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Single atrioventricular valve regurgitation

MedGen UID:
1815103
Concept ID:
C5706197
Pathologic Function
HPO: HP:0034377

Definition

Regurgitation of the single atrioventricular valve regurgitation (AVVR) in the context of a congenital heart defect with a single ventricle. Roughly three quarters of patients following Fontan procedure display AVVR. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSingle atrioventricular valve regurgitation

Professional guidelines

PubMed

Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
Kawasaki Y, Sasaki T, Forbes TJ, Ross RD, Kobayashi D
Heart Vessels 2021 Aug;36(8):1246-1255. Epub 2021 Feb 15 doi: 10.1007/s00380-021-01798-y. PMID: 33590306
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332

Recent clinical studies

Etiology

Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
King G, Buratto E, Celermajer DS, Grigg L, Alphonso N, Robertson T, Bullock A, Ayer J, Iyengar A, d'Udekem Y, Konstantinov IE
J Am Coll Cardiol 2022 May 10;79(18):1832-1845. Epub 2022 Mar 21 doi: 10.1016/j.jacc.2022.02.022. PMID: 35331598
Arrigoni SC, IJsselhof R, Postmus D, Vonk JM, François K, Bové T, Hazekamp MG, Rijnberg FM, Meyns B, van Puyvelde J, Poncelet AJ, de Beco G, van de Woestijne PC, Bogers AJJC, Schoof PH, Ebels T
J Thorac Cardiovasc Surg 2022 Mar;163(3):1166-1175. Epub 2021 May 18 doi: 10.1016/j.jtcvs.2021.05.015. PMID: 34099273
Tseng SY, Siddiqui S, Di Maria MV, Hill GD, Lubert AM, Kutty S, Opotowsky AR, Possner M, Morales DLS, Quintessenza JA, Alsaied T
J Am Heart Assoc 2020 Jun 2;9(11):e015737. Epub 2020 May 16 doi: 10.1161/JAHA.119.015737. PMID: 32419552Free PMC Article
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332

Diagnosis

Dahmen V, Heinisch PP, Staehler H, Schaeffer T, Burri M, Röhlig C, Klawonn F, Hager A, Ewert P, Hörer J, Ono M
Eur J Cardiothorac Surg 2023 Jun 1;63(6) doi: 10.1093/ejcts/ezad078. PMID: 36857588
Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
Inai K
Pediatr Int 2022 Jan;64(1):e14983. doi: 10.1111/ped.14983. PMID: 34480813
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332
Wallis GA, Debich-Spicer D, Anderson RH
Orphanet J Rare Dis 2011 May 14;6:22. doi: 10.1186/1750-1172-6-22. PMID: 21569592Free PMC Article

Therapy

King G, Buratto E, Celermajer DS, Grigg L, Alphonso N, Robertson T, Bullock A, Ayer J, Iyengar A, d'Udekem Y, Konstantinov IE
J Am Coll Cardiol 2022 May 10;79(18):1832-1845. Epub 2022 Mar 21 doi: 10.1016/j.jacc.2022.02.022. PMID: 35331598
Arrigoni SC, IJsselhof R, Postmus D, Vonk JM, François K, Bové T, Hazekamp MG, Rijnberg FM, Meyns B, van Puyvelde J, Poncelet AJ, de Beco G, van de Woestijne PC, Bogers AJJC, Schoof PH, Ebels T
J Thorac Cardiovasc Surg 2022 Mar;163(3):1166-1175. Epub 2021 May 18 doi: 10.1016/j.jtcvs.2021.05.015. PMID: 34099273
Tseng SY, Siddiqui S, Di Maria MV, Hill GD, Lubert AM, Kutty S, Opotowsky AR, Possner M, Morales DLS, Quintessenza JA, Alsaied T
J Am Heart Assoc 2020 Jun 2;9(11):e015737. Epub 2020 May 16 doi: 10.1161/JAHA.119.015737. PMID: 32419552Free PMC Article
Nakata T, Hoashi T, Shimada M, Ozawa H, Higashida A, Kurosaki K, Ichikawa H
Semin Thorac Cardiovasc Surg 2019 Autumn;31(3):526-534. Epub 2019 May 28 doi: 10.1053/j.semtcvs.2019.05.019. PMID: 31150828
King G, Gentles TL, Winlaw DS, Cordina R, Bullock A, Grigg LE, Alphonso N, Radford DJ, Zannino D, Buratto E, d'Udekem Y
Eur J Cardiothorac Surg 2017 Jun 1;51(6):1037-1043. doi: 10.1093/ejcts/ezx025. PMID: 28369323

