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KMID : 1164620190520030162
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Korean Journal of Thoracic and Cardiovascular Surgery
2019 Volume.52 No. 3 p.162 ~ p.164
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Ventricular Septal Defect Closure in a Neonate with Osteogenesis Imperfecta
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Jang Woo-Sung
Choi Hee-Jeong
Kim Jae-Bum
Kim Jae-Hyun
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Abstract
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A male patient weighing 2.5 kg was admitted for respiratory difficulty, and a large ventricular septal defect (VSD) was diagnosed. During care, sudden right leg swelling with a femur shaft fracture occurred. The patient’s father had a history of recurrent lower extremity fractures; thus, osteogenesis imperfecta was considered. The patient’s respiratory difficulty became aggravated, and VSD repair in the neonatal period was therefore performed with gentle sternal traction and great vessel manipulation under total intravenous anes-thesia to prevent malignant hyperthermia. The patient was discharged without notable problems, except minor wound dehiscence. Outpatient genetic testing revealed that the patient had a COL1A1/COL1A2 mutation.
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KEYWORD
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Osteogenesis imperfecta, Malignant hypertherm, Cardiac surgery
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