Staged Reconstruction For HLHS

The Bidirectional Cavopulmonary Shunt
  • Tom R. Karl
Part of the Developments in Cardiovascular Medicine book series (DICM, volume 246)


The Norwood operation (with its technical variations) is the only useful reconstructive surgical procedure for neonates with HLHS and related abnormalities [1]. While the Norwood operation allows the neonate to survive well beyond the first few weeks of life, it is not a stable solution for the long term. The postoperative Norwood physiology presents several problems, some of which center on RV function in the presence of pulmonary recirculation. The volume load that is necessarily imposed on the heart in HLHS may place the RV in an unfavorable position on the Starling curve, which typically has a leftward shift in comparison to the normal LV. The systemic tricuspid valve, which lacks annular definition and is supported by septal-derived papillary muscles, is functionally exquisitely sensitive to volume loading of the RV, especially when required to eject at systemic arterial pressure. Tricuspid insufficiency may thus be the limiting factor for future suitability for a Fontan operation. Finally, the small prosthetic (PTFE) tubes typically employed to create a controlled source of pulmonary blood flow (MBTS) are destined to become obstructed with thrombus, and/or neointima. Even a widely patent shunt may become inadequate as the baby grows. At the same time, the low diastolic arterial pressure typically associated with a MBTS may place the baby at continued risk for myocardial ischemia as activity increases. The presence of the shunt may also contribute to ongoing limitation of growth and distortion of the PAs. For survivors of the Norwood operation, an unacceptably high late mortality has been observed for infants who have not yet undergone further staging operations [2]. For all of these reasons, a further, more definitive procedure is required.


