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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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Published in: | Circulation Journal 2007, Vol.71(8), pp.1321-1322 |
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description | 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) |
doi_str_mv | 10.1253/circj.71.1321 |
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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. 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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 |
title | Surgical Repair for Atrial Septal Defect Associated With Myotonic Dystrophy |
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