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Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

MedGen UID:
868824
Concept ID:
C4023234
Congenital Abnormality
HPO: HP:0011678

Definition

A type of tetralogy of Fallot with pulmonary atresia in which all pulmonary blood flow is derived from major aortopulmonary collateral arteries (MAPCA). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

Professional guidelines

PubMed

Tansel T, Onursal E, Eker R, Ertugrul T, Dayioglu E
Cardiol Young 2005 Dec;15(6):621-6. doi: 10.1017/S1047951105001800. PMID: 16297257

Recent clinical studies

Etiology

McElhinney DB, Asija R, Zhang Y, Jaggi A, Shek J, Peng LF, Boltz MG, Ma M, Martin E, Hanley FL
J Am Coll Cardiol 2023 Sep 19;82(12):1206-1222. doi: 10.1016/j.jacc.2023.06.041. PMID: 37704311
Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349
Ganigara M, Sagiv E, Buddhe S, Bhat A, Chikkabyrappa SM
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):208-217. Epub 2020 May 26 doi: 10.1177/1089253220920480. PMID: 32450763
Sidell DR, Koth AM, Bauser-Heaton H, McElhinney DB, Wise-Faberowski L, Tracy MC, Hanley FL, Asija R; Pulmonary Artery Reconstruction Program at Stanford University
Pediatr Pulmonol 2017 Dec;52(12):1599-1604. Epub 2017 May 15 doi: 10.1002/ppul.23732. PMID: 28504356

Diagnosis

Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174
Zucker EJ
Pediatr Radiol 2022 Dec;52(13):2485-2497. Epub 2021 Aug 24 doi: 10.1007/s00247-021-05179-5. PMID: 34427695
Gindes L, Salem Y, Gasnier R, Raucher A, Tamir A, Assa S, Weissman-Brenner A, Weisz B, Kasif E, Achiron R
J Matern Fetal Neonatal Med 2022 Dec;35(25):5400-5408. Epub 2021 Feb 1 doi: 10.1080/14767058.2021.1881475. PMID: 33525939
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349
Sidell DR, Koth AM, Bauser-Heaton H, McElhinney DB, Wise-Faberowski L, Tracy MC, Hanley FL, Asija R; Pulmonary Artery Reconstruction Program at Stanford University
Pediatr Pulmonol 2017 Dec;52(12):1599-1604. Epub 2017 May 15 doi: 10.1002/ppul.23732. PMID: 28504356

Therapy

Wise-Faberowski L, Irvin M, Sidell DR, Rajashekara S, Asija R, Chan FP, Hanley FL, McElhinney DB
Cardiol Young 2019 May;29(5):610-614. Epub 2019 May 2 doi: 10.1017/S1047951119000301. PMID: 31044684
Koth AM, Sakarovitch C, Sidell DR, Schultz LM, Freccero A, Rizzuto S, Hanley FL, Asija R
Cardiol Young 2018 Nov;28(11):1329-1332. Epub 2018 Aug 2 doi: 10.1017/S1047951118001270. PMID: 30070195
Apostolopoulou SC, Vagenakis G, Rammos S
Cardiol Young 2017 Nov;27(9):1861-1864. Epub 2017 Aug 8 doi: 10.1017/S1047951117001548. PMID: 28786367
Sidell DR, Koth AM, Bauser-Heaton H, McElhinney DB, Wise-Faberowski L, Tracy MC, Hanley FL, Asija R; Pulmonary Artery Reconstruction Program at Stanford University
Pediatr Pulmonol 2017 Dec;52(12):1599-1604. Epub 2017 May 15 doi: 10.1002/ppul.23732. PMID: 28504356
Asija R, Hanley FL, Roth SJ
Pediatr Crit Care Med 2013 May;14(4):384-9. doi: 10.1097/PCC.0b013e318272062b. PMID: 23439458

Prognosis

Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174
Zucker EJ
Pediatr Radiol 2022 Dec;52(13):2485-2497. Epub 2021 Aug 24 doi: 10.1007/s00247-021-05179-5. PMID: 34427695
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349
Ganigara M, Sagiv E, Buddhe S, Bhat A, Chikkabyrappa SM
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):208-217. Epub 2020 May 26 doi: 10.1177/1089253220920480. PMID: 32450763
Carotti A
World J Pediatr Congenit Heart Surg 2020 Jan;11(1):34-38. doi: 10.1177/2150135119884914. PMID: 31835990

Clinical prediction guides

Clouthier KL, Taylor AC, Xuhuai J, Liu Y, Parker S, Van Eyk J, Reddy S
World J Pediatr Congenit Heart Surg 2024 Mar;15(2):162-173. Epub 2023 Dec 21 doi: 10.1177/21501351231213626. PMID: 38128927
Zheng WC, Buratto E, Caldaroni F, Ishigami S, Konstantinov IE, d'Udekem Y
World J Pediatr Congenit Heart Surg 2023 Nov;14(6):716-722. doi: 10.1177/21501351231181109. PMID: 37933694
Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174
Ma X, Barboza LA, Siyahian A, Reinhartz O, Maeda K, Reddy VM, Hanley FL, Riemer RK
Pediatr Res 2014 Oct;76(4):341-6. Epub 2014 Jul 7 doi: 10.1038/pr.2014.101. PMID: 25000348
Asija R, Roth SJ, Hanley FL, Peng L, Liu K, Abbott J, Zhuo H, Matthay M
J Thorac Cardiovasc Surg 2014 Oct;148(4):1560-5. Epub 2014 Jan 22 doi: 10.1016/j.jtcvs.2014.01.017. PMID: 24534681

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