A surgical case of atrial septal defect with myotonic dystrophy

A case report is presented of a 16-year-old woman with myotonic dystrophy, who required open-heart surgery for an atrial septal defect. We successfully repaired an atrial septal defect using normothermic cardiopulmonary bypass and electrically maintained ventricular fibrillation. Anesthesia using fentanyl, vecronium and isoflurane was uneventful and there were no postoperative complications. Anesthetic agents must be selected with care, and ventilation requires meticulous attention.