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Tetralogy of Fallot with atrioventricular canal defect

MedGen UID:
868825
•Concept ID:
C4023235
•
Congenital Abnormality
HPO: HP:0011677

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTetralogy of Fallot with atrioventricular canal defect

Professional guidelines

PubMed

Detection rates of a national fetal anomaly screening programme: A national cohort study.
Aldridge N, Pandya P, Rankin J, Miller N, Broughan J, Permalloo N, McHugh A, Stevens S
BJOG 2023 Jan;130(1):51-58. Epub 2022 Sep 12 doi: 10.1111/1471-0528.17287. PMID: 36054171
Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.
Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O
Anatol J Cardiol 2022 Sep;26(9):702-709. doi: 10.5152/AnatolJCardiol.2022.1461. PMID: 35943311Free PMC Article
Current options and outcomes for the management of atrioventricular septal defect.
Shuhaiber JH, Ho SY, Rigby M, Sethia B
Eur J Cardiothorac Surg 2009 May;35(5):891-900. Epub 2009 Feb 24 doi: 10.1016/j.ejcts.2009.01.009. PMID: 19243971

Recent clinical studies

Etiology

Routine Perioperative Esmolol After Infant Tetralogy of Fallot Repair: Single-Center Retrospective Study of Hemodynamics.
Affolter JT, Well A, Gottlieb EA, Fraser CD Jr
Pediatr Crit Care Med 2022 Dec 1;23(12):e583-e589. Epub 2022 Oct 5 doi: 10.1097/PCC.0000000000003088. PMID: 36200768
Predictors of reoperation and mortality after complete atrioventricular septal defect repair.
Fong LS, Betts K, Ayer J, Andrews D, Nicholson IA, Winlaw DS, Orr Y; Australian CAVSD study group
Eur J Cardiothorac Surg 2021 Dec 27;61(1):45-53. doi: 10.1093/ejcts/ezab221. PMID: 34002204
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Cor triatriatum sinister.
Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580
Perioperative Management of DORV.
Spaeth JP
Semin Cardiothorac Vasc Anesth 2014 Sep;18(3):281-9. Epub 2014 Mar 21 doi: 10.1177/1089253214528048. PMID: 24659409

Diagnosis

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.
Stephens EH, Tingo J, Najjar M, Yilmaz B, Levasseur S, Dayton JD, Mosca RS, Chai P, Quaegebeur JM, Bacha EA
World J Pediatr Congenit Heart Surg 2017 Mar;8(2):189-195. doi: 10.1177/2150135116682719. PMID: 28329461
Cor triatriatum sinister.
Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580
Perioperative Management of DORV.
Spaeth JP
Semin Cardiothorac Vasc Anesth 2014 Sep;18(3):281-9. Epub 2014 Mar 21 doi: 10.1177/1089253214528048. PMID: 24659409
Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.
Gatzoulis MA, Shore D, Yacoub M, Shinebourne EA
Br Heart J 1994 Jun;71(6):579-83. doi: 10.1136/hrt.71.6.579. PMID: 8043343Free PMC Article

Therapy

Outcomes after implantation of right-sided mechanical valve prostheses in congenital heart disease.
Abozied O, Miranda W, Younis A, Burchill L, Jain C, Connolly H, Dearani J, Stephens EH, Egbe AC
Heart 2023 Nov 10;109(23):1765-1771. doi: 10.1136/heartjnl-2023-322666. PMID: 37407219
Cor triatriatum sinister.
Kadner A, Meszaros K, Mueller C, Schoenhoff F, Hutter D, Carrel T
Multimed Man Cardiothorac Surg 2014;2014 Epub 2014 May 30 doi: 10.1093/mmcts/mmu005. PMID: 24878580
Surgical management of complete atrioventricular septal defect with tetralogy of fallot.
Hu R, Zhang H, Xu Z, Liu J, Su Z, Ding W
Ann Thorac Cardiovasc Surg 2014;20(5):341-6. Epub 2013 Oct 3 doi: 10.5761/atcs.oa.12-02198. PMID: 24088923
Surgical strategy for atrioventricular septal defect and tetralogy of Fallot or double-outlet right ventricle.
Raju V, Burkhart HM, Rigelman Hedberg N, Eidem BW, Li Z, Connolly H, Schaff HV, Dearani JA
Ann Thorac Surg 2013 Jun;95(6):2079-84; discussion 2084-5. Epub 2013 Apr 18 doi: 10.1016/j.athoracsur.2013.02.016. PMID: 23602064
Outcome after repair of atrioventricular septal defect with tetralogy of Fallot.
Shuhaiber JH, Robinson B, Gauvreau K, Breitbart R, Mayer JE, Del Nido PJ, Pigula F
J Thorac Cardiovasc Surg 2012 Feb;143(2):338-43. Epub 2011 Sep 8 doi: 10.1016/j.jtcvs.2011.05.031. PMID: 21855095

