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Early cardiac evaluation in children with non-specific mitochondrial disease with isolated mitochondrial respiratory chain complex I defect.

Authors
 Ran Baik  ;  Rita Yu  ;  Young Mock Lee  ;  Hoon Chul Kang  ;  Joon Soo Lee  ;  Heung Dong Kim 
Citation
 JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Vol.48(11) : 1016-1020, 2012 
Journal Title
 JOURNAL OF PAEDIATRICS AND CHILD HEALTH 
ISSN
 1034-4810 
Issue Date
2012
MeSH
Arrhythmias, Cardiac/diagnostic imaging ; Arrhythmias, Cardiac/etiology ; Child ; Child, Preschool ; Early Diagnosis ; Echocardiography ; Electrocardiography ; Electron Transport Complex I*/metabolism ; Female ; Humans ; Male ; Mitochondrial Diseases/complications ; Mitochondrial Diseases/diagnostic imaging ; Mitochondrial Diseases/physiopathology* ; Myocardial Contraction/physiology ; Retrospective Studies ; Ventricular Dysfunction, Left/diagnostic imaging ; Ventricular Dysfunction, Left/etiology
Keywords
Arrhythmias, Cardiac/diagnostic imaging ; Arrhythmias, Cardiac/etiology ; Child ; Child, Preschool ; Early Diagnosis ; Echocardiography ; Electrocardiography ; Electron Transport Complex I*/metabolism ; Female ; Humans ; Male ; Mitochondrial Diseases/complications ; Mitochondrial Diseases/diagnostic imaging ; Mitochondrial Diseases/physiopathology* ; Myocardial Contraction/physiology ; Retrospective Studies ; Ventricular Dysfunction, Left/diagnostic imaging ; Ventricular Dysfunction, Left/etiology
Abstract
AIMS: We evaluated echocardiography and electrocardiography (ECG) results in children with non-specific mitochondrial disease (MD) in order to study early cardiac involvement, a well-known complication of the disease. METHODS: Among non-specific MD children whose isolated mitochondrial respiratory chain complex I defect was confirmed by muscle biopsy and satisfied the criteria of MD, 27 who had no cardiac symptoms were evaluated by echocardiography and ECG. RESULTS: Three (11.1%) out of the 27 non-specific MD patients had left ventricular ejection fraction of less than 55% and two of them (7.4%) had fractional shortening of less than 26%. ECG abnormalities were observed in 16 of the non-specific MD patients (59.3%). Prolongation of heart rate-corrected QT interval was seen in 11 (40.7%) and widening of the QRS interval in eight (29.6%). Left ventricular ejection fraction and fractional shortening of the patients were significantly decreased compared with those in the control group while heart rate-corrected QT interval was prolonged in the former group. QRS interval was more widened in non-specific MD patients, but without statistical significance. CONCLUSION: The potentially severe cardiac involvement observed in our subjects suggests that early cardiac evaluation after confirming the diagnosis of MD and regular follow-up tests should be strongly recommended in children even in cases without typical cardiac manifestations.
Full Text
http://onlinelibrary.wiley.com/doi/10.1111/j.1440-1754.2012.02585.x/abstract
DOI
23038991
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Hoon Chul(강훈철) ORCID logo https://orcid.org/0000-0002-3659-8847
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/91031
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