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Transitional atrioventricular canal defect

MedGen UID:
868878
•Concept ID:
C4023289
•
Congenital Abnormality
HPO: HP:0011578

Definition

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

Term Hierarchy

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

Recent clinical studies

Etiology

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
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
Surgical Outcomes in Children With Partial and Transitional Atrioventricular Septal Defect.
Nakayama Y, Shinkawa T, Hoki R, Kobayashi K, Yoshida H, Sato Y, Niinami H
World J Pediatr Congenit Heart Surg 2022 Jul;13(4):451-457. doi: 10.1177/21501351221100140. PMID: 35757952
Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.
Krupickova S, Morgan GJ, Cheang MH, Rigby ML, Franklin RC, Battista A, Spanaki A, Bonello B, Ghez O, Anderson D, Tsang V, Michielon G, Marek J, Fraisse A
Heart 2018 Sep;104(17):1411-1416. Epub 2017 Dec 28 doi: 10.1136/heartjnl-2017-312195. PMID: 29288192
Technical Performance Score Predicts Partial/Transitional Atrioventricular Septal Defect Outcomes.
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
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

Heart-Lung Transplant via an Eighth-Time Sternotomy.
Riggs KW, Chin CW, Bryant R, Zafar F, Chin C, Schecter MG, Morales DLS
World J Pediatr Congenit Heart Surg 2021 Jan;12(1):136-138. Epub 2019 Apr 24 doi: 10.1177/2150135118815876. PMID: 31018757
Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.
Krupickova S, Morgan GJ, Cheang MH, Rigby ML, Franklin RC, Battista A, Spanaki A, Bonello B, Ghez O, Anderson D, Tsang V, Michielon G, Marek J, Fraisse A
Heart 2018 Sep;104(17):1411-1416. Epub 2017 Dec 28 doi: 10.1136/heartjnl-2017-312195. PMID: 29288192
Technical Performance Score Predicts Partial/Transitional Atrioventricular Septal Defect Outcomes.
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
Atrioventricular valve regurgitation in the fetus with atrioventricular canal defect: transition from prenatal to postnatal life.
Davey B, Thacker D, Rychik J
Pediatr Cardiol 2013;34(8):1797-802. Epub 2013 May 12 doi: 10.1007/s00246-013-0711-7. PMID: 23666083Free PMC Article
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

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
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
Contemporary results after repair of partial and transitional atrioventricular septal defects.
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
Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.
Krupickova S, Morgan GJ, Cheang MH, Rigby ML, Franklin RC, Battista A, Spanaki A, Bonello B, Ghez O, Anderson D, Tsang V, Michielon G, Marek J, Fraisse A
Heart 2018 Sep;104(17):1411-1416. Epub 2017 Dec 28 doi: 10.1136/heartjnl-2017-312195. PMID: 29288192
Surgical interventions for atrioventricular septal defect subtypes: the pediatric heart network experience.
Kaza AK, Colan SD, Jaggers J, Lu M, Atz AM, Sleeper LA, McCrindle BW, Lambert LM, Margossian R, Lacro RV, Richmond ME, Natarajan S, Minich LL; Pediatric Heart Network Investigators
Ann Thorac Surg 2011 Oct;92(4):1468-75; discussion 1475. Epub 2011 Aug 26 doi: 10.1016/j.athoracsur.2011.04.109. PMID: 21872212Free PMC Article
Reoperation for left atrioventricular valve regurgitation after atrioventricular septal defect repair.
Malhotra SP, Lacour-Gayet F, Mitchell MB, Clarke DR, Dines ML, Campbell DN
Ann Thorac Surg 2008 Jul;86(1):147-51; discussion 151-2. doi: 10.1016/j.athoracsur.2008.03.040. PMID: 18573414

Prognosis

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
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
Contemporary results after repair of partial and transitional atrioventricular septal defects.
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
Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.
Krupickova S, Morgan GJ, Cheang MH, Rigby ML, Franklin RC, Battista A, Spanaki A, Bonello B, Ghez O, Anderson D, Tsang V, Michielon G, Marek J, Fraisse A
Heart 2018 Sep;104(17):1411-1416. Epub 2017 Dec 28 doi: 10.1136/heartjnl-2017-312195. PMID: 29288192
Technical Performance Score Predicts Partial/Transitional Atrioventricular Septal Defect Outcomes.
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
Partial and transitional atrioventricular septal defect outcomes.
Minich LL, Atz AM, Colan SD, Sleeper LA, Mital S, Jaggers J, Margossian R, Prakash A, Li JS, Cohen MS, Lacro RV, Klein GL, Hawkins JA; Pediatric Heart Network Investigators
Ann Thorac Surg 2010 Feb;89(2):530-6. doi: 10.1016/j.athoracsur.2009.10.047. PMID: 20103337Free PMC Article

Clinical prediction guides

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability.
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
Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.
Krupickova S, Morgan GJ, Cheang MH, Rigby ML, Franklin RC, Battista A, Spanaki A, Bonello B, Ghez O, Anderson D, Tsang V, Michielon G, Marek J, Fraisse A
Heart 2018 Sep;104(17):1411-1416. Epub 2017 Dec 28 doi: 10.1136/heartjnl-2017-312195. PMID: 29288192
Technical Performance Score Predicts Partial/Transitional Atrioventricular Septal Defect Outcomes.
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
Atrioventricular valve regurgitation in the fetus with atrioventricular canal defect: transition from prenatal to postnatal life.
Davey B, Thacker D, Rychik J
Pediatr Cardiol 2013;34(8):1797-802. Epub 2013 May 12 doi: 10.1007/s00246-013-0711-7. PMID: 23666083Free PMC Article
Partial and transitional atrioventricular septal defect outcomes.
Minich LL, Atz AM, Colan SD, Sleeper LA, Mital S, Jaggers J, Margossian R, Prakash A, Li JS, Cohen MS, Lacro RV, Klein GL, Hawkins JA; Pediatric Heart Network Investigators
Ann Thorac Surg 2010 Feb;89(2):530-6. doi: 10.1016/j.athoracsur.2009.10.047. PMID: 20103337Free PMC Article

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