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Congenitally uncorrected transposition of the great arteries with cardiac malformation

MedGen UID:
1843053
•Concept ID:
C5679811
•
Congenital Abnormality
Synonyms: congenitally uncorrected transposition of the great arteries with cardiac malformation; Congenitally uncorrected transposition of the great vessels with cardiac malformation; congenitally uncorrected transposition of the great vessels with cardiac malformation; TGA with cardiac malformation
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016303
Orphanet: ORPHA216729

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenitally uncorrected transposition of the great arteries with cardiac malformation

Recent clinical studies

Etiology

Multidisciplinary management of a pregnant woman with uncorrected transposition of the great arteries at term pregnancy: A case report.
Castillo Reyther RA, Ling Garcia JP, Krebs Larraga K, Alvarado Jalomo SA, Romero Becerra IM, Guerra Hernandez M, Urbina Gonzalez H, Vazquez Morales KG, Morales Montesinos E, Kway VB
Int J Gynaecol Obstet 2022 Oct;159(1):320-323. Epub 2022 Jul 31 doi: 10.1002/ijgo.14329. PMID: 35789087
Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease.
Trojnarska O, Gwizdała A, Katarzyński S, Katarzyńska A, Szyszka A, Lanocha M, Grajek S, Kramer L
Cardiol J 2009;16(2):133-41. PMID: 19387960
Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease.
Bhat AH, Sahn DJ
Curr Opin Pediatr 2004 Oct;16(5):500-7. doi: 10.1097/01.mop.0000140996.24408.1a. PMID: 15367842
Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results.
Devaney EJ, Charpie JR, Ohye RG, Bove EL
J Thorac Cardiovasc Surg 2003 Mar;125(3):500-7. doi: 10.1067/mtc.2003.158. PMID: 12658191
Cerebral emboli during cardiac surgery in children.
O'Brien JJ, Butterworth J, Hammon JW, Morris KJ, Phipps JM, Stump DA
Anesthesiology 1997 Nov;87(5):1063-9. doi: 10.1097/00000542-199711000-00009. PMID: 9366457

Diagnosis

Intra-isthmus reentry associated with uncorrected double inlet left ventricle and transposition of the great arteries.
Dhillon PS, Li A, Gonna H, Ward DE
Congenit Heart Dis 2013 Mar-Apr;8(2):E56-60. Epub 2012 Jan 6 doi: 10.1111/j.1747-0803.2011.00616.x. PMID: 22222181
Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease.
Trojnarska O, Gwizdała A, Katarzyński S, Katarzyńska A, Szyszka A, Lanocha M, Grajek S, Kramer L
Cardiol J 2009;16(2):133-41. PMID: 19387960
Congenitally corrected transposition and degenerative severe aortic stenosis.
Faganello G, Nelson M, Stuart G
Cardiol Young 2008 Oct;18(5):523-4. Epub 2008 Aug 28 doi: 10.1017/S1047951108002679. PMID: 18752714
Congenitally corrected transposition of great arteries with ischemic symptoms in middle age.
Zaidi SM, Al-Sharary MM, Al-Khuwaitir TS, Sajid NU
Saudi Med J 2007 Oct;28(10):1597-9. PMID: 17914527
Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease.
Bhat AH, Sahn DJ
Curr Opin Pediatr 2004 Oct;16(5):500-7. doi: 10.1097/01.mop.0000140996.24408.1a. PMID: 15367842

Therapy

Congenitally corrected transposition of great arteries with ischemic symptoms in middle age.
Zaidi SM, Al-Sharary MM, Al-Khuwaitir TS, Sajid NU
Saudi Med J 2007 Oct;28(10):1597-9. PMID: 17914527
Cerebral emboli during cardiac surgery in children.
O'Brien JJ, Butterworth J, Hammon JW, Morris KJ, Phipps JM, Stump DA
Anesthesiology 1997 Nov;87(5):1063-9. doi: 10.1097/00000542-199711000-00009. PMID: 9366457

Prognosis

Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease.
Trojnarska O, Gwizdała A, Katarzyński S, Katarzyńska A, Szyszka A, Lanocha M, Grajek S, Kramer L
Cardiol J 2009;16(2):133-41. PMID: 19387960
'Hair-on-end' skull changes resembling thalassemia caused by marrow expansion in uncorrected complex cyanotic heart disease.
Walor DM, Berdon WE, Westra SJ
Pediatr Radiol 2005 Jul;35(7):698-701. Epub 2005 Mar 18 doi: 10.1007/s00247-005-1403-0. PMID: 15776229
Combined arterial switch and Senning operation for congenitally corrected transposition of the great arteries: patient selection and intermediate results.
Devaney EJ, Charpie JR, Ohye RG, Bove EL
J Thorac Cardiovasc Surg 2003 Mar;125(3):500-7. doi: 10.1067/mtc.2003.158. PMID: 12658191

Clinical prediction guides

Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease.
Trojnarska O, Gwizdała A, Katarzyński S, Katarzyńska A, Szyszka A, Lanocha M, Grajek S, Kramer L
Cardiol J 2009;16(2):133-41. PMID: 19387960

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