Skip to main content
SpringerLink
Log in

Orthotopic Heart Transplantation as an Alternative Treatment Strategy for Hypoplastic Left Heart Syndrome

  • Reference work entry
  • First Online:
Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

  • 418 Accesses

Abstract

Heart transplantation as a primary therapy for infants with hypoplastic left heart syndrome (HLHS) has decreased in frequency over the last two decades as outcomes of surgical palliation have improved. However, waitlist mortality has decreased, and transplantation remains a viable option for some infants with HLHS, especially those at highest risk of poor outcomes following surgical palliation. Pre-transplant management is complicated by the need to maintain ductal patency while balancing systemic and pulmonary blood flow, which leads to prolonged exposure of the pulmonary vasculature to high pulmonary artery pressure and resistance. Primary graft failure and pulmonary hypertension, with or without isolated right ventricular failure, are most likely to complicate the postoperative period. Given small patient size and a higher risk of complications with frequent biopsies, rejection surveillance in this population is usually noninvasive. Immunosuppression can be minimized due to a more plastic immune system, with many infant heart transplant recipients on a single-drug maintenance immunosuppression regimen for the long term. During the most recent era, overall survival from the time of listing is 60 % at 5 years. Infants that make it to transplant have the best graft survival of all age groups, with a median graft survival of 18 years.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 1,299.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 1,799.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Backer CL, Idriss FS, Zales VR, Mavroudis C (1990) Cardiac transplantation for hypoplastic left heart syndrome: a modified technique [see comment]. Ann Thorac Surg 50:894–898

    Article  CAS  PubMed  Google Scholar 

  2. Chiavarelli M, Gundry SR, Razzouk AJ, Bailey LL (1993) Cardiac transplantation for infants with hypoplastic left-heart syndrome. JAMA 270:2944–2947

    Article  CAS  PubMed  Google Scholar 

  3. Mayer JE Jr (1990) Cardiac transplantation for neonates with hypoplastic left heart syndrome. Ann Thorac Surg 50:864–865

    Article  PubMed  Google Scholar 

  4. Reyes A 2nd, Bove EL, Mosca RS, Kulik TJ, Ludomirsky A (1997) Tricuspid valve repair in children with hypoplastic left heart syndrome during staged surgical reconstruction. Circulation 96:II-341–II-343, discussion II-4–II-5

    Google Scholar 

  5. Norwood WI, Lang P, Hansen DD (1983) Physiologic repair of aortic atresia–hypoplastic left heart syndrome. N Engl J Med 308:23–26

    Article  CAS  PubMed  Google Scholar 

  6. Chrisant MR, Naftel DC, Drummond-Webb J et al (2005) Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Heart Lung Transplant 24:576–582

    Article  CAS  PubMed  Google Scholar 

  7. Bove EL, Lloyd TR (1996) Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. Ann Surg 224:387–394, discussion 94–95

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Sano S, Ishino K, Kado H et al (2004) Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: a multi-institutional study. Ann Thorac Surg 78:1951–1957, discussion 7–8

    Article  PubMed  Google Scholar 

  9. Wernovsky G (2008) The paradigm shift toward surgical intervention for neonates with hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 162:849–854

    Article  PubMed  Google Scholar 

  10. Khairy P, Fernandes SM, Mayer JE Jr et al (2008) Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 117:85–92

    Article  PubMed  Google Scholar 

  11. Torres A Jr (2010) To (enterally) feed or not to feed (the infant with hypoplastic left heart syndrome) is no longer the question. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensiv Crit Care Soc 11:431–432

    Google Scholar 

  12. Jenkins PC, Flanagan MF, Jenkins KJ et al (2000) Survival analysis and risk factors for mortality in transplantation and staged surgery for hypoplastic left heart syndrome. J Am Coll Cardiol 36:1178–1185

