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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

Results of surgical treatment for infective endocarditis in children.
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

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.
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
20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries.
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
Long-Term Results after Fallot Repair.
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
Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349
Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management.
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

Diagnosis

Long-Term Results after Fallot Repair.
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
Comprehensive diagnostic catheterization in children with major aortopulmonary collateral arteries: A review of catheterization technique and anatomic nomenclature.
Adamson GT, Peng LF, Perry SB, Hanley FL, McElhinney DB
Catheter Cardiovasc Interv 2022 Mar;99(4):1129-1137. Epub 2021 Nov 20 doi: 10.1002/ccd.30013. PMID: 34800077
Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.
Zucker EJ
Pediatr Radiol 2022 Dec;52(13):2485-2497. Epub 2021 Aug 24 doi: 10.1007/s00247-021-05179-5. PMID: 34427695
Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus.
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
Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349

Therapy

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants.
Sengupta A, Lee JM, Gauvreau K, Colan SD, Del Nido PJ, Mayer JE Jr, Nathan M
J Thorac Cardiovasc Surg 2023 Dec;166(6):1718-1728.e4. Epub 2023 May 8 doi: 10.1016/j.jtcvs.2023.04.014. PMID: 37164053
Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals.
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
Pulmonary vasodilator therapy in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals: case series and review of literature.
Apostolopoulou SC, Vagenakis G, Rammos S
Cardiol Young 2017 Nov;27(9):1861-1864. Epub 2017 Aug 8 doi: 10.1017/S1047951117001548. PMID: 28786367
Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals.
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
Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.
Lim ZS, Vettukattill JJ, Salmon AP, Veldtman GR
Int J Cardiol 2008 Oct 13;129(3):339-43. Epub 2007 Nov 14 doi: 10.1016/j.ijcard.2007.08.016. PMID: 18006164

Prognosis

Long-Term Results after Fallot Repair.
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
Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.
Zucker EJ
Pediatr Radiol 2022 Dec;52(13):2485-2497. Epub 2021 Aug 24 doi: 10.1007/s00247-021-05179-5. PMID: 34427695
Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.
Quinlan CA, Latham GJ, Joffe D, Ross FJ
Semin Cardiothorac Vasc Anesth 2021 Sep;25(3):218-228. doi: 10.1177/10892532211027395. PMID: 34380349
Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management.
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
Tetralogy of Fallot with major aortopulmonary collaterals: early total repair.
McElhinney DB, Reddy VM, Hanley FL
Pediatr Cardiol 1998 Jul-Aug;19(4):289-96. doi: 10.1007/s002469900312. PMID: 9636252

Clinical prediction guides

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.
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
Long-Term Results after Fallot Repair.
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
Tetralogy of Fallot: aorto-pulmonary collaterals and pulmonary arteries have distinctly different transcriptomes.
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
Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance.
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
Tetralogy of Fallot with major aortopulmonary collateral arteries.
Ramsay JM, Macartney FJ, Haworth SG
Br Heart J 1985 Feb;53(2):167-72. doi: 10.1136/hrt.53.2.167. PMID: 3966958Free PMC Article

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