Prognosis

Moye SC, Kidambi S, Lee JY, Cowles TH, Gilligan-Steinberg SD, Bryan AY, Wilkerson R, Woo YJ, Ma MR
Ann Biomed Eng 2023 Aug;51(8):1738-1746. Epub 2023 Mar 25 doi: 10.1007/s10439-023-03178-1. PMID: 36966247
Freud LR, Seed M
Can J Cardiol 2022 Jul;38(7):897-908. Epub 2022 Apr 13 doi: 10.1016/j.cjca.2022.04.003. PMID: 35429589
Arrigoni SC, IJsselhof R, Postmus D, Vonk JM, François K, Bové T, Hazekamp MG, Rijnberg FM, Meyns B, van Puyvelde J, Poncelet AJ, de Beco G, van de Woestijne PC, Bogers AJJC, Schoof PH, Ebels T
J Thorac Cardiovasc Surg 2022 Mar;163(3):1166-1175. Epub 2021 May 18 doi: 10.1016/j.jtcvs.2021.05.015. PMID: 34099273
Rodriguez FH, Book WM
Heart 2020 Jan;106(2):105-110. Epub 2019 Oct 29 doi: 10.1136/heartjnl-2019-314937. PMID: 31662332
Wallis GA, Debich-Spicer D, Anderson RH
Orphanet J Rare Dis 2011 May 14;6:22. doi: 10.1186/1750-1172-6-22. PMID: 21569592Free PMC Article

Clinical prediction guides

Moye SC, Kidambi S, Lee JY, Cowles TH, Gilligan-Steinberg SD, Bryan AY, Wilkerson R, Woo YJ, Ma MR
Ann Biomed Eng 2023 Aug;51(8):1738-1746. Epub 2023 Mar 25 doi: 10.1007/s10439-023-03178-1. PMID: 36966247
King G, Buratto E, Celermajer DS, Grigg L, Alphonso N, Robertson T, Bullock A, Ayer J, Iyengar A, d'Udekem Y, Konstantinov IE
J Am Coll Cardiol 2022 May 10;79(18):1832-1845. Epub 2022 Mar 21 doi: 10.1016/j.jacc.2022.02.022. PMID: 35331598
Inai K
Pediatr Int 2022 Jan;64(1):e14983. doi: 10.1111/ped.14983. PMID: 34480813
Ijsselhof R, Gauvreau K, Nido PD, Nathan M
World J Pediatr Congenit Heart Surg 2020 Mar;11(2):247-248. doi: 10.1177/2150135119893648. PMID: 32093554Free PMC Article
Miana LA, Manuel V, Turquetto AL, Issa HN, Guerreiro GP, Caneo LF, Jatene FB, Jatene MB
World J Pediatr Congenit Heart Surg 2020 Jan;11(1):22-28. doi: 10.1177/2150135119884916. PMID: 31835992

Recent systematic reviews

Ponzoni M, Azzolina D, Vedovelli L, Gregori D, Di Salvo G, D'Udekem Y, Vida V, Padalino MA
Eur J Cardiothorac Surg 2022 Nov 3;62(6) doi: 10.1093/ejcts/ezac535. PMID: 36367236
Loomba RS, Flores S, Villarreal EG, Bronicki RA, Anderson RH
World J Pediatr Congenit Heart Surg 2019 Sep;10(5):616-623. doi: 10.1177/2150135119859882. PMID: 31496417
Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR
J Heart Lung Transplant 2019 Jul;38(7):739-747. Epub 2019 Apr 4 doi: 10.1016/j.healun.2019.04.001. PMID: 31006521

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