    Article  CAS  PubMed  Google Scholar 

  13. Jenkins PC, Flanagan MF, Sargent JD et al (2001) A comparison of treatment strategies for hypoplastic left heart syndrome using decision analysis. J Am Coll Cardiol 38:1181–1187

    Article  CAS  PubMed  Google Scholar 

  14. Altmann K, Printz BF, Solowiejczky DE, Gersony WM, Quaegebeur J, Apfel HD (2000) Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome. Am J Cardiol 86:964–968

    Article  CAS  PubMed  Google Scholar 

  15. Hehir DA, Dominguez TE, Ballweg JA et al (2008) Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants. J Thorac Cardiovasc Surg 136:94.e3–99.e3

    Article  Google Scholar 

  16. Guleserian KJ, Armsby LB, Thiagarajan RR, del Nido PJ, Mayer JE Jr (2006) Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg 81:2250–2257, discussion 8

    Article  PubMed  Google Scholar 

  17. Tweddell JS, Sleeper LA, Ohye RG, et al (2012) Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg

    Google Scholar 

  18. Guleserian KJ, Schechtman KB, Zheng J et al (2011) Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease: a multi-institutional study. J Heart Lung Transplant 30:1023–1032

    Article  PubMed  Google Scholar 

  19. Ohye RG, Sleeper LA, Mahony L et al (2010) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362:1980–1992

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Kon AA (2009) Ethics of cardiac transplantation in hypoplastic left heart syndrome. Pediatr Cardiol 30:725–728

    Article  PubMed  PubMed Central  Google Scholar 

  21. Gaynor JW, Mahle WT, Cohen MI et al (2002) Risk factors for mortality after the Norwood procedure. Eur J Cardiothorac Surg 22:82–89

    Article  PubMed  Google Scholar 

  22. Ghanayem NS, Hoffman GM, Mussatto KA et al (2010) Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 140:857–863

    Article  PubMed  Google Scholar 

  23. Patel A, Hickey E, Mavroudis C et al (2010) Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome. Ann Thorac Surg 89:1805–1813, discussion 13–14

    Article  PubMed  Google Scholar 

  24. Vida VL, Bacha EA, Larrazabal A et al (2008) Surgical outcome for patients with the mitral stenosis-aortic atresia variant of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 135:339–346

    Article  PubMed  Google Scholar 

  25. Glatz JA, Fedderly RT, Ghanayem NS, Tweddell JS (2008) Impact of mitral stenosis and aortic atresia on survival in hypoplastic left heart syndrome. Ann Thorac Surg 85:2057–2062

    Article  PubMed  Google Scholar 

  26. Ashburn DA, McCrindle BW, Tchervenkov CI et al (2003) Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 125:1070–1082

    Article  PubMed  Google Scholar 

  27. Singh TP, Almond CS, Piercey G, Gauvreau K (2011) Trends in wait-list mortality in children listed for heart transplantation in the United States: era effect across racial/ethnic groups. Am J Transplant Off J Am Soc Transplant Am SocTransplant Surg 11:2692–2699

    Article  CAS  Google Scholar 

  28. West LJ, Pollock-Barziv SM, Dipchand AI et al (2001) ABO-incompatible heart transplantation in infants. N Engl J Med 344:793–800

    Article  CAS  PubMed  Google Scholar 

  29. Almond CS, Gauvreau K, Thiagarajan RR et al (2010) Impact of ABO-incompatible listing on wait-list outcomes among infants listed for heart transplantation in the United States. Circulation 121:1926–1933

    Article  PubMed  PubMed Central  Google Scholar 

  30. Patel ND, Weiss ES, Scheel J, Cameron DE, Vricella LA (2008) ABO-incompatible heart transplantation in infants: analysis of the united network for organ sharing database. J Heart Lung Transplant Off Publ Int Soc Heart Transplant 27:1085–1089

    Article  Google Scholar 

  31. Henderson HT, Canter CE, Mahle WT et al (2012) ABO-incompatible heart transplantation: analysis of the Pediatric Heart Transplant Study (PHTS) database. J Heart Lung Transplant Off Publ Int Soc Heart Transplant 31:173–179

