The pulmonary circulation in transposition of the great arteries

  • S. G. Haworth
Conference paper


In the days before a Rashkind septostomy the average life-expectancy was 1.3 months in babies with transposition of the great arteries (TGA) and an intact ventricular septum (IVS), and 3.4 months in those with a TGA and ventricular septal defect (VSD) [14]. Those with a VSD survived longer because mixing of the pulmonary and systemic circulations persisted after closure of the foramen ovale and ductus arteriosus. A high effective pulmonary blood flow ensures a greater systemic arterial oxygen saturation. The presence of a large VSD however, leads to the rapid development of severe pulmonary vascular disease [6, 17]. In the presence of an intact ventricular septum a patent ductus arteriosus also leads to the early development of pulmonary vascular disease and should be closed surgically at 3 months of age if it has not already closed spontaneously. In transposition as a whole, patient outcome correlates with the pulmonary arterial pressure [13]. The outlook for patients with an IVS was transformed by the introduction of the Senning operation in 1959 and the Mustard repair in 1964, but not until the revival of the arterial switch procedure in 1976 did the long-term outlook improve for those with a VSD [16,18,20,21,22]. This procedure enables an early repair to be carried out with an acceptable risk, before severe pulmonary vascular disease has developed.


Ventricular Septal Defect Ventricular Septal Defect Pulmonary Vascular Disease Intimal Proliferation Intact Ventricular Septum 
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  1. 1.
    Adatia IT, Haworth SG (1990) Advanced pulmonary vascular disease: 24 hour urinary excretion of vasoactive eicosanoids. Circulation (Suppl III) 82:A0395Google Scholar
  2. 2.
    Adatia I, Haworth SG (1991) Endothelin in pulmonary hypertensive congenital heart disease. Am Thorac Soc (abstract) 143: A403Google Scholar
  3. 3.
    Allen K, Haworth SG (1988) Human postnatal pulmonary arterial remodelling: ultrastructural studies of smooth muscle cell and connective tissue maturation. Lab Invest 59:702–709PubMedGoogle Scholar
  4. 4.
    Allen K, Haworth SG (1989) Cytoskeletal features of immature pulmonary vascular smooth muscle cells and the influence of pulmonary hypertension on normal human development. J Pathol 158:311–317PubMedCrossRefGoogle Scholar
  5. 5.
    Bowyer JJ, Busst CM, Denison DM, Shinebourne E A (1986) Effect of long-term oxygen treatment at home in children with pulmonary vascular disease. Br Heart J 55:385–390PubMedCrossRefGoogle Scholar
  6. 6.
    Ferencz C (1966) Transposition of the great vessels. Pathophysiologic considerations based upon a study of the lungs. Circulation 33:232–241CrossRefGoogle Scholar
  7. 7.
    Hall SM, Haworth SG (1991) Onset and evolution of pulmonary vascular disease in young children: abnormal postnatal remodelling studied in lung biopsies. J Pathol (in press)Google Scholar
  8. 8.
    Haworth SG (1984) Pulmonary vascular disease in different types of congenital heart disease. Implications for interpretation of lung biopsy findings in early childhood. Br Heart J 52:557–571PubMedCrossRefGoogle Scholar
  9. 9.
    Haworth SG, Radley-Smith R, Yacoub M (1987) Lung biopsy findings in transposition of the great arteries with ventricular septal defect: potentially reversible pulmonary vascular disease is not always synonymous with operability. J Am Coll Cardiol 9:327–333PubMedCrossRefGoogle Scholar
  10. 10.
    Heath D, Edwards JE (1958) The pathology of hypertensive pulmonary vascular disease. A description of six grades of structural changes in the pulmonary artery with special reference to congenital cardiac septal defect. Circulation 18:533–547PubMedCrossRefGoogle Scholar
  11. 11.
    Jones ODH, Shore DF, Rigby ML, Leijala M, Scallan J, Shinebourne EA, Lincoln JCR (1981) The use of tolazoline hydrochloride as a pulmonary vasodilator in potentially fatal episodes of pulmonary vasoconstriction after cardiac surgery in children. Circulation 64 (Suppl II): 134–139Google Scholar
  12. 12.
    Kriett JM, Kaye MP (1991) The registry of the international society for heart and lung transplantation: Eight offical report. J Heart Lung Transplant 10:491–498PubMedGoogle Scholar
  13. 13.
    Leanage R, Agnetti A, Graham G, Taylor J, Macartney FJ (1981) Factors influencing survival after balloon atrial septostomy for complete transposition of the great arteries. Br Heart J 45:559–572PubMedCrossRefGoogle Scholar
  14. 14.
    Liebman J, Cullum L, Belloc NB (1969) Natural history of transposition of the great arteries. Anatomy and birth and death characteristics. Circulation 40:237–262PubMedCrossRefGoogle Scholar
  15. 15.
    Lindesmith GG, Stiles QR, Tucker BL, Gallaher ME, Stanton RE, Meyer BW (1972) The Mustard operation as a palliative procedure. J Thorac Cardiovasc Surg 63:75–80PubMedGoogle Scholar
  16. 16.
    Mustard WT, Keith JD, Trusler GA, Fowler R, Kidd L (1964) The surgical management of transposition of the great vessels. J Thorac Cardiovasc Surg 48:953–958PubMedGoogle Scholar
  17. 17.
    Newfeld EA, Paul MH, Muster AJ, Idriss FS (1974) Pulmonary vascular disease in complete transposition of the great arteries. A study of 200 patients. Am J Cardiol 34:75–82PubMedCrossRefGoogle Scholar
  18. 18.
    Senning A (1959) Surgical correction of transposition of the great arteries. Surgery 45:966–980PubMedGoogle Scholar
  19. 19.
    Stark J, De Levai M, Macartney F, Taylor J (1980) Surgery for transposition of the great arteries with pulmonary vascular obstructive disease. Pediatr Cardiol 1:241 (abstract)Google Scholar
  20. 20.
    Yacoub MH, Radley-Smith R, Hilton CJ (1976) Anatomical correction of complete transposition of the great arteries and ventricular septal defect in infancy. Br Med J 1:1112–1116PubMedCrossRefGoogle Scholar
  21. 21.
    Yacoub M, Radley-Smith R, Maclaurin R (1977) Two-stage operation for anatomical correction of transposition of the great arteries with intact ventricular septum (abstract). Br Heart J 39:925Google Scholar
  22. 22.
    Yacoub M, Bernhard A, Lange P, Radley-Smith R, Keck E, Stephan E, Heintzen P (1980) Clinical and hemodynamic results of the two-stage anatomic correction of simple transposition of the great arteries. Circulation 62 (Suppl I): 190–196Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

  • S. G. Haworth
    • 1
    • 2
  1. 1.Hospital for Sick ChildrenLondonUK
  2. 2.Hospital for Sick ChildrenLondonUK

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