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Hypoplastic Left Heart Syndrome

  • Viktor Hraška
  • Peter Murín

Abstract

Hypoplastic left heart syndrome is characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and the aortic arch. This chapter elaborates:

1. Anatomy

2. Indication for surgery

3. Approach and cardiopulmonary bypass strategy

4. Norwood stage I palliation with double aortic cannulation and whole body perfusion (video)

5. Double barrel Damus-Kaye-Stansel anastomosis with reconstruction of the aortic arch and modified BT shunt (video)

6. Norwood stage I. palliation after bilateral pulmonary artery banding - staged approach (video)

7. Biventricular repair – the first stage: Norwood stage I. palliation with Sano shunt and enlargement of the ventricular septal defect (video)

8. Biventricular repair – the second stage: Intraventricular rerouting of the left ventricle to the systemic outflow and of the right ventricle to a pulmonary artery conduit (video)

Keywords

Aortic Arch Patent Ductus Arteriosus Ventricular Septal Defect Hypoplastic Left Heart Syndrome Biventricular Repair 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Supplementary material

Clip 1: Preoperative findings.

Clip 2: Orientation and anatomy.

Clip 3: Proximal shunt anastomosis.

Clip 4: Cardiopulmonary bypass initiation and mobilisation of the aortic arch.

Clip 5: Transection of the pulmonary trunk and reconstruction of the pulmonary artery bifurcation.

Clip 6: Incision of the aortic arch and side-to-side anastomosis of the aortic root with the pulmonary artery trunk.

Clip 7: Reconstruction of the aortic arch and the ascending aorta.

Clip 8: Distal shunt anastomosis.

Clip 9: Final result.

Clip 10: Postoperative findings.

Fullversion

Clip 1: Preoperative findings.

Clip 2: Orientation and anatomy.

Clip 3: Proximal shunt anastomosis. Insertion of the aortic cannula to the shunt.

Clip 4: Transection of the patent ductus arteriosus.

Clip 5: Mobilisation of the aortic arch.

Clip 6: Trimming of the homograft patch. Reconstruction of the pulmonary artery bifurcation.

Clip 7: Initiation of antegrade cerebral perfusion.

Clip 8: Incision of the aortic arch. Resection of the coarctation.

Clip 9: Partial end-to-end anastomosis of the descending aorta.

Clip 10: “Double barrel” side-to-side anastomosis between the aortic and pulmonary trunks.

Clip 11: Reconstruction of the aortic arch and ascending aorta.

Clip 12: Distal shunt anastomosis.

Clip 13: Final result.

Clip 14: Postoperative findings.

Fullversion

Clip 1: Preoperative findings.

Clip 2: Orientation and anatomy.

Clip 3: Proximal shunt anastomosis.

Clip 4: Dissection of the pulmonary artery branches and debanding.

Clip 5: Mobilization of the aortic arch.

Clip 6: Transection and closure of the pulmonary artery bifurcation.

Clip 7: Antegrade cardioplegia delivery and initiation of antegrade cerebral perfusion.

Clip 8: Incision of the aortic arch. Resection of the coarctation.

Clip 9: Side-to-side anastomosis of the pulmonary trunk and the aortic root.

Clip 10: Reconstruction of the aortic arch and ascending aorta.

Clip 11: Distal shunt anastomosis.

Clip 12: Final result.

Clip 13: Postoperative findings.

Fullversion

Clip 1: Preoperative findings.

Clip 2: Orientation and anatomy.

Clip 3: Transection of the pulmonary artery trunk.

Clip 4: Distal shunt anastomosis.

Clip 5: Right ventriculotomy.

Clip 6: Incision of the aorta and removal of all ductal tissue.

Clip 7: Side-to-side anastomosis between the aortic root and the pulmonary artery trunk.

Clip 8: Reconstruction of the aortic arch and ascending aorta.

Clip 9: Enlargement of the ventricular septal defect. Proximal shunt anastomosis.

Clip 10: Final result.

Clip 11: Postoperative findings.