    Article  Google Scholar 

  32. Dipchand AI, PollockBarZiv SM, Manlhiot C, West LJ, VanderVliet M, McCrindle BW (2010) Equivalent outcomes for pediatric heart transplantation recipients: ABO-blood group incompatible versus ABO-compatible. Am J Transplant Off J Am Soc Transplant Am Soc Transplant Surg 10:389–397

    Article  CAS  Google Scholar 

  33. Urschel S, Campbell PM, Meyer SR et al (2010) Absence of donor-specific anti-HLA antibodies after ABO-incompatible heart transplantation in infancy: altered immunity or age? Am J Transplant Off J Am Soc Transplant Am Soc Transplant Surg 10:149–156

    Article  CAS  Google Scholar 

  34. Urschel S, Dalla Pozza R, Loeff M et al (2009) Successful ABO-incompatible heart transplantation in a child despite blood-group sensitization after ventricular assist device support. Pediatr Transplant 13:773–776

    Article  CAS  PubMed  Google Scholar 

  35. West LJ (2006) B-cell tolerance following ABO-incompatible infant heart transplantation. Transplantation 81:301–307

    Article  PubMed  Google Scholar 

  36. Conway J, Chrisant MR, West LJ, et al Abstract 11335: Outcome of fetal listing for heart transplantation. Circulation 124:A11335

    Google Scholar 

  37. Boucek MM, Mashburn C, Dunn SM et al (2008) Pediatric heart transplantation after declaration of cardiocirculatory death. N Engl J Med 359:709–714

    Article  CAS  PubMed  Google Scholar 

  38. Mathur M, Castleberry D, Job L (2011) Identifying potential heart donors among newborns undergoing circulatory determination of death. J Heart Lung Transplant 30:389–394

    Article  PubMed  Google Scholar 

  39. Bourke KD, Sondheimer HM, Ivy DD et al (2003) Improved pretransplant management of infants with hypoplastic left heart syndrome enables discharge to home while waiting for transplantation. Pediatr Cardiol 24:538–543

    Article  CAS  PubMed  Google Scholar 

  40. Auerbach SR, Richmond ME, Chen JM et al (2012) Multiple risk factors before pediatric cardiac transplantation are associated with increased graft loss. Pediatr Cardiol 33:49–54

    Article  PubMed  Google Scholar 

  41. Davies RR, Russo MJ, Mital S et al (2008) Predicting survival among high-risk pediatric cardiac transplant recipients: an analysis of the United Network for Organ Sharing database. J Thorac Cardiovasc Surg 135:147.e2–155.e2

    Google Scholar 

  42. Krushansky E, Burbano N, Morell V, et al (2011) Preoperative management in patients with single-ventricle physiology. Congenital Heart Disease 2011:no-no

    Google Scholar 

  43. Tabbutt S, Ramamoorthy C, Montenegro LM et al (2001) Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 104:I-159–I-164

    Article  CAS  Google Scholar 

  44. Pietra B, Ivy DD, Sondheimer HM et al (2001) Comparison of pretransplant transfusion with or without cyclosporin inhibition on post transplant outcome. J Heart Lung Transplant 20:222–223

    Article  CAS  PubMed  Google Scholar 

  45. Mitchell MB, Campbell DN, Boucek MM et al (2003) Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome. Eur J Cardiothorac Surg 23:735–742

    Article  PubMed  Google Scholar 

  46. Miyamoto S, Pietra B, Chan K-C et al (2009) Long-term outcome of palliation with internal pulmonary artery bands after primary heart transplantation for hypoplastic left heart syndrome. Pediatr Cardiol 30:419–425

    Article  PubMed  PubMed Central  Google Scholar 

  47. Boucek MM, Mashburn C, Chan KC (2005) Catheter-based interventional palliation for hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu:72–77

