Skip to main content
Log in

Opinion statement

  • Untreated, the prognosis for patients with tricuspid atresia (TA) is poor. Recent advances in medical and surgical therapy, particularly the application of Fontan principle, have markedly improved the long-term outlook for children with this condition.

  • Palliative procedures to normalize the pulmonary blood flow and to relieve interatrial or interventricular obstruction should be undertaken promptly.

  • Staged total cavopulmonary connection to bypass the right atrium and right ventricle by an initial bidirectional Glenn procedure and followed by extracardiac conduit diversion of inferior vena caval flow into the pulmonary arteries appears to be the current procedure of choice in the surgical management of TA.

  • Total cavopulmonary diversion appears to be superior to conventional Fontan-Kreutzer operations, but long-term follow-up results are needed to confirm this impression.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. Rao PS: Terminology: tricuspid atresia or univentricular heart? In Tricuspid Atresia. Edited by Rao PS. Mount Kisco, NY: Futura Publishing Co.; 1982:3–6.

    Google Scholar 

  2. Rao PS: Tricuspid atresia. In Pediatric Cardiovascular edicine. Edited by Moller JH, Hoffman JIE. New York:Churchill Livingstone; 2000:421–441. up-to-date comprehensive review of diagnosis and Management of patients with tricuspid atresia

    Google Scholar 

  3. Rao PS: Classification of tricuspid atresia. In Tricuspid Atresia, edn 2. Edited by Rao PS. Mount Kisco, NY:Futura Publishing Co.; 1992:59–79.

    Google Scholar 

  4. Rao PS: A unified classification for tricuspid atresia. Am Heart J 1980, 99:799–804.

    Article  PubMed  CAS  Google Scholar 

  5. Rao PS: Cardiac catheterization in tricuspid atresia.In Tricuspid Atresia. Edited by Rao PS. Mount Kisco, NY:Futura Publishing Co.; 1982:153–178.

    Google Scholar 

  6. Rao PS: Natural history of ventricular septal defects in tricuspid atresia. In Tricuspid Atresia, edn 2. Edited byRao PS. Mount Kisco, NY: Futura Publishing Co.;1992:261–293.

    Google Scholar 

  7. Rao PS, Alpert BS, Covitz W: Left ventricular function in tricuspid atresia. In Tricuspid Atresia, edn 2. Edited byRao PS. Mount Kisco, NY: Futura Publishing Co.;1992:247–259.

    Google Scholar 

  8. Rao PS: Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. Br Heart J 1977, 39:276–288.

    PubMed  CAS  Google Scholar 

  9. Rao PS: Further observations on the spontaneous closure of physiologically advantageous ventricular septal defects in tricuspid atresia: surgical implications. Ann Thorac Surg 1983, 35:121–131.

    Article  PubMed  CAS  Google Scholar 

  10. Rao PS: Tricuspid atresia. In Fetal and Neonatal Cardiology, Edited by Long WA, Philadelphia, PA:W.B. Saunders; 1990:525–540.

    Google Scholar 

  11. Rao PS: Tricuspid atresia: anatomy, imaging and natural history. In Congenital Heart Disease. Edited by Freedom RM, Braunwald E. Philadelphia: Current Medicine Inc.;1997:14.1–14.14. An excellent pictorial representation of varois anatomic types of TA, as well of the natural history.

    Google Scholar 

  12. Chopra PS, Rao PS: Corrective surgery for tricuspid atresia: which modifications of Fontan-Kreutzer procedure should be used? A review. Am Heart J 1992, 123:758–767.

    Article  PubMed  CAS  Google Scholar 

  13. Rao PS, Thapar MK, Galal O, Wilson AD: Follow-up results of balloon angioplasty of native coarctation in neonates and infants. Am Heart J 1990, 120:1310–1314.

    Article  PubMed  CAS  Google Scholar 

  14. Dajani AS, Taubert KA, Wilson W, et al.: Prevention of bacterial endocarditis: recommendations by the American Heart Association. J Am Med Assoc 1997, 277:1794–1801.

