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Unicuspid aortic valve

MedGen UID:
576684
•Concept ID:
C0345001
•
Congenital Abnormality
Synonyms: unicuspid aortic valve; unicuspid aortic valves
SNOMED CT: Unicuspid aortic valve (253610004)
 
HPO: HP:0012561

Definition

The presence of an aortic valve with one instead of the normal three cusps (flaps). [from HPO]

Professional guidelines

PubMed

Treatment strategies for mixed aortic valve disease in nonelderly patients.
Von Stumm M, Petersen J, Westermann D, Reichenspurner H, Girdauskas E
Expert Rev Cardiovasc Ther 2019 Dec;17(12):873-882. Epub 2019 Dec 13 doi: 10.1080/14779072.2019.1702520. PMID: 31813303
Echocardiographic criteria to detect unicuspid aortic valve morphology.
Ewen S, Karliova I, Weber P, Schirmer SH, Abdul-Khaliq H, Schöpe J, Mahfoud F, Schäfers HJ
Eur Heart J Cardiovasc Imaging 2019 Jan 1;20(1):40-44. doi: 10.1093/ehjci/jex344. PMID: 29346529

Recent clinical studies

Therapy

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients.
Robinson DL, Minich LL, Menon SC, Ou Z, Eckhauser AW, Ware AL
Cardiol Young 2023 Dec;33(12):2610-2615. Epub 2023 Apr 20 doi: 10.1017/S104795112300077X. PMID: 37078183
Treatment strategies for mixed aortic valve disease in nonelderly patients.
Von Stumm M, Petersen J, Westermann D, Reichenspurner H, Girdauskas E
Expert Rev Cardiovasc Ther 2019 Dec;17(12):873-882. Epub 2019 Dec 13 doi: 10.1080/14779072.2019.1702520. PMID: 31813303
Unicuspid aortic valve concomitant with aortic insufficiency presenting with infectious endocarditis: a case report.
Yuzawa-Tsukada N, Tanaka TD, Morimoto S, Yoshimura M
J Med Case Rep 2019 Sep 20;13(1):297. doi: 10.1186/s13256-019-2239-9. PMID: 31537200Free PMC Article
Clinical and Echocardiographic Outcomes after Aortic Valve Repair in Patients with Bicuspid or Unicuspid Aortic Valve.
Ram E, Sternik L, Lipey A, Ben Zekry S, Ben-Avi R, Moshkovitz Y, Raanani E
Isr Med Assoc J 2018 Jul;20(7):423-428. PMID: 30109791
Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease.
Egbe AC, Connolly HM, Poterucha JT, Warnes CA
Congenit Heart Dis 2017 Jan;12(1):24-31. Epub 2016 Jul 4 doi: 10.1111/chd.12391. PMID: 27375186

Prognosis

Functionally unicuspid aortic valve in an adult: Case report and literature review.
Abraham A, Charles K, Pozo D, Bishev D, Pondicherry-Harish R
Curr Probl Cardiol 2024 Feb;49(2):102223. Epub 2023 Nov 30 doi: 10.1016/j.cpcardiol.2023.102223. PMID: 38042228
Considerations in the Surgical Management of Unicuspid Aortic Stenosis.
Gorton AJ, Anderson EP, Reimer JA, Abdelhady K, Sawaqed R, Massad MG
Pediatr Cardiol 2021 Jun;42(5):993-1001. Epub 2021 May 28 doi: 10.1007/s00246-021-02541-0. PMID: 34047809
Focus on a rare clinical entity: unicuspid aortic valve disease.
Naito S, Sequeira-Gross T, Petersen J, Holst T, Reichenspurner H, Girdauskas E
Expert Rev Cardiovasc Ther 2020 Sep;18(9):625-633. Epub 2020 Aug 31 doi: 10.1080/14779072.2020.1811685. PMID: 32811206
Bicuspidization and Annuloplasty Provide a Functioning Configuration to the Unicuspid Aortic Valve.
Igarashi T, Matsushima S, Shimizu A, Ehrlich T, Karliova I, Schäfers HJ
Ann Thorac Surg 2020 Jul;110(1):111-119. Epub 2019 Nov 28 doi: 10.1016/j.athoracsur.2019.10.023. PMID: 31786287
Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease.
Egbe AC, Connolly HM, Poterucha JT, Warnes CA
Congenit Heart Dis 2017 Jan;12(1):24-31. Epub 2016 Jul 4 doi: 10.1111/chd.12391. PMID: 27375186

Clinical prediction guides

Unicuspid Aortic Valve in Patients Undergoing the Ross Procedure.
Gofus J, Fila P, Vobornik M, Ondrasek J, Nemec P, Sterba J, Cermakova E, Tuna M, Vojacek J
Ann Thorac Surg 2023 Mar;115(3):626-631. Epub 2022 Apr 14 doi: 10.1016/j.athoracsur.2022.03.061. PMID: 35430219
Focus on a rare clinical entity: unicuspid aortic valve disease.
Naito S, Sequeira-Gross T, Petersen J, Holst T, Reichenspurner H, Girdauskas E
Expert Rev Cardiovasc Ther 2020 Sep;18(9):625-633. Epub 2020 Aug 31 doi: 10.1080/14779072.2020.1811685. PMID: 32811206
Clinical and Echocardiographic Outcomes After Aortic Valve Repair Surgery.
Ram E, Orlov B, Shinfeld A, Kogan A, Sternik L, Raanani E
Innovations (Phila) 2019 Jun;14(3):209-217. doi: 10.1177/1556984519845657. PMID: 31203738
Echocardiographic criteria to detect unicuspid aortic valve morphology.
Ewen S, Karliova I, Weber P, Schirmer SH, Abdul-Khaliq H, Schöpe J, Mahfoud F, Schäfers HJ
Eur Heart J Cardiovasc Imaging 2019 Jan 1;20(1):40-44. doi: 10.1093/ehjci/jex344. PMID: 29346529
Bicuspid and Unicuspid Aortic Valve: Fate of Moderate/Severe Mixed Aortic Valve Disease.
Egbe AC, Connolly HM, Poterucha JT, Warnes CA
Congenit Heart Dis 2017 Jan;12(1):24-31. Epub 2016 Jul 4 doi: 10.1111/chd.12391. PMID: 27375186

Recent systematic reviews

Unicuspid aortic valve in children: a systematic review spanning four decades.
Mookadam F, Thota VR, Lopez AM, Emani UR, Tajik AJ
J Heart Valve Dis 2010 Nov;19(6):678-83. PMID: 21214089
Unicuspid aortic valve in adults: a systematic review.
Mookadam F, Thota VR, Garcia-Lopez AM, Emani UR, Alharthi MS, Zamorano J, Khandheria BK
J Heart Valve Dis 2010 Jan;19(1):79-85. PMID: 20329493

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