Title: | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
Author: |
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Date: | 2018-06-07 |
Language: | English |
University/Institute: | Háskóli Íslands University of Iceland |
School: | Heilbrigðisvísindasvið (HÍ) School of Health Sciences (UI) |
Department: | Læknadeild (HÍ) Faculty of Medicine (UI) |
Series: | Journal of Cardiothoracic Surgery;13(1) |
ISSN: | 1749-8090 |
DOI: | 10.1186/s13019-018-0755-0 |
Subject: | Unicuspid aortic valve; Congenital aortic stenosis; Aortic valve replacement; Case report; Blóðrásarsjúkdómar |
URI: | https://hdl.handle.net/20.500.11815/942 |
Citation: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
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Abstract: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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Description:Publisher's version (útgefin grein)
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