title: Surgical repair for atrial septal defect associated with myotonic dystrophy.
abstract: 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.
authors.affiliation: Department of Cardiovascular Surgery, Kinki University School of Medicine, Osaka-Sayama, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Ogawa
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Saga
authors.forename: Susumu
authors.initials: S
authors.lastname: Nakamoto
mesh_terms: D020413:3' Untranslated Regions; D006344:Heart Septal Defects, Atrial / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D065636:Myotonin-Protein Kinase; D017346:Protein Serine-Threonine Kinases / Q000235:genetics*; D013318:Stroke Volume / Q000235:genetics; D019680:Trinucleotide Repeat Expansion
chemical_list: D020413:3' Untranslated Regions; C585517:DMPK protein, human; D065636:Myotonin-Protein Kinase; D017346:Protein Serine-Threonine Kinases