Pulmonary Blood Flow Hypoplastic Left Heart Syndrome Fontan Operation Tricuspid Insufficiency Univentricular Heart 
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  1. 1.
    Norwood WI, Kirklin JK, Sanders JP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol 1980;45:87.PubMedCrossRefGoogle Scholar
  2. 2.
    Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ 3rd.Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. Circulation. 2000;102:111136–41.Google Scholar
  3. 3.
    Forbess JM, Cook N, Serraf A, Burke RP, Mayer JE Jr, Jonas RA. An institutional experience with second-and third-stage palliative procedures for hypoplastic left heart syndrome: the impact of the bidirectional cavopulmonary shunt. J Am Coll Cardiol 1997;29:665–70PubMedCrossRefGoogle Scholar
  4. 4.
    Morita K, Kurosawa H, Mizuno A, Sakamoto Y, Tanaka K, Uno Y, Kawada N, Hanai M, Sugiyama K. The role of a staged approach for high-risk Fontan candidates. Jpn J Thorac Cardiovasc Surg 1999;47:478–88.PubMedCrossRefGoogle Scholar
  5. 5.
    Freedom RM, Nykanen D, Benson LN. The physiology of the bidirectional cavopulmonary connection. Jpn J Thorac Cardiovasc Surg 1999 Oct;47(10):478–88 Ann Thorac Surg 1998;66:664–7.Google Scholar
  6. 6.
    McElhinney DB, Reddy VM, Moore P, Hanley FL. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997;63:1676–84.PubMedCrossRefGoogle Scholar
  7. 7.
    Carlon CA, Mondini PG, de Marchi R. Su una nuova anastomosi vasale per la terapia chirurgica di alcuni visi cardiovasculari. [A new vascular anastomosis for surgical treatment of some cardiovascular anomalies]. Ital Chir 1950; 6:760–5.Google Scholar
  8. 8.
    Dogliotti, AM, Actis-Dato A, Venere G, Tarquini A. L’ intervento di anastomosi vena cava-arteria polmonare nella tetrade di Fallot e in altre cardiopatie. [Surgical creation of the vena cava-pulmonary artery anastomosis in Fallot tetralogy and other cardiac pathology]. Minerva Cardioangiol 1961; 9:577–93.PubMedGoogle Scholar
  9. 9.
    Azzolina G, Eufrate S, Pensa P. Tricuspid atresia: experience in surgical management with a modified cavopulmonary anastomosis. Thorax 1972; 27:111–5.PubMedCrossRefGoogle Scholar
  10. 10.
    Glenn WWL. Circulatory bypass of the right side of the heart. IV. Shunt between the superior vena cava and distal right pulmonary artery: report of clinical application. N Engl J Med 1958;259:117–20PubMedCrossRefGoogle Scholar
  11. 11.
    Konstantinov IE, Alexi-Meskishvit VV. Cavo-pulmonary shunt: from the first experiments to clinical practice. Ann Thorac Surg 1999; 68:1100–6.PubMedCrossRefGoogle Scholar
  12. 12.
    Santamore WP, Barnea 0, Riordan CJ, Ross MP, Austin EH. Theoretical optimization of pulmonary-to-systemic flow ratio after a bidirectional cavopulmonary anastomosis. Am J Physiol 1998 Feb; 274(2 Pt 2):H694–700.PubMedGoogle Scholar
  13. 13.
    Fogel et al. MRI estimation of ventricular geometry and performance JACC 1996; 28: 212–21.Google Scholar
  14. 14.
    Rychik J, Jacobs ML, Norwood WI Jr. Acute changes in left ventricular geometry after volume reduction operation. Ann Thorac Surg 1995 Nov; 60(5):1267–73;PubMedCrossRefGoogle Scholar
  15. 15.
    Penny DJ, Redington AN. Diastolic ventricular function after the Fontan operation. Am J Cardiol. 1992;69:974–5.PubMedCrossRefGoogle Scholar
  16. 16.
    Hjortdal VE, Stenbog EV, Ravn HB, Emmertsen K, Jensen KT, Pedersen EB, Olsen KH, Hansen OK, Sorensen KE. Neurohormonal activation late after cavopulmonary connection. Heart 2000;83:439–43.PubMedCrossRefGoogle Scholar
  17. 17.
    Pruckmayer M, Zacherl S, Salzer-Muhar U, Schlemmer M, Leitha T. Scintigraphic assessment of pulmonary and whole-body blood flow patterns after surgical intervention in congenital heart disease. J Nucl Med 1999 Sep;40(9):1477–83.PubMedGoogle Scholar
  18. 18.
    Salzer-Muhar U, Marx M, Ties M, Proll E, Wimmer M. Doppler flow profiles in the right and left pulmonary artery in children with congenital heart disease and a bidirectional cavopulmonary shunt. Pediatr Cardiol 1994 Nov-Dec;15(6):302–7.Google Scholar
  19. 19.
    Mahle WT, Wernovsky G, Bridges ND, Linton AB, Paridon SM. Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology. J Am Coll Cardiol 1999 Nov 1;34(5):1637–43.PubMedCrossRefGoogle Scholar
  20. 20.
