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Complete atrioventricular canal-tetralogy of fallot syndrome

MedGen UID:
768726
•Concept ID:
C3640086
•
Disease or Syndrome
Synonyms: CAVC type C; CAVC-Fallot tetralogy syndrome; CAVC-tetralogy of Fallot; Complete atrioventricular canal defect-tetralogy of Fallot; complete atrioventricular canal type C; complete atrioventricular canal-tetralogy of fallot syndrome; Complete Atrioventricular Septal Defect with Tetralogy of Fallot; Complete atrioventricular septal defect-tetralogy of Fallot; Complete AVSD-tetralogy of Fallot
 
Monarch Initiative: MONDO:0020408
Orphanet: ORPHA99068

Definition

A rare congenital heart anomaly in which there is coexistence of tetralogy of Fallot and complete atrioventricular septal defect. The latter is characterized by defects in the atrial and ventricular septa and a common atrioventricular valve. [from NCI]

Professional guidelines

PubMed

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
Neonatal ECG screening for congenital heart disease in Down syndrome.
Narchi H
Ann Trop Paediatr 1999 Mar;19(1):51-4. doi: 10.1080/02724939992635. PMID: 10605520
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

Recent clinical studies

Etiology

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
Twelve Years of Complete Atrioventricular Septal Defect Repair.
Sena A, Sheytanov V, Liebrich M, Narr A, Schweigmann U, Uhlemann F, Doll N, Tzanavaros I
Rev Port Cir Cardiotorac Vasc 2019 Jul-Sep;26(3):187-193. PMID: 31734969
Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.
Wu MH, Lu CW, Chen HC, Chiu SN, Kao FY, Huang SK
Heart Rhythm 2015 Mar;12(3):604-609. Epub 2014 Nov 24 doi: 10.1016/j.hrthm.2014.11.026. PMID: 25461497
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
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

Diagnosis

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
Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate.
Chaturvedi K, Prasad D, Ashwath R, Strainic JP, Snyder CS
Tex Heart Inst J 2016 Dec;43(6):546-549. Epub 2016 Dec 1 doi: 10.14503/THIJ-15-5692. PMID: 28100981Free PMC Article
22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.
Mercer-Rosa L, Pinto N, Yang W, Tanel R, Goldmuntz E
J Thorac Cardiovasc Surg 2013 Oct;146(4):868-73. Epub 2013 Jan 11 doi: 10.1016/j.jtcvs.2012.12.028. PMID: 23312975Free PMC Article
Cyanotic congenital heart disease with decreased pulmonary blood flow in children.
Waldman JD, Wernly JA
Pediatr Clin North Am 1999 Apr;46(2):385-404. doi: 10.1016/s0031-3955(05)70125-5. PMID: 10218082
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

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
22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.
Mercer-Rosa L, Pinto N, Yang W, Tanel R, Goldmuntz E
J Thorac Cardiovasc Surg 2013 Oct;146(4):868-73. Epub 2013 Jan 11 doi: 10.1016/j.jtcvs.2012.12.028. PMID: 23312975Free PMC Article
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
Adults with Down syndrome: safety and long-term outcome of cardiac operation.
Majdalany DS, Burkhart HM, Connolly HM, Abel MD, Dearani JA, Warnes CA, Schaff HV
Congenit Heart Dis 2010 Jan-Feb;5(1):38-43. doi: 10.1111/j.1747-0803.2009.00349.x. PMID: 20136856
Is complete heart block after surgical closure of ventricular septum defects still an issue?
Andersen HØ, de Leval MR, Tsang VT, Elliott MJ, Anderson RH, Cook AC
Ann Thorac Surg 2006 Sep;82(3):948-56. doi: 10.1016/j.athoracsur.2006.04.030. PMID: 16928514

Prognosis

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
Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.
Wu MH, Lu CW, Chen HC, Chiu SN, Kao FY, Huang SK
Heart Rhythm 2015 Mar;12(3):604-609. Epub 2014 Nov 24 doi: 10.1016/j.hrthm.2014.11.026. PMID: 25461497
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
Biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle.
Devaney EJ, Lee T, Gelehrter S, Hirsch JC, Ohye RG, Anderson RH, Bove EL
Ann Thorac Surg 2010 Feb;89(2):537-42; discussion 542-3. doi: 10.1016/j.athoracsur.2009.10.049. PMID: 20103338
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

Clinical prediction guides

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
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
22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.
Mercer-Rosa L, Pinto N, Yang W, Tanel R, Goldmuntz E
J Thorac Cardiovasc Surg 2013 Oct;146(4):868-73. Epub 2013 Jan 11 doi: 10.1016/j.jtcvs.2012.12.028. PMID: 23312975Free PMC Article
Primary biventricular repair of atrioventricular septal defects: an analysis of reoperations.
Vohra HA, Chia AX, Yuen HM, Vettukattil JJ, Veldtman G, Gnanapragasam J, Roman K, Salmon AP, Haw MP
Ann Thorac Surg 2010 Sep;90(3):830-7. doi: 10.1016/j.athoracsur.2010.03.108. PMID: 20732503
Arrhythmias late after repair of tetralogy of fallot: a Japanese Multicenter Study.
Nakazawa M, Shinohara T, Sasaki A, Echigo S, Kado H, Niwa K, Oyama K, Yokota M, Iwamoto M, Fukushima N, Nagashima M, Nakamura Y; Study Group for Arrhythmias Long-Term After Surgery for Congenital Heart Disease: ALTAS-CHD study
Circ J 2004 Feb;68(2):126-30. doi: 10.1253/circj.68.126. PMID: 14745146

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