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

Ran Baik, Jung Hyun Chae, Young Mock Lee, Hoon Chul Kang, Joon Soo Lee, Heung Dong Kim

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)644-647
Number of pages4
JournalKorean Journal of Pediatrics
Volume53
Issue number5
DOIs
Publication statusPublished - 2010 Jan 1

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Mitochondrial Diseases
Electrocardiography
Atrial Premature Complexes
Wolff-Parkinson-White Syndrome
Ventricular Premature Complexes
Bundle-Branch Block
Atrioventricular Block
Enzyme Assays
Electron Transport
Retrospective Studies

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Pediatrics

Cite this

Baik, Ran ; Chae, Jung Hyun ; Lee, Young Mock ; Kang, Hoon Chul ; Lee, Joon Soo ; Kim, Heung Dong. / Electrocardiography as an early cardiac screening test in children with mitochondrial disease. In: Korean Journal of Pediatrics. 2010 ; Vol. 53, No. 5. pp. 644-647.
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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.",
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Electrocardiography as an early cardiac screening test in children with mitochondrial disease. / Baik, Ran; Chae, Jung Hyun; Lee, Young Mock; Kang, Hoon Chul; Lee, Joon Soo; Kim, Heung Dong.

In: Korean Journal of Pediatrics, Vol. 53, No. 5, 01.01.2010, p. 644-647.

Research output: Contribution to journalArticle

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N2 - 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.

AB - 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.

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