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High incidence of progressive stenosis in aberrant left subclavian artery with right aortic arch

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Abstract

Right aortic arch with aberrant left subclavian artery (RAA/aLSCA) is a rare aortic arch anomaly. The clinical association of aLSCA stenosis with RAA/aLSCA has not yet been fully elucidated. The aim of this study was to investigate the diagnosis, incidence, management and outcome of aLSCA stenosis in infants with prenatally diagnosed RAA/aLSCA. Ten fetuses who were diagnosed as having RAA/aLSCA in Kyushu University Hospital between January 2011 and December 2014 were enrolled. The maternal and child medical records were reviewed to investigate sex, gestational age at the fetal diagnosis, gestational age and body weight at birth, the findings of computed tomography (CT), Doppler ultrasonography of the vertebral artery and angiography, and the complications and outcomes of aLSCA stenosis. In 8 of 10 patients, aLSCA stenosis was identified on the first CT examination after birth. No patients had dysphagia or respiratory distress. The stenosis spontaneously resolved in 3 patients. In 4 of the 5 remaining patients, aLSCA stenosis progressed, including one case in which complete occlusion occurred—the case was associated with retrograde flow from the left vertebral artery supplying the distal LSCA. Balloon angioplasty was successfully used to treat stenosis in two cases. The subclavian steal phenomenon and developmental problems were not observed in any patients. aLSCA stenosis was identified in 80% of patients with RAA/aLSCA after birth. The early detection and elective treatment of stenotic lesions may be required to prevent complete occlusion during the development of the cardiovascular and cerebrovascular systems.

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Acknowledgements

We thank Brian Quinn for his linguistic assistance with this paper. This work was supported in part by a grant-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science, and Technology of Japan (15K19622).

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Authors and Affiliations

  1. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Mamoru Muraoka, Hazumu Nagata, Yuichiro Hirata, Kiyoshi Uike, Eiko Terashi, Eiji Morihana, Masayuki Ochiai, Kenichiro Yamamura & Shouichi Ohga

  2. Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

    Yasuyuki Fujita & Kiyoko Kato

Authors
  1. Mamoru Muraoka
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  2. Hazumu Nagata
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  3. Yuichiro Hirata
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  4. Kiyoshi Uike
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  5. Eiko Terashi
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  6. Eiji Morihana
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  7. Masayuki Ochiai
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  8. Yasuyuki Fujita
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  9. Kiyoko Kato
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  10. Kenichiro Yamamura
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  11. Shouichi Ohga
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Contributions

Dr. Muraoka carried out the primary analyses to draft the manuscript. Dr. Nagata and Dr. Yamamura conceptualized and designed the study, and reviewed the manuscript. Dr. Muraoka and Dr. Nagata contributed equally to this work. Dr. Hirata, Dr. Uike, Dr. Terashi and Dr. Morihana assisted in implementing the examinations and interventions. Dr. Fujita and Prof. Kato implemented and supported the fetal diagnosis. Prof. Ohga helped to complete the project, and reviewed and revised manuscript; and all authors approved the final manuscript for submission.

Corresponding author

Correspondence to Hazumu Nagata.

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Conflict of interest

The authors declare no conflicts of interest in association with the present study.

Funding source

This work was supported in part by a grant-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science, and Technology of Japan (15K19622).

Financial disclosure

The authors have no financial relationships relevant to this article to disclose.

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Muraoka, M., Nagata, H., Hirata, Y. et al. High incidence of progressive stenosis in aberrant left subclavian artery with right aortic arch. Heart Vessels 33, 309–315 (2018). https://doi.org/10.1007/s00380-017-1056-6

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  • DOI: https://doi.org/10.1007/s00380-017-1056-6

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