    Google Scholar 

  48. Boucek MM, Mashburn C, Kunz E, Chan KC (2005) Ductal anatomy: a determinant of successful stenting in hypoplastic left heart syndrome. Pediatr Cardiol 26:200–205

    Article  CAS  PubMed  Google Scholar 

  49. Johnson JN, Ansong AK, Li JS et al (2011) Celiac artery flow pattern in infants with single right ventricle following the Norwood procedure with a modified Blalock-Taussig or right ventricle to pulmonary artery shunt. Pediatr Cardiol 32:479–486

    Article  PubMed  PubMed Central  Google Scholar 

  50. McElhinney DB, Hedrick HL, Bush DM et al (2000) Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics 106:1080–1087

    Article  CAS  PubMed  Google Scholar 

  51. del Castillo SL, McCulley ME, Khemani RG et al (2010) Reducing the incidence of necrotizing enterocolitis in neonates with hypoplastic left heart syndrome with the introduction of an enteral feed protocol. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensiv Crit Care Soc 11:373–377

    Google Scholar 

  52. Braudis NJ, Curley MA, Beaupre K et al (2009) Enteral feeding algorithm for infants with hypoplastic left heart syndrome poststage I palliation. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensiv Crit Care Soc 10:460–466

    Google Scholar 

  53. Shirali GS, Cephus CE, Kuhn MA et al (1998) Posttransplant recoarctation of the aorta: a twelve year experience. J Am Coll Cardiol 32:509–514

    Article  CAS  PubMed  Google Scholar 

  54. del Nido PJ, Bailey LL, Kirklin JK (2007) Pediatric heart transplantation. Elsevier, Philadelphia, pp 83–102

    Google Scholar 

  55. Bauer J, Dapper F, Demirakca S, Knothe C, Thul J, Hagel KJ (1997) Perioperative management of pulmonary hypertension after heart transplantation in childhood. J Heart Lung Transplant 16:1238–1247

    CAS  PubMed  Google Scholar 

  56. Kieler-Jensen N, Lundin S, Ricksten SE (1995) Vasodilator therapy after heart transplantation: effects of inhaled nitric oxide and intravenous prostacyclin, prostaglandin E1, and sodium nitroprusside. J Heart Lung Transplant Off Publ Int Soc Heart Transplant 14:436–443

    CAS  Google Scholar 

  57. Backer CL (2009) Outcome of extracorporeal membrane oxygenation for early primary graft failure after pediatric heart transplantation. J Am Coll Cardiol 54:738–739

    Article  PubMed  Google Scholar 

  58. Mitchell MB, Campbell DN, Bielefeld MR, Doremus T (2000) Utility of extracorporeal membrane oxygenation for early graft failure following heart transplantation in infancy. J Heart Lung Transplant 19:834–839

    Article  CAS  PubMed  Google Scholar 

  59. Kirk R, Edwards LB, Kucheryavaya AY et al (2011) The registry of the international society for heart and lung transplantation: fourteenth pediatric heart transplantation report—2011. J Heart Lung Transplant 30:1095–1103

    Article  PubMed  Google Scholar 

  60. Karamichalis JM, Miyamoto SD, Campbell DN, et al (2010) Pediatric cardiac retransplant: differing patterns of primary graft failure by age at first transplant. J Thorac Cardiovasc Surg

    Google Scholar 

  61. Boucek MM, Mathis CM, Boucek RJ Jr et al (1994) Prospective evaluation of echocardiography for primary rejection surveillance after infant heart transplantation: comparison with endomyocardial biopsy. J Heart Lung Transplant 13:66–73

    CAS  PubMed  Google Scholar 

  62. Harrison AM, Davis S, Reid JR et al (2005) Neonates with hypoplastic left heart syndrome have ultrasound evidence of abnormal superior mesenteric artery perfusion before and after modified Norwood procedure. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensiv Crit Care Soc 6:445–447

