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Partial atrioventricular canal

MedGen UID:
868879
Concept ID:
C4023290
Congenital Abnormality
Synonyms: Partial atrioventricular canal defect; Partial atrioventricular septal defects; Partial AVSD; Partial common atrioventricular canal
SNOMED CT: Partial defect of atrioventricular canal (718216009); Partial atrioventricular canal (718216009)
Modes of inheritance:
 
HPO: HP:0011577
Monarch Initiative: MONDO:0015275
Orphanet: ORPHA1330

Definition

A specific combination of heart defects including a primum atrial septal defect and cleft anterior mitral valve leaflet. There is not an inlet ventricular septal defect present. There are two valve annuluses and two valve orifices. [from HPO]

Conditions with this feature

Professional guidelines

PubMed

Gembruch U, Knöpfle G, Chatterjee M, Bald R, Redel DA, Födisch HJ, Hansmann M
Am Heart J 1991 May;121(5):1489-97. doi: 10.1016/0002-8703(91)90156-c. PMID: 2017980
Hardesty RL, Zuberbuhler JR, Bahnson HT
Arch Surg 1975 Nov;110(11):1391-6. doi: 10.1001/archsurg.1975.01360170131020. PMID: 1191034

Recent clinical studies

Etiology

Putotto C, Masci M, Magliozzi M, Novelli A, Marino B, Digilio MC, Toscano A
Birth Defects Res 2024 Jul;116(7):e2382. doi: 10.1002/bdr2.2382. PMID: 38975735
Gellis L, McGeoghegan P, Lu M, Feins E, Sleeper L, Emani S, Friedman K, Baird C
J Thorac Cardiovasc Surg 2023 Oct;166(4):1168-1177. Epub 2023 May 7 doi: 10.1016/j.jtcvs.2023.04.004. PMID: 37160215
Mery CM, Zea-Vera R, Chacon-Portillo MA, Zhang W, Binder MS, Kyle WB, Adachi I, Heinle JS, Fraser CD Jr
J Thorac Cardiovasc Surg 2019 Mar;157(3):1117-1127.e4. Epub 2018 Nov 24 doi: 10.1016/j.jtcvs.2018.10.154. PMID: 31079807
Devlin PJ, Backer CL, Eltayeb O, Mongé MC, Hauck AL, Costello JM
Ann Thorac Surg 2016 Jul;102(1):170-7. Epub 2016 Apr 25 doi: 10.1016/j.athoracsur.2016.01.085. PMID: 27112649
Manning PB
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007:42-6. doi: 10.1053/j.pcsu.2007.02.002. PMID: 17433990

Diagnosis

Katewa A, Sahu B, Prabhu A, Haranal M
Asian Cardiovasc Thorac Ann 2023 Feb;31(2):97-101. Epub 2022 Nov 27 doi: 10.1177/02184923221142378. PMID: 36437568
Song L, Ling Y, An Q
J Cardiothorac Surg 2019 Jun 11;14(1):106. doi: 10.1186/s13019-019-0931-x. PMID: 31186038Free PMC Article
Tishler B, Gauvreau K, Colan SD, Del Nido P, Nathan M
Ann Thorac Surg 2018 May;105(5):1461-1468. Epub 2017 Dec 19 doi: 10.1016/j.athoracsur.2017.11.043. PMID: 29269131
Devlin PJ, Backer CL, Eltayeb O, Mongé MC, Hauck AL, Costello JM
Ann Thorac Surg 2016 Jul;102(1):170-7. Epub 2016 Apr 25 doi: 10.1016/j.athoracsur.2016.01.085. PMID: 27112649
Digilio MC, Romana Lepri F, Dentici ML, Henderson A, Baban A, Roberti MC, Capolino R, Versacci P, Surace C, Angioni A, Tartaglia M, Marino B, Dallapiccola B
Eur J Hum Genet 2013 Feb;21(2):200-4. Epub 2012 Jul 11 doi: 10.1038/ejhg.2012.145. PMID: 22781091Free PMC Article

