Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report
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 successf...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2018-06-01
|
| Series: | Journal of Cardiothoracic Surgery |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13019-018-0755-0 |
| _version_ | 1811238297227231232 |
|---|---|
| author | Arnar B. Ingason Gunnlaugur Sigfusson Bjarni Torfason |
| author_facet | Arnar B. Ingason Gunnlaugur Sigfusson Bjarni Torfason |
| author_sort | Arnar B. Ingason |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T12:39:18Z |
| format | Article |
| id | doaj.art-50e0a8ba674141c98e3955f4f138c73d |
| institution | Directory Open Access Journal |
| issn | 1749-8090 |
| language | English |
| last_indexed | 2024-04-12T12:39:18Z |
| publishDate | 2018-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Cardiothoracic Surgery |
| spelling | doaj.art-50e0a8ba674141c98e3955f4f138c73d2022-12-22T03:32:49ZengBMCJournal of Cardiothoracic Surgery1749-80902018-06-011311410.1186/s13019-018-0755-0Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case reportArnar B. Ingason0Gunnlaugur Sigfusson1Bjarni Torfason2Department of Medicine, University of IcelandChildren’s Hospital, Landspitali University HospitalDepartment of Medicine, University of IcelandAbstract 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.http://link.springer.com/article/10.1186/s13019-018-0755-0Unicuspid aortic valveCongenital aortic stenosisAortic valve replacementCase report |
| spellingShingle | Arnar B. Ingason Gunnlaugur Sigfusson Bjarni Torfason Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report Journal of Cardiothoracic Surgery Unicuspid aortic valve Congenital aortic stenosis Aortic valve replacement Case report |
| title | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
| title_full | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
| title_fullStr | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
| title_full_unstemmed | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
| title_short | Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report |
| title_sort | congenital aortic stenosis due to unicuspid unicommissural aortic valve a case report |
| topic | Unicuspid aortic valve Congenital aortic stenosis Aortic valve replacement Case report |
| url | http://link.springer.com/article/10.1186/s13019-018-0755-0 |
| work_keys_str_mv | AT arnarbingason congenitalaorticstenosisduetounicuspidunicommissuralaorticvalveacasereport AT gunnlaugursigfusson congenitalaorticstenosisduetounicuspidunicommissuralaorticvalveacasereport AT bjarnitorfason congenitalaorticstenosisduetounicuspidunicommissuralaorticvalveacasereport |