    Bradley SM, Mosca RS, Hennein HA, Crowley DC, Kulik TJ, Bove EL. Bidirectional superior cavopulmonary connection in young infants. 98: Circulation 1996 Nov 1;94(9 Suppl):II5–11.Google Scholar
  21. 21.
    Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham FIN Jr. Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 1985;90:391–8.PubMedGoogle Scholar
  22. 22.
    Jacobs ML, Rychik J, Rome JJ, Apostolopoulou S, Pizarro C, Murphy JD, Norwood WI Jr. Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg 1996 Aug 62(2):456–61;CrossRefGoogle Scholar
  23. 23.
    Cohen MI, Bridges ND, Gaynor JW, Hoffman TM, Wernovsky G, Vetter VL, Spray TL, and Rhodes LA Modifications to the cavopulmonary anatomosis do not eliminate early sinus node dysunction.“ J Thorac Cardiovasc Surg 2000: In press.Google Scholar
  24. 24.
    Tweddell JS, Berger S, Frommelt PC, Pelech AN, Lewis DA, Fedderly RT, Frommelt MA, McManus TS, Mussatto KA, Kessel MW, Litwin SB. Aprotinin improves outcome of single-ventricle palliation. Ann Thorac Surg. 1996 62:1329–35.PubMedCrossRefGoogle Scholar
  25. 25.
    Koutlas TC, Gaynor JW, Nicolson SC, Steven JM, Wernovsky G, Spray TL. Modified ultrafiltration reduces postoperative morbidity after cavopulmonary connection. Ann Thorac Surg 1997;64:1:37–42.PubMedCrossRefGoogle Scholar
  26. 26.
    Norwood WI, Jacobs ML. Fontan’s procedure in two stages. Am J Surg 1993 Nov; 166(5):548–51.PubMedCrossRefGoogle Scholar
  27. 27.
    Bove EL. Surgical treatment for hypoplastic left heart syndrome. Jpn JThorac Cardiovasc Surg 1999 Feb;47(2):47–56.CrossRefGoogle Scholar
  28. 28.
    Spray TL. Fenestrated Fontan for hypoplastic left heart syndrome. Operative techniques in cardiac and thoracic surgery 1997;2:236–52.CrossRefGoogle Scholar
  29. 29.
    Reyes A 2nd, Bove EL, Mosca RS, Kulik TJ, Ludomirsky A. Tricuspid valve repair in children with hypoplastic left heart syndrome during staged surgical reconstruction. Circulation. 1997 Nov 4;96(9 Suppl):II-341–3; discussion 11–344-5.Google Scholar
  30. 30.
    Bradley SM Bradley SM, Simsic JM, Mulvihill DM. Hyperventilation impairs oxygenation after bidirectional superior cavopulmonary connection. Circulation 1998 Nov10;98(19 Suppl):11372–6; discussion 11376–7.Google Scholar
  31. 31.
    Yahagi N, Kumon K, Tanigami H, Watanabe Y, Haruna M, Hayashi H, Imanaka H,Takeuchi M, Takamoto S. Cardiac surgery and inhaled nitric oxide: indication and follow-up (2–4 years). Artif Organs 1998 Oct;22(10):886–91.PubMedCrossRefGoogle Scholar
  32. 32.
    Gamillscheg A, Zobel G, Urlesberger B, Berger J, Dacar D, Stein JI, Rigler B, Metzler H, Beitzke A. Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. J Thorac Cardiovasc Surg 1997 Mar;113(3):435–42.PubMedCrossRefGoogle Scholar
  33. 33.
    Chang RK, Alejos JC, Atkinson D, Jensen R, Drant S, Galindo A, Laks H. Bubble contrast echocardiography in detecting pulmonary arteriovenous Shunting in children with univentricular heart after cavopulmonary anastomosis. J Am Coll Cardiol 1999 Jun;33(7):2052–8.CrossRefGoogle Scholar
  34. 34.
    Vettukattil JJ, Slavik Z, Lamb RK, Monro JL, Keeton BR, Tsang VT, Aldous AJ, Zivanovic A, Johns S, Lewington V, Salmon AP. Intrapulmonary arteriovenous shunting may be a universal phenomenon in patients with the superior cavopulmonary anastomosis: a radionuclide study. Heart 2000 Apr;83(4):425–8PubMedCrossRefGoogle Scholar
  35. 35.
    Kobayashi J, Matsuda H, Nakano S, Shimazaki Y, Ikawa S, Mitsuno M, Takahashi Y, Kawashima Y, Arisawa J, Matsushita T. Hemodynamic effects of bidirectional cavopulmonary shunt with pulsatile pulmonary flow. Circulation 1991 Nov;84(5 Suppl):111219–25.Google Scholar
  36. 36.
    Knight WB, Mee RB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg. 1995 Apr;59(4):999–1001.PubMedCrossRefGoogle Scholar
  37. 37.
    Haller JA Jr. Bidirectional inferior vena cava-pulmonary artery shunt. J Thorac Cardiovasc Surg 1997 Dec;114(6):1123 J Thorac Cardiovasc Surg 1997 Dec;114(6):1123.Google Scholar
  38. 38.
    Triedman JK, Bridges ND, Mayer JE Jr, Lock JE. Prevalence and risk factors for aortopulmonary collateral vessels after Fontan and bidirectional Glenn procedures. J Am Coll Cardiol 1993 Jul;22(1):207–15.PubMedCrossRefGoogle Scholar
  39. 39.