Prognosis

Routine Perioperative Esmolol After Infant Tetralogy of Fallot Repair: Single-Center Retrospective Study of Hemodynamics.
Affolter JT, Well A, Gottlieb EA, Fraser CD Jr
Pediatr Crit Care Med 2022 Dec 1;23(12):e583-e589. Epub 2022 Oct 5 doi: 10.1097/PCC.0000000000003088. PMID: 36200768
Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect.
Ramgren JJ, Zindovic I, Nozohoor S, Gustafsson R, Hakacova N, Sjögren J
J Thorac Cardiovasc Surg 2022 Apr;163(4):1437-1444. Epub 2021 Aug 20 doi: 10.1016/j.jtcvs.2021.08.039. PMID: 34503843
Predictors of reoperation and mortality after complete atrioventricular septal defect repair.
Fong LS, Betts K, Ayer J, Andrews D, Nicholson IA, Winlaw DS, Orr Y; Australian CAVSD study group
Eur J Cardiothorac Surg 2021 Dec 27;61(1):45-53. doi: 10.1093/ejcts/ezab221. PMID: 34002204
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
Incidence and Mortality of Adults With Pulmonary Hypertension and Congenital Heart Disease.
Schwartz SS, Madsen N, Laursen HB, Hirsch R, Olsen MS
Am J Cardiol 2018 Jun 15;121(12):1610-1616. Epub 2018 Mar 14 doi: 10.1016/j.amjcard.2018.02.051. PMID: 29655882

Clinical prediction guides

National population-based estimates for major birth defects, 2016-2020.
Stallings EB, Isenburg JL, Rutkowski RE, Kirby RS, Nembhard WN, Sandidge T, Villavicencio S, Nguyen HH, McMahon DM, Nestoridi E, Pabst LJ; National Birth Defects Prevention Network
Birth Defects Res 2024 Jan;116(1):e2301. doi: 10.1002/bdr2.2301. PMID: 38277408Free PMC Article
Predictors of reoperation and mortality after complete atrioventricular septal defect repair.
Fong LS, Betts K, Ayer J, Andrews D, Nicholson IA, Winlaw DS, Orr Y; Australian CAVSD study group
Eur J Cardiothorac Surg 2021 Dec 27;61(1):45-53. doi: 10.1093/ejcts/ezab221. PMID: 34002204
Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Japan and Europe.
Hörer J, Hirata Y, Tachimori H, Ono M, Vida V, Herbst C, Kansy A, Jacobs JP, Tobota Z, Sakamoto K, Ebels T, Maruszewski B
World J Pediatr Congenit Heart Surg 2021 May;12(3):312-319. doi: 10.1177/2150135120988634. PMID: 33942682
Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.
Minnella GP, Crupano FM, Syngelaki A, Zidere V, Akolekar R, Nicolaides KH
Ultrasound Obstet Gynecol 2020 May;55(5):637-644. doi: 10.1002/uog.21956. PMID: 31875326
National population-based estimates for major birth defects, 2010-2014.
Mai CT, Isenburg JL, Canfield MA, Meyer RE, Correa A, Alverson CJ, Lupo PJ, Riehle-Colarusso T, Cho SJ, Aggarwal D, Kirby RS; National Birth Defects Prevention Network
Birth Defects Res 2019 Nov 1;111(18):1420-1435. Epub 2019 Oct 3 doi: 10.1002/bdr2.1589. PMID: 31580536Free PMC Article

Recent systematic reviews

High-risk genes involved in common septal defects of congenital heart disease.
Chaithra S, Agarwala S, Ramachandra NB
Gene 2022 Oct 5;840:146745. Epub 2022 Jul 18 doi: 10.1016/j.gene.2022.146745. PMID: 35863714
Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.
Lenko E, Kulyabin Y, Zubritskiy A, Gorbatykh Y, Naberukhin Y, Nichay N, Bogachev-Prokophiev A, Karaskov A
Interact Cardiovasc Thorac Surg 2018 Jan 1;26(1):98-105. doi: 10.1093/icvts/ivx267. PMID: 29049707

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