Surgical Repair for Atrial Septal Defect Associated With Myotonic Dystrophy

Myotonic dystrophy is a well-known hazard of anesthesia for various kinds of surgery. A 47-year-old male who had an increased CTG repeat of approximately 700 copies in the 3'-untranslated region of the myotonic dystrophy protein kinase gene underwent closure of an atrial septal defect under nor...

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Bibliographic Details
Published in:Circulation Journal 2007, Vol.71(8), pp.1321-1322
Main Authors: Ogawa, Tatsuya, Saga, Toshihiko, Nakamoto, Susumu
Format: Article
Language:English
Subjects:
3' Untranslated Regions
Atrial septal defect
CTG repeat
Heart Septal Defects, Atrial - surgery
Humans
Male
Middle Aged
Myocardial protection
Myotonic dystrophy
Myotonic Dystrophy - complications
Myotonic Dystrophy - genetics
Myotonic Dystrophy - physiopathology
Myotonin-Protein Kinase
Open heart surgery
Protein-Serine-Threonine Kinases - genetics
Stroke Volume - genetics
Trinucleotide Repeat Expansion
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Summary:Myotonic dystrophy is a well-known hazard of anesthesia for various kinds of surgery. A 47-year-old male who had an increased CTG repeat of approximately 700 copies in the 3'-untranslated region of the myotonic dystrophy protein kinase gene underwent closure of an atrial septal defect under normothermic beating heart. A strong correlation between reduced left ventricular ejection fraction and stroke volume, and the number of CTG repeats, has been reported. Because this correlation is not completely understood, even if the preoperative cardiac function is normal, it is important to check the number of CTG repeats and the patients who have a large number of them should be carefully treated. (Circ J 2007; 71: 1321 - 1322)
ISSN:1346-9843
1347-4820
DOI:10.1253/circj.71.1321