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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

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
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

Etiology

Schulz A, Taylor L, Buratto E, Ivanov Y, Zhu M, Brizard CP, Konstantinov IE
Semin Thorac Cardiovasc Surg 2023 Winter;35(4):713-721. Epub 2022 Aug 4 doi: 10.1053/j.semtcvs.2022.07.013. PMID: 35932981
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
Malagoli A, Albini A, Baldini L, Grassi L, Tondi S
Echocardiography 2021 Mar;38(3):504-505. Epub 2021 Feb 21 doi: 10.1111/echo.15003. PMID: 33611817
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
Mookadam F, Thota VR, Lopez AM, Emani UR, Tajik AJ
J Heart Valve Dis 2010 Nov;19(6):678-83. PMID: 21214089

Diagnosis

Pan J
Cardiology 2022;147(2):207-215. Epub 2021 Dec 29 doi: 10.1159/000521623. PMID: 34965530
Vilardell P, Moral S, Morales M, Ballesteros E, Brugada R
Acta Cardiol 2022 Jul;77(5):463-464. Epub 2021 Jun 3 doi: 10.1080/00015385.2021.1897259. PMID: 34080949
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
Si MS, Conte JV, Romano JC, Romano MA, Andersen ND, Gerdisch MW, Kupferschmid JP, Fiore AC, Bakhos M, Bonilla JJ, Burke JR, Rankin JS, Wei LM, Badhwar V, Turek JW
Ann Thorac Surg 2021 Apr;111(4):1359-1366. Epub 2020 Jun 30 doi: 10.1016/j.athoracsur.2020.04.147. PMID: 32619617
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

Therapy

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
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
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
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
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

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
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
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
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
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

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
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
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
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
Kolesar A, Toporcer T, Bajmoczi M, Luczy J, Candik P, Sabol F
Ann Thorac Surg 2018 May;105(5):1351-1356. Epub 2018 Jan 31 doi: 10.1016/j.athoracsur.2017.12.031. PMID: 29391147

Recent systematic reviews

Mookadam F, Thota VR, Lopez AM, Emani UR, Tajik AJ
J Heart Valve Dis 2010 Nov;19(6):678-83. PMID: 21214089
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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