Clinical Course of Patients with Prenatally Diagnosed Vascular Rings with Right Aortic Arch with Aberrant Left Subclavian Artery and Left Ductus Arteriosus

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  • Shiono Nobuko
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan Department of Obstetrics and Gynecology Hokkaido University Graduate School of Medicine, Hokkaido, Japan.
  • Inamura Noboru
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Torigoe Fumiko
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Tanaka Tomohiko
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Mihara Seiko
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Narita Jun
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Kawazu Yukiko
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Hamamichi Yuji
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan
  • Kayatani Futoshi
    Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

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Other Title
  • 胎児超音波検査にて右側大動脈弓，左鎖骨下動脈起始異常， 左動脈管に伴う血管輪と診断された症例の出生後経過

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Objectives: We herein report a case of a series of prenatally diagnosed vascular rings with right aortic arch with aberrant left subclavian artery （RAA with ALSA） and left ductus arteriosus, describing the clinical outcomes after birth. The aim of this study was to uncover the most appropriate prenatal diagnostic and postnatal follow-up methods for vascular rings with RAA with ALSA.<br> Methods: Among 1,695 pregnant females examined from January 2001 to December 2011, eleven patients had a prenatal diagnosis of vascular rings with RAA with ALSA. The diagnosis was confirmed by postnatal echocardiography or computed tomography and/or based on the surgical findings. This was a retrospective analysis of these 11 cases.<br> Results: Of the 11 cases with a prenatal diagnosis of RAA with ALSA, five were born in our hospital. Two of the 11 postnatal developed respiratory symptoms requiring surgery at one month and at one year and five months after birth, respectively. The former case had a diagnosis of double aortic arch （DAA） based on the surgical findings after birth. The remaining nine cases were asymptomatic. Only one case with RAA with ALSA was found to have VSD and a chromosome 22q11.2 deletion after birth. The others had no structural anomalies or major cardiac defects.<br> Conclusions: In addition to using the three-vessel trachea view, it is important to identify the cervical vessels arising from the aortic arch during the differentiation of RAA with ALSA and DAA on fetal echocardiography, paying special attention to detect cases with a DAA with a very diminutive aortic arch. The delivery should be planned in perinatal institutions when it is difficult to differentiate DAA from RAA with ALSA. Because some cases with vascular rings with RAA with ALSA develop symptoms long after birth, meticulous follow-up and appropriate explanation to the family are recommended.

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