Therapy

Gellis L, McGeoghegan P, Lu M, Feins E, Sleeper L, Emani S, Friedman K, Baird C
J Thorac Cardiovasc Surg 2023 Oct;166(4):1168-1177. Epub 2023 May 7 doi: 10.1016/j.jtcvs.2023.04.004. PMID: 37160215
Song L, Ling Y, An Q
J Cardiothorac Surg 2019 Jun 11;14(1):106. doi: 10.1186/s13019-019-0931-x. PMID: 31186038Free PMC Article
Fernando RJ, Johnson SD, Carter T
Anesthesiology 2019 Oct;131(4):896-897. doi: 10.1097/ALN.0000000000002795. PMID: 31116104
Mery CM, Zea-Vera R, Chacon-Portillo MA, Zhang W, Binder MS, Kyle WB, Adachi I, Heinle JS, Fraser CD Jr
J Thorac Cardiovasc Surg 2019 Mar;157(3):1117-1127.e4. Epub 2018 Nov 24 doi: 10.1016/j.jtcvs.2018.10.154. PMID: 31079807
Aeba R, Kudo M, Okamoto K, Yozu R
Ann Thorac Surg 2012 May;93(5):e137-9. doi: 10.1016/j.athoracsur.2011.12.071. PMID: 22541239

Prognosis

Gellis L, McGeoghegan P, Lu M, Feins E, Sleeper L, Emani S, Friedman K, Baird C
J Thorac Cardiovasc Surg 2023 Oct;166(4):1168-1177. Epub 2023 May 7 doi: 10.1016/j.jtcvs.2023.04.004. PMID: 37160215
Song L, Ling Y, An Q
J Cardiothorac Surg 2019 Jun 11;14(1):106. doi: 10.1186/s13019-019-0931-x. PMID: 31186038Free PMC Article
Mery CM, Zea-Vera R, Chacon-Portillo MA, Zhang W, Binder MS, Kyle WB, Adachi I, Heinle JS, Fraser CD Jr
J Thorac Cardiovasc Surg 2019 Mar;157(3):1117-1127.e4. Epub 2018 Nov 24 doi: 10.1016/j.jtcvs.2018.10.154. PMID: 31079807
Devlin PJ, Backer CL, Eltayeb O, Mongé MC, Hauck AL, Costello JM
Ann Thorac Surg 2016 Jul;102(1):170-7. Epub 2016 Apr 25 doi: 10.1016/j.athoracsur.2016.01.085. PMID: 27112649
El-Rassi I, Charafedine F, Majdalani M, Arabi M, Khater D, Bitar F
Multimed Man Cardiothorac Surg 2015;2015 Epub 2015 Dec 17 doi: 10.1093/mmcts/mmv037. PMID: 26685152

Clinical prediction guides

Lee WJ, Moon CH, Yoon W, Kim M, Kim WJ, Kim KM, Lee H, Jeong SM, Yu JH, Kim DH
J Vet Sci 2023 May;24(3):e47. doi: 10.4142/jvs.23088. PMID: 37271514Free PMC Article
Gellis L, McGeoghegan P, Lu M, Feins E, Sleeper L, Emani S, Friedman K, Baird C
J Thorac Cardiovasc Surg 2023 Oct;166(4):1168-1177. Epub 2023 May 7 doi: 10.1016/j.jtcvs.2023.04.004. PMID: 37160215
Tishler B, Gauvreau K, Colan SD, Del Nido P, Nathan M
Ann Thorac Surg 2018 May;105(5):1461-1468. Epub 2017 Dec 19 doi: 10.1016/j.athoracsur.2017.11.043. PMID: 29269131
Zhou T, Li J, Lai H, Zhu K, Sun Y, Wang Y, Ding W, Hong T, Wang C
Interact Cardiovasc Thorac Surg 2018 Mar 1;26(3):468-473. doi: 10.1093/icvts/ivx349. PMID: 29069357
Digilio MC, Romana Lepri F, Dentici ML, Henderson A, Baban A, Roberti MC, Capolino R, Versacci P, Surace C, Angioni A, Tartaglia M, Marino B, Dallapiccola B
Eur J Hum Genet 2013 Feb;21(2):200-4. Epub 2012 Jul 11 doi: 10.1038/ejhg.2012.145. PMID: 22781091Free PMC Article

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