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Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report

Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report


Titill: Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report
Höfundur: Ingason, Arnar   orcid.org/0000-0003-0943-2523
Sigfússon, Gunnlaugur
Torfason, Bjarni
Útgáfa: 2018-06-07
Tungumál: Enska
Háskóli/Stofnun: Háskóli Íslands
University of Iceland
Svið: Heilbrigðisvísindasvið (HÍ)
School of Health Sciences (UI)
Deild: Læknadeild (HÍ)
Faculty of Medicine (UI)
Birtist í: Journal of Cardiothoracic Surgery;13(1)
ISSN: 1749-8090
DOI: 10.1186/s13019-018-0755-0
Efnisorð: Unicuspid aortic valve; Congenital aortic stenosis; Aortic valve replacement; Case report; Blóðrásarsjúkdómar
URI: https://hdl.handle.net/20.500.11815/942

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Tilvitnun:

Ingason, A. B., Sigfusson, G., & Torfason, B. (2018). Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report. Journal of Cardiothoracic Surgery, 13(1), 61. doi:10.1186/s13019-018-0755-0

Útdráttur:

Background Unicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely present in infancy. We report a 17-year-old patient with congenital aortic stenosis secondary to unicuspid unicommissural aortic valve that was successfully treated with aortic valve replacement. Case presentation The patient was diagnosed with aortic stenosis after a murmur was heard in the newborn nursery and subsequently underwent aortic balloon valvuloplasty 6 weeks after birth. He had been regularly followed up since and underwent numerous cardiac catheterizations, including another aortic balloon valvuloplasty at age 13. During follow-up at age 17, the patient presented with symptomatic severe aortic stenosis and mild left ventricular hypertrophy. Aortic valve replacement was planned since the patient was nearly adult-sized and to reduce the risk of cardiac decompensation. During the operation an unicuspid unicommissural aortic valve was revealed. The patient recovered well post-operatively. He was discharged 5 days after the surgery in good condition and was completely symptom-free at follow-up 6 weeks later. Conclusions Unicuspid aortic valve is a rare congenital anomaly that can cause congenital aortic stenosis. It is seldom diagnosed pre-operatively but should be suspected in infants presenting with aortic stenosis.

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This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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