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Intermediate atrioventricular canal defect

MedGen UID:
868880
•Concept ID:
C4023291
•
Congenital Abnormality
Synonym: Intermediate atrioventricular septal defect
 
HPO: HP:0011576

Definition

A specific combination of heart defects with a primum atrial septal defect, cleft anterior mitral valve leaflet, and inlet ventricular defect. There is one valve annulus and two valve orifices. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntermediate atrioventricular canal defect

Recent clinical studies

Etiology

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect.
Jacquemart E, Bessière F, Combes N, Ladouceur M, Iserin L, Gardey K, Henaine R, Dulac A, Cohen S, Belli E, Jannot AS, Chevalier P, Ly R, Clavier S, Legendre A, Petit J, Maltret A, Di Filippo S, Hascoët S, Marijon E, Waldmann V
JACC Clin Electrophysiol 2022 Mar;8(3):331-340. Epub 2021 Oct 27 doi: 10.1016/j.jacep.2021.09.004. PMID: 35331427
Early and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.
Cui H, Nie Z, Ou Y, Zhou K, Chen J, Cen J, Xu G, Wen S, Liu X, Zhuang J
J Card Surg 2020 Jan;35(1):75-82. Epub 2019 Nov 6 doi: 10.1111/jocs.14330. PMID: 31692110
Long-term results after surgical repair of atrioventricular septal defect.
Schleiger A, Miera O, Peters B, Schmitt KRL, Kramer P, Buracionok J, Murin P, Cho MY, Photiadis J, Berger F, Ovroutski S
Interact Cardiovasc Thorac Surg 2019 May 1;28(5):789-796. doi: 10.1093/icvts/ivy334. PMID: 30590597
Tetralogy of Fallot with atrioventricular septal defect: surgical strategies for repair and midterm outcome of pulmonary valve-sparing approach.
Gupta U, Polimenakos AC, El-Zein C, Ilbawi MN
Pediatr Cardiol 2013 Apr;34(4):861-71. Epub 2012 Oct 27 doi: 10.1007/s00246-012-0558-3. PMID: 23104595
Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C
Ann Thorac Surg 2000 Apr;69(4 Suppl):S36-43. doi: 10.1016/s0003-4975(99)01235-7. PMID: 10798414

Diagnosis

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.
Yang S, Liang M, Qin G, Tang Y, Liang Y, Pang Y, Yang Z
J Clin Ultrasound 2024 May;52(4):405-414. Epub 2024 Feb 16 doi: 10.1002/jcu.23651. PMID: 38362713
Three-dimensional transesophageal echocardiography in diagnosis of intermediate atrioventricular septal defect in the adult: case report and literature review.
Schusterova I, Jakubova M, Vachalcova M, Sieradzka K, Gibarty C, Poruban T, Gasparovic I, Artemiou P
J Cardiothorac Surg 2021 Jul 30;16(1):209. doi: 10.1186/s13019-021-01596-7. PMID: 34330308Free PMC Article
Early and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.
Cui H, Nie Z, Ou Y, Zhou K, Chen J, Cen J, Xu G, Wen S, Liu X, Zhuang J
J Card Surg 2020 Jan;35(1):75-82. Epub 2019 Nov 6 doi: 10.1111/jocs.14330. PMID: 31692110
Long-term results after surgical repair of atrioventricular septal defect.
Schleiger A, Miera O, Peters B, Schmitt KRL, Kramer P, Buracionok J, Murin P, Cho MY, Photiadis J, Berger F, Ovroutski S
Interact Cardiovasc Thorac Surg 2019 May 1;28(5):789-796. doi: 10.1093/icvts/ivy334. PMID: 30590597
Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.
Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C
Ann Thorac Surg 2000 Apr;69(4 Suppl):S36-43. doi: 10.1016/s0003-4975(99)01235-7. PMID: 10798414