Fullversion

Clip 1: Preoperative findings.

Clip 2: Orientation and anatomy.

Clip 3: Resection of the Sano shunt.

Clip 4: Intracardiac anatomy and transatrial enlargement of the ventricular septal defect.

Clip 5: Resection of the shunt and transventricular enlargement of the ventricular septal defect.

Clip 6: Creation of the intraventricular tunnel.

Clip 7: Distal conduit anastomosis.

Clip 8: Proximal conduit anastomosis.

Clip 9: Final result.

Clip 10: Postoperative findings.

Fullversion

Recommended References

  1. Asou T (2002) Arch reconstruction without circulatory arrest: Historical perspectives and initial clinical results. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5:89–94.Google Scholar
  2. Asou T, Kado H, Imoto Y et al (1996) Selective cerebral perfusion technique during aortic arch repair in neonates. Ann Thorac Surg 61:1546–1548CrossRefPubMedGoogle Scholar
  3. Azakie T, Merklinger SL, McCrindle BW et al (2001) Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience. Ann Thorac Surg 72:1349–1353CrossRefPubMedGoogle Scholar
  4. Banka P, Porras D, Mayer JM et al (2014) Single ventricle (non HLHS). In: DaCruz EM, Ivy D, Jagger J (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive care. Springer Verlag, London, pp 1861–1883CrossRefGoogle Scholar
  5. Chock VY, Amir G, Davis CR et al (2006) Antegrade cerebral perfusion reduces apoptotic neuronal injury in a neonatal piglet model of cardiopulmonary bypass. J ThoracGoogle Scholar
  6. Cardiovasc Surg 131:659–665.Google Scholar
  7. Daebritz SH, Nollert GD, Zurakowski D at al (2000) Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations. J Thorac Cardiovasc Surg 119:358–367.Google Scholar
  8. Driscoll DJ (2007) Long-term results of the Fontan operation. Pediatr Cardiol 28:438–442.Google Scholar
  9. Erez E, Tam VK, Kanter KR et al (2001) Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction. Ann Thorac Surg 71:1974–1977CrossRefPubMedGoogle Scholar
  10. Fischbach J, Sinzobahamvya N, Haun C et al (2013) Interventions after Norwood operation: comparison of Sano and modified Blalock-Taussig shunt. Pediatr Cardiol 34:112–118.Google Scholar
  11. Fraser CD, Andropoulos DB (2008) Principles of antegrade cerebral perfusion during arch reconstruction in newborn/infants. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 11:61–68.Google Scholar
  12. Galantowicz M, Cheatham JP, Phillips A et al (2008) Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg 85:2063–2070CrossRefPubMedGoogle Scholar
  13. Ghanayem NS, Tweddell JS, Hoffman GM et al (2006) Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring, and the results of early cavopulmonary anastomosis. Cardiol Young 1(16 Suppl):61–66CrossRefGoogle Scholar
  14. Goldberg CS, Bove EL, Devaney EJ et al (2007) A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: outcomes for infants with functional single ventricle. J Thorac Cardiovasc Surg 133:880–887.Google Scholar
  15. Graham EM, Atz AM, Bradley SM et al (2007) Does a ventriculotomy have deleterious effects following palliation in the Norwood procedure using a shunt placed from the right ventricle to the pulmonary arteries? Cardiol Young 17:145–150.Google Scholar
  16. Gruber PJ, Spray TL (2014) Single ventricle (non HLHS). In: DaCruz EM, Ivy D, Jagger J (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive care. Springer Verlag, London, pp 1787–1808CrossRefGoogle Scholar
  17. Hoffman GM, Ghanayem NS, Kampine JM et al (2000) Venous saturation and the anaerobicGoogle Scholar
  18. threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 70:1515–1520.Google Scholar