    Article  CAS  Google Scholar 

  15. Park SC, Neches WH, Zuberbuhler JR, et al.: Clinical use of blade atrial septostomy. Circulation 1978, 58:600–606.

    PubMed  CAS  Google Scholar 

  16. Rao PS: Transcatheter blade atrial septostomy. Cathet Cardiovasc Diagn 1984, 10:335–342.

    Article  PubMed  CAS  Google Scholar 

  17. McCredie RM, Lee CL, Swinburn MJ, et al.: Balloon dilatation pulmonary valvuloplasty in pulmonary stenosis. Aust New Zealand J Med 1986, 16:20–23.

    CAS  Google Scholar 

  18. Rao PS, Wilson AD, Thapar MK, Brais M: Balloon pulmonary valvuloplasty in the management of cyanotic congenital heart defects. Cathet Cardiovasc Diagn 1992, 25:16–24.

    Article  PubMed  CAS  Google Scholar 

  19. Rao PS, Levy JM, Chopra PS: Balloon angioplasty of stenosed Blalock-Taussig anastomosis: role of balloon on a wire in dilating occluded shunts. Am Heart J 1990, 120:1173–1178.

    Article  PubMed  CAS  Google Scholar 

  20. Balfour IC, Rao PS: Pulmonary stenosis. Current Treat Options Cardiovasc Med 2000, 2:489–498. An excellent review of transcatheter management of pulmonary stenosis.

    Google Scholar 

  21. Rao PS: Static balloon dilatation of the atrial septum [editorial]. Am Heart J 1993, 125:1824–1827.

    Article  PubMed  CAS  Google Scholar 

  22. Rao PS, Chandar JS, Sideris EB: Role of inverted buttoned device in transcatheter occlusion of atrial septal defect or patent foramen ovale with right-to-left atrial shunting associated with previously operated complex congenital cardiac anomalies. Am J Cardiol 1997, 80:914–921.

    Article  PubMed  CAS  Google Scholar 

  23. Kidd L: Balloon atrial septostomy: current perspective. In Transcatheter Therapy in Pediatric Cardiology. Edited by Rao PS. New York: Wiley-Liss; 1993:7–15.

    Google Scholar 

  24. Rao PS, Covitz W, Chopra PS: Principles of palliative management of patients with tricuspid atresia. In Tricuspid Atresia, edn 2. Edited by Rao PS. Mount Kisco, NY: Futura Publishing Co.; 1992:297–320.

    Google Scholar 

  25. Annecchino FP, Fontan F, Chauve A, et al.: An operation for the correction of tricuspid atresia. Ann Thorac Surg 1979, 29:317–321.

    Article  Google Scholar 

  26. Ottenkamp J, Wenink ACG, Quaegebeur JM, et al.: Tricuspid atresia: morphology of the outlet chamber with special emphasis on surgical implications. J Thorac Cardiovasc Surg 1985, 89:597–603.

    PubMed  CAS  Google Scholar 

  27. Billingsley AM, Laks H, Boyce SM, et al.: Definitive repair in some patients with pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1989, 97:746–754.

    PubMed  CAS  Google Scholar 

  28. Bridges ND, Lock JE, Castaneda AR: Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients with increased risk. Circulation 1990, 82:1681–1689.

    PubMed  CAS  Google Scholar 

  29. De Leval MR, Kilner P, Gewilling M, et al.: Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operation. J Thorac Cardiovasc Surg 1988, 96:682–695.

    PubMed  Google Scholar 

  30. Sharma S, Goudy S, Walker P, et al.: In vitro flow experiments for determination of optimal geometry of total cavopulmonary connection for surgical repair of children with functional single ventricle. J Am Coll Cardiol 1996, 27:1264–1269.

    Article  PubMed  CAS  Google Scholar 

  31. Bonnet D, Sidi D, Vouché PR: Absorbable pulmonary artery banding in tricuspid atresia. Ann Thorac Surg 2000, In Press.

  32. Rao PS:Absorbable pulmonary artery band in tricuspid atresia [editorial]. Ann Thorac Surg 2000,

  33. Konertz W, Schneider M, Herwig V, et al.: Modified hemi-Fontan operation and subsequent nonsurgical completion. J Thorac Cardiovasc Surg 1995, 110:865–867. Description of an innovative simplification of the Fontan procedure that avoids two surgical procedures.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Rao, P.S. Tricuspid atresia. Curr Treat Options Cardio Med 2, 507–520 (2000). https://doi.org/10.1007/s11936-000-0046-6

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11936-000-0046-6

Keywords

Navigation