Therapy

Modified single patch: are we still worried about subaortic stenosis?
Backer CL, Eltayeb O, Mongé MC, Wurlitzer KC, Hack MA, Boles LH, Sarwark AE, Costello JM, Robinson JD
Ann Thorac Surg 2015 May;99(5):1671-5; discussion 1675-6. Epub 2015 Mar 29 doi: 10.1016/j.athoracsur.2015.01.032. PMID: 25825201
Tracheostomy after pediatric cardiac surgery: frequency, indications, and outcomes.
Cotts T, Hirsch J, Thorne M, Gajarski R
J Thorac Cardiovasc Surg 2011 Feb;141(2):413-8. Epub 2010 Jul 16 doi: 10.1016/j.jtcvs.2010.06.027. PMID: 20638080
Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect.
Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K
Ann Thorac Surg 2004 Jun;77(6):2157-62. doi: 10.1016/j.athoracsur.2003.12.019. PMID: 15172287

Prognosis

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.
Yang S, Liang M, Qin G, Tang Y, Liang Y, Pang Y, Yang Z
J Clin Ultrasound 2024 May;52(4):405-414. Epub 2024 Feb 16 doi: 10.1002/jcu.23651. PMID: 38362713
Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect.
Jacquemart E, Bessière F, Combes N, Ladouceur M, Iserin L, Gardey K, Henaine R, Dulac A, Cohen S, Belli E, Jannot AS, Chevalier P, Ly R, Clavier S, Legendre A, Petit J, Maltret A, Di Filippo S, Hascoët S, Marijon E, Waldmann V
JACC Clin Electrophysiol 2022 Mar;8(3):331-340. Epub 2021 Oct 27 doi: 10.1016/j.jacep.2021.09.004. PMID: 35331427
Long-term results after surgical repair of atrioventricular septal defect.
Schleiger A, Miera O, Peters B, Schmitt KRL, Kramer P, Buracionok J, Murin P, Cho MY, Photiadis J, Berger F, Ovroutski S
Interact Cardiovasc Thorac Surg 2019 May 1;28(5):789-796. doi: 10.1093/icvts/ivy334. PMID: 30590597
Tetralogy of Fallot with atrioventricular septal defect: surgical strategies for repair and midterm outcome of pulmonary valve-sparing approach.
Gupta U, Polimenakos AC, El-Zein C, Ilbawi MN
Pediatr Cardiol 2013 Apr;34(4):861-71. Epub 2012 Oct 27 doi: 10.1007/s00246-012-0558-3. PMID: 23104595
Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect.
Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K
Ann Thorac Surg 2004 Jun;77(6):2157-62. doi: 10.1016/j.athoracsur.2003.12.019. PMID: 15172287

Clinical prediction guides

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.
Yang S, Liang M, Qin G, Tang Y, Liang Y, Pang Y, Yang Z
J Clin Ultrasound 2024 May;52(4):405-414. Epub 2024 Feb 16 doi: 10.1002/jcu.23651. PMID: 38362713
Long-term results after surgical repair of atrioventricular septal defect.
Schleiger A, Miera O, Peters B, Schmitt KRL, Kramer P, Buracionok J, Murin P, Cho MY, Photiadis J, Berger F, Ovroutski S
Interact Cardiovasc Thorac Surg 2019 May 1;28(5):789-796. doi: 10.1093/icvts/ivy334. PMID: 30590597
A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect.
Sojak V, Kooij M, Yazdanbakhsh A, Koolbergen DR, Bruggemans EF, Hazekamp MG
Eur J Cardiothorac Surg 2016 Feb;49(2):538-44; discussion 544-5. Epub 2015 Apr 7 doi: 10.1093/ejcts/ezv115. PMID: 25855593
Preoperative NT-proBNP values in patients with atrioventricular septal defect and its role as a predictor of early postoperative course.
Bobik L, Kovacikova L, Zahorec M, Danova K
Bratisl Lek Listy 2015;116(11):648-53. doi: 10.4149/bll_2015_124. PMID: 26621160
Modified single patch: are we still worried about subaortic stenosis?
Backer CL, Eltayeb O, Mongé MC, Wurlitzer KC, Hack MA, Boles LH, Sarwark AE, Costello JM, Robinson JD
Ann Thorac Surg 2015 May;99(5):1671-5; discussion 1675-6. Epub 2015 Mar 29 doi: 10.1016/j.athoracsur.2015.01.032. PMID: 25825201

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