  19. Hraska V, Murin P (2012) Surgical Management of Congenital Heart Disease I: Complex Transposition of Great Arteries Right and Left Ventricular Outflow Tract Obstruction, Ebstein’s Anomaly. A Video Manual. Springer, HeidelbergCrossRefGoogle Scholar
  20. Hraska V, Nosal M, Sykora P et al (2000) Results of modified Norwood´s operation for hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 18:214–219.Google Scholar
  21. Honjo O, Benson LN, Mewhort HE et al (2009) Clinical outcomes, program evolution, and PAGoogle Scholar
  22. growth in single ventricle palliation using hybrid and Norwood palliative strategies. Ann Thorac Surg 87:1885–1892Google Scholar
  23. Imoto Y, Kado H, Shiokawa Y et al (1999) Norwood procedure without circulatory arrest. Ann Thorac Surg 68:559–561.Google Scholar
  24. Jacobs ML (1998) Hypoplastic left heart syndrome. In: Kaiser LR, Kron IL, Spray TL (eds) Mastery of cardiothoracic surgery. Lippincott-Raven Publishers, Philadelphia, pp 858–866Google Scholar
  25. Larrazabal LA, Selamet Tierney ES et al (2008) Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome. Ann Thorac Surg 86:869–874.Google Scholar
  26. de Leval MR, McKay R, Jones M et al (1981) Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-PA shunts. J Thorac Cardiovasc Surg 81:112–119.Google Scholar
  27. Mayer JE (1998) Tricuspid atresia/Single ventricle and the Fontan operation. In: Kaiser LR, Kron IL, Spray TL (eds) Mastery of cardiothoracic surgery. Lippincott-Raven Publishers, Philadelphia, pp 848–857Google Scholar
  28. Ohye RG, Sleeper LA, Mahony L et al (2012) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362:1980–1992CrossRefGoogle Scholar
  29. Photiadis J, Sinzobahamvya N, Haun C et al (2012) Does the shunt-type determine midterm outcome after Norwood operation? Eur J Cardiothorac Surg 42: 209–216.Google Scholar
  30. Photiadis J, Sinzobahamvya N, Hraska V et al (2012) Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature. Thorac Cardiovasc Surg 60:181–188.Google Scholar
  31. Rüffer A, Danch A, Gottschalk U et al (2009) The Norwood procedure – does the type of shunt determine outcome? Thorac Cardiovasc Surg. 57:270–275.Google Scholar
  32. Sadahiro M, Haneda K, Mohri H et al (1994) Experimental study of cerebral autoregulation during cardiopulmonary bypass with or without pulsatile perfusion. J Thorac Cardiovasc Surg 108:446–454.Google Scholar
  33. Schindler E, Photiadis J, Lagutka S et al (2010) Influence of Two pefusion Strategies onGoogle Scholar
  34. Oxygen Metabolism in Paediatric Cardiac Surgery. Evaluation of the High-Flow, Low ResistanceTechnique. Eur J Cardiothorac Surg. 37:651–657Google Scholar
  35. Simsic JM, Bradley SM, Stroud MR et al (2005) Risk factors for interstage death after the Norwood procedure. Pediatr Cardiol 26:400–403.Google Scholar
  36. Stasik CN, Gelehrter S, Goldberg CS et al (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 131:412–417.Google Scholar
  37. Stoica SC, Philips AB, Egan M et al (2009) The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome. Ann Thorac Surg 88:1939–1946CrossRefPubMedGoogle Scholar
  38. Tanaka J, Shiki K, Asou T, et al (1988) Cerebral autoregulation during deep hypothermic nonpulsatile cardiopulmonary bypass with selective cerebral perfusion in dogs. J Thorac Cardiovasc Surg 95:124–132.Google Scholar
  39. Tchervenkov CI,Jacobs ML,Tahta SA (2000) Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome. Annals 69:S170–179Google Scholar
  40. Tweddell JS, Hoffman GM, Mussatto KA et al (2002) Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 115(106):I82–I89Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Viktor Hraška
    • 1
  • Peter Murín
    • 1
  1. 1.Department of Cardiac SurgeryGerman Pediatric Heart Center, Sankt AugustinSankt AugustinGermany

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