Japanese Journal of Cardiovascular Surgery Vol45,No1

Bentall Procedure for Aortic Root Dilatation in a Patient with Turner Syndrome

Hirofumi Nakagawa Akihiro Nabuchi Masahiro Terada
Takuya Miyazaki Hiroshi Okuyama Masahiro Endo

(Department of Cardiovascular Surgery, Tokyo Heart Center*, Tokyo, Japan)

A 30-year-old woman who had no specific symptom was diagnosed with Turner syndrome at the age of 6 years. Subsequently, she was followed up at a hospital. However, she stopped going to the hospital when she was 18 years old. At 30 years of age, she underwent examinations involving echocardiography and enhanced chest CT at a hospital, which revealed severe aortic valve regurgitation and extreme dilatation of the aortic root. We performed the Bentall procedure through a median sternotomy following which she had an uncomplicated postoperative course. Aortic root enlargement increases the risk of aortic dissection in patients with Turner syndrome. However, no aortic events occurred before the surgery in this case. We considered the reason was related to the mosaic karyotype of this case.

Jpn. J. Cardiovasc. Surg. 45:21-25(2016)

Keywords:Turner syndrome;aortic root dilatation;Bentall procedure;acute aortic dissection;bicuspid aortic valve

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