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Pediatric Cardiology

, Volume 5, Issue 3, pp 217–220 | Cite as

Complex transposition with interrupted right aortic arch and partial Di George syndrome: Successful palliation with combined medical and surgical therapy

  • Walter J. Duncan
  • Michael J. Tyrrell
  • Baikunth Bharadwaj
  • Alan M. Rosenberg
  • Maria-Louise Schroeder
  • William T. Bingham
Case Reports

Summary

A five-day-old infant with transposition of the great arteries, ventricular septal defect, and an interrupted right aortic arch underwent successful balloon septostomy, pulmonary artery banding, and aortic arch repair. The infant also had abnormal facies with severe refractory hypocalcemia and depressed T-lymphocyte number and function believed to represent a partial Di George syndrome. The hypocalcemia resolved following treatment with a vitamin-D analogue, T-cell number increased, and T-cell function improved, but both remained subnormal.

Key words

Interrupted aortic arch Di George syndrome 

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References

  1. 1.
    Bailey LL, Jacobsen JG, Vyhmeister E, Petry E (1978) Interrupted aortic arch complex: successful total correction in the neonate.Ann Thorac Surg 26:66–70CrossRefGoogle Scholar
  2. 2.
    Boyd JD (1950) Development of the thyroid and parathyroid glands and thymus.Anat Rec 7:455–457Google Scholar
  3. 3.
    Cleveland WW, Fogel BD, Brown WT, Kay HEM (1968) Foetal thymic transplant in a case of Di George's syndrome.Lancet 2:1211–1214PubMedCrossRefGoogle Scholar
  4. 4.
    Conley ME, Beckwith JB, Mancer JFK, Tenckhoff L (1979) The spectrum of the Di George syndrome.J Pediatr 94:883–890PubMedCrossRefGoogle Scholar
  5. 5.
    Di George AM (1968) Congenital absence of thymus and its immunologic consequences: concurrence with congenital hypoparathyroidism. In: Bergsma D, Good RA (eds)Immunological deficiency in man. National Foundation—March of Dimes, New York, pp 116–123Google Scholar
  6. 6.
    Freedom RM, Rosen FS, Nadas AS (1972) Congenital cardiovascular disease and anomalies of the third and fourth pharyngeal pouch.Circulation 46:165–172PubMedGoogle Scholar
  7. 7.
    Harvey JC, Dungan WT, Elders MJ, Hughes ER (1970) Third and fourth pharyngeal pouch syndrome, associated vascular anomalies and hypocalcemic seizures.Clin Pediatr 9:496–498Google Scholar
  8. 8.
    Huff DS, Lischner HW (1973) Congenital malformations associated with Di George syndrome.Am J Pathol 71:6aGoogle Scholar
  9. 9.
    Lobdell DH (1959) Congenital absence of the parathyroid glands.Arch Pathol 67:412–415Google Scholar
  10. 10.
    Losman JG, Joffe HS, Beck W, Barnard C (1974) Successful total repair of interrupted aortic arch associated with ventricular septal defect and large patent ductus arteriosus.Am J Cardiol 33:566–571PubMedCrossRefGoogle Scholar
  11. 11.
    Murphy DA, Collins G, Dobell ARC (1971) Surgical correction of type A congenital aortic arch interruption.Ann Thorac Surg 11:593–597PubMedCrossRefGoogle Scholar
  12. 12.
    Norton JB, Ullyot DJ, Stewart ET, Rudolph AM, Edmunds LH (1970) Aortic arch atresia with transposition of the great arteries: physiologic considerations and surgical management.Surgery 67:1011–1016PubMedGoogle Scholar
  13. 13.
    Pabst HF, Wright WC, LeRiche J, Stiehm ER (1976) Partial Di George syndrome with substantial cell-mediated immunity.Am J Dis Child 130:316–319PubMedGoogle Scholar
  14. 14.
    Sturn JT, Van Heekeren DW, Borkat G (1981) Surgical treatment of interrupted aortic arch in infancy with expanded polytetrafluoroethylene grafts.J Thorac Cardiovasc Surg 81:245–250Google Scholar
  15. 15.
    Trusler GA, Izukawa T (1975) Interrupted septal defect: direct repair through a median sternotomy incision.J Thorac Cardiovasc Surg 69:126–131PubMedGoogle Scholar
  16. 16.
    Waldhausen JA, Boruchow I, Miller WW, Rashkind WJ (1969) Transposition of the great arteries with ventricular septal defect.Circulation (Suppl 1)39:215–221Google Scholar

Copyright information

© Springer-Verlag 1984

Authors and Affiliations

  • Walter J. Duncan
    • 1
  • Michael J. Tyrrell
    • 1
  • Baikunth Bharadwaj
    • 2
  • Alan M. Rosenberg
    • 1
  • Maria-Louise Schroeder
    • 1
  • William T. Bingham
    • 1
  1. 1.Department of PediatricsUniversity Hospital, University of SaskatchewanSaskatoonCanada
  2. 2.Department of SurgeryUniversity Hospital, University of SaskatchewanSaskatoonCanada

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