    Elizari A, Somerville J. Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease. Cardiol Young 1999 May;9(3):257–65.PubMedGoogle Scholar
  40. 40.
    Gatzoulis MA, Shinebourne EA, Redington AN, Rigby ML, Ho SY, Shore DF. Increasing cyanosis early after cavopulmonary connection caused by abnormal systemic venous channels. Br Heart J 1995 Feb;73(2):182–6.PubMedCrossRefGoogle Scholar
  41. 41.
    Filippini LH, Ovaert C, Nykanen DG, Freedom RM. Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections. Heart 1998 May;79(5):509–12.Google Scholar
  42. 42.
    Cochrane AD, Brizard CP, Penny DJ, Johansson S, Comas JV, Mahn T, Karl TR. Management of the univentricular connection: are we improving? Eur J Cardiothorac Surg 1997 Jul;12(1):107–15.PubMedCrossRefGoogle Scholar
  43. 43.
    Lamberti JJ, Mainwaring RD, Spicer RL, Uzark KC, Moore JW. Factors influencing perioperative morbidity during palliation of the univentricular heart. Ann Thorac Surg 1995 Dec;60(6 Suppl):S550–3.PubMedCrossRefGoogle Scholar
  44. 44.
    Reddy VM, McElhinney DB, Moore P, Petrossian E, Hanley FL. Pulmonary artery growth after bidirectional cavopulmonary shunt: is there a cause for concern? J Thorac Cardiovasc Surg 1996 Nov; 112(5): 1180–90; discussion 1190–2.PubMedCrossRefGoogle Scholar
  45. 45.
    Mainwaring RD, Lamberti JJ, Uzark K. The bidirectional Glenn procedure: palliation of the univentricular heart. Adv Card Surg 1994;5:115–40.PubMedGoogle Scholar
  46. 46.
    Mendelsohn AM, Bove EL, Lupinetti FM, Crowley DC, Lloyd TR, Beekman RH 3rd. Central pulmonary artery growth patterns after the bidirectional Glenn procedure. J Thorac Cardiovasc Surg 1994 May;107(5):1284–90.PubMedGoogle Scholar
  47. 47.
    Uemura H, Yagihara T, Kawashima Y, Okada K, Kamiya T, Anderson RH. Use of the bidirectional Glenn procedure in the presence of forward flow from the ventricles to the pulmonary arteries. Circulation 1995 Nov 1;92(9 Suppl):11228–32.CrossRefGoogle Scholar
  48. 48.
    Miyaji K, Shimada M, Sekiguchi A, Ishizawa A, Isoda T. Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan. Ann Thorac Surg 1996 Mar; 61(3):845–50.PubMedCrossRefGoogle Scholar
  49. 49.
    Penny DJ, Pawade A, Wilkinson JL, Karl TR. Pulmonary artery size after bidirectional cavopulmonary connection. J Card Surg. 1995 Jan;10(1):21–6.PubMedCrossRefGoogle Scholar
  50. 50.
    Magee A, Sim E, Benson LN, Williams WG, Trusler GA, Freedom RM. Augmentation of pulmonary blood flow with an axillary arteriovenous fistula after a cavopulmonary shunt. J Thorac Cardiovasc Surg 1996 Jan;111(1):176–80M117.PubMedCrossRefGoogle Scholar
  51. 51.
    Frommelt MA, Frommelt PC, Berger S, Pelech AN, Lewis DA, Tweddell JS, Litwin SB. Does an additional source of pulmonary blood flow alter outcome after a bidirectional cavopulmonary shunt? Circulation 1995 Nov 1;92(9 Suppl):11240–4.CrossRefGoogle Scholar
  52. 52.
    Mainwaring RD, Lamberti JJ, Uzark K, Spicer RL, Cocalis MW, Moore JW. Effect of accessory pulmonary blood flow on survival after the bidirectional Glenn procedure. Circulation 1999 Nov 9;100(19 Suppl):II151–6.CrossRefGoogle Scholar
  53. 53.
    Mainwaring RD, Lamberti JJ, Uzark K, Spicer RL. Bidirectional Glenn. Is accessory pulmonary blood flow good or bad? Circulation 1995; 92(9 Suppl):11294–7.Google Scholar
  54. 54.
    Borini I, Marasini M, Dalmonte P, Bava GL, Lerzo F, Cervo G, Moretti R, Bertolini A, Zannini L. Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: clinical experience in 21 cases. Cardiovasc Surg 1997 Dec;5(6):588–92.PubMedCrossRefGoogle Scholar
  55. 55.
    Knott-Craig CJ, Fryar-Dragg T, Overholt ED, Razook JD, Ward KE, Elkins RC. Modified hemi-Fontan operation: an alternative definitive palliation forhigh-risk patients. Ann Thorac Surg 1995 Dec;60(6 Suppl):5554–7.Google Scholar
  56. 56.
    Bonnet D, Acar P, Aggoun Y, Bonhoeffer P, Villain E, Sidi D, Kachaner JArch Can partial cavo-pulmonary connection be considered an alternative to the Fontan procedure? Mal Coeur Vaiss 1998 May;91(5):569–73Google Scholar

Copyright information

© Springer Science+Business Media New York 2003

Authors and Affiliations

  • Tom R. Karl
    • 1
  1. 1.Division of Cardiothoracic SurgeryUniversity of California-San Francisco Children’s HospitalUSA

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