-3473-7305    FAX : +82-2-3473-7307   E-mail:
BrowseCurrent IssueFor Authors and ReviewersAbout
Copyright© The Korean Pediatric Society. All right reserved.