    Google Scholar 

  63. Tantengco MV, Dodd D, Frist WH, Boucek MM, Boucek RJ (1993) Echocardiographic abnormalities with acute cardiac allograft rejection in children: correlation with endomyocardial biopsy. J Heart Lung Transplant 12:S203–S210

    CAS  PubMed  Google Scholar 

  64. Razzouk AJ, Chinnock RE, Gundry SR et al (1996) Transplantation as a primary treatment for hypoplastic left heart syndrome: intermediate-term results. Ann Thorac Surg 62:1–7, discussion 8

    Article  CAS  PubMed  Google Scholar 

  65. Dionigi B, Razzouk AJ, Hasaniya NW, Chinnock RE, Bailey LL (2008) Late outcomes of pediatric heart transplantation are independent of pre-transplant diagnosis and prior cardiac surgical intervention. J Heart Lung Transplant 27:1090–1095

    Article  PubMed  Google Scholar 

  66. Kuhn MA, Jutzy KR, Deming DD et al (2000) The medium-term findings in coronary arteries by intravascular ultrasound in infants and children after heart transplantation. J Am Coll Cardiol 36:250–254

    Article  CAS  PubMed  Google Scholar 

  67. Nicolas RT, Kort HW, Balzer DT et al (2006) Surveillance for transplant coronary artery disease in infant, child and adolescent heart transplant recipients: an intravascular ultrasound study. J Heart Lung Transplant 25:921–927

    Article  PubMed  Google Scholar 

  68. Hussain T, Burch M, Fenton MJ et al (2007) Positive pretransplantation cytomegalovirus serology is a risk factor for cardiac allograft vasculopathy in children. Circulation 115:1798–1805

    Article  PubMed  Google Scholar 

  69. Ibrahim JE, Sweet SC, Flippin M et al (2002) Rejection is reduced in thoracic organ recipients when transplanted in the first year of life. J Heart Lung Transplant 21:311–318

    Article  PubMed  Google Scholar 

  70. Ikle L, Hale K, Fashaw L et al (2003) Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation. J Pediatr 142(1):20–25

    Article  PubMed  Google Scholar 

  71. Newburger JW, Sleeper LA, Bellinger DC et al (2012) Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial. Circulation 125(17):2081–2091

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Cardiology, University of Colorado Health Science Center, The Heart Institute, Children’s Hospital Colorado, 13123 East 16th Avenue, Aurora, CO, 80045, USA

    Scott R. Auerbach

  2. Pediatric Cardiac Surgery, Department of Surgery, University of Colorado Denver Health Science Center, 1056 E 19th Ave B200, Aurora, CO, 80045, USA

    David N. Campbell

  3. The Heart Institute, Department of Pediatrics, University of Colorado Denver, Children’s Hospital Colorado, School of Medicine, 13123 East 16th Avenue, Aurora, CO, 80045, USA

    Shelley D. Miyamoto

Authors
  1. Scott R. Auerbach
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. David N. Campbell
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shelley D. Miyamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Scott R. Auerbach .

Editor information

Editors and Affiliations

  1. Dept. of Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Eduardo M. Da Cruz

  2. Dept. Pediatric Cardiology, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    Dunbar Ivy

  3. Pediatric Cardiac Surgery, Children's Hospital Colorado, Univ. of Colorado, Denver, Aurora, Colorado, USA

    James Jaggers

Electronic supplementary material

Video 104.1

AVI file: 18438 KB

Video 104.2

AVI file: 698 KB

Video 104.3

AVI file: 837 KB

Video 104.4

MPG file: 572 KB

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this entry

Cite this entry

Auerbach, S.R., Campbell, D.N., Miyamoto, S.D. (2014). Orthotopic Heart Transplantation as an Alternative Treatment Strategy for Hypoplastic Left Heart Syndrome. In: Da Cruz, E., Ivy, D., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4619-3_40

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-4619-3_40

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-4618-6

  • Online ISBN: 978-1-4471-4619-3

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation