eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure: Follow-up

Author: Richard G Ohye, MD, Director, Pediatric Cardiac Transplantation, Fellowship Program Director, Pediatric Cardiac Surgery, Assistant Professor, Department of Surgery, Section of Cardiac Surgery, University of Michigan Medical Center
Coauthor(s): Ralph S Mosca, MD, Director, Pediatric Cardiac Surgery, Associate Professor, Department of Surgery, New York Presbyterian Medical Center; Edward L Bove, MD, Associate Director, PICU, CS Mott Children's Hospital; Director, Department of Surgery, Section of Thoracic Surgery, Division of Pediatric Cardiovascular Surgery, Professor, University of Michigan Medical Center
Contributor Information and Disclosures

Updated: Jul 15, 2008

Outcome and Prognosis

First-stage palliation

Bove et al at the University of Michigan studied first-stage palliation of hypoplastic left heart syndrome (HLHS) from January 1990 to August 1995 in 158 patients.13 All patients had classic HLHS, defined as right ventricle–dependent circulation, in association with atresia or severe hypoplasia of the aortic valve. Patients were subdivided into a standard-risk (n=127) population and a high-risk (n=31) population. High-risk patients included those undergoing the Norwood procedure after age 1 month, patients with severe obstruction to pulmonary venous return, and patients with significant noncardiac congenital conditions (ie, prematurity, low birth weight, chromosomal anomalies).

Hospital survivors numbered 120 (76%). The hospital survival rate was significantly better in the 127 standard-risk patients (86%) than in the high-risk group (42%). The risk factor analysis failed to reveal any effect on outcome by the morphologic subgroup, ascending aorta size, shunt size, initial pH at hospital presentation, or duration of circulatory arrest.

Among 151 patients at The Children's Hospital of Philadelphia in a report by Norwood et al, 42 (28%) early deaths and 9 (5%) late deaths occurred.14 In a Children's Hospital Boston series reported by Jonas et al, 78 neonates underwent palliative reconstructive surgery from 1983-1991.15 Hospital deaths numbered 29 (37%). Analysis of deaths revealed a greater risk of hospital death for infants with aortic atresia and mitral atresia, especially those with ascending aortic dimensions of less than 2 mm. However, in the authors' experience, these conditions have not been associated with increased risk.

The results for the hospital survival for the Norwood procedure has continued to improve. In 2002, Tweddell and colleagues reported a 93% hospital survival in 81 patients undergoing a Norwood procedure for HLHS.16

Second-stage palliation

Hospital records of 114 patients undergoing the hemi-Fontan procedure for HLHS between August 1993 and April 1998 at the University of Michigan Medical Center were reviewed by Douglas et al.17 The overall hospital survival rate was 98% (112 patients). Sinus rhythm was present in 92% of patients. At the time of publication, 79 of the patients had undergone the completion Fontan procedure, with 74 survivors (94%). A similar study by Forbess et al from the Children's Hospital Boston also revealed that a cavopulmonary anastomosis performed as a second-stage procedure for HLHS reduced mortality and improved intermediate survival rates.18

Third-stage palliation

One hundred consecutive patients with classic HLHS underwent a Fontan procedure at the University of Michigan between February 1992 and April 1998.19 The survival rate in patients (n=52) undergoing surgery in the second half of the study and treated with a prior hemi-Fontan procedure at second-stage palliation was 98%. No deaths have occurred in patients undergoing the last 125 consecutive Fontan procedures for HLHS. Several other centers also have reported significant improvements in survival rates following the Fontan procedure in patients with HLHS.

Neurodevelopmental outcomes

As survivals have improved, other endpoints, such as patient neurodevelopmental outcome, have become of increasing interest to the healthcare provider caring for the patient with HLHS. Similar to any patient with cyanotic congenital heart disease, patients with HLHS are at risk for neurodevelopmental delay for multiple reasons. Cyanosis, congestive heart failure, and CNS abnormalities are associated with HLHS and can contribute to developmental delay. In addition, CPB and hypothermic circulatory arrest at the time of repair can cause neurologic injury.

In a recent study from the University of Michigan Medical Center, Goldberg and colleagues evaluated 51 patients with single ventricle physiology, 26 patients with HLHS, and 25 patients with other cardiac anomalies.20 The primary testing methods were the Wechsler Preschool and Primary Scales of Intelligence, revised for children aged 34-87 months, and the Wechsler Intelligence Scale, third edition, for children aged 72 months to 17 years. Additional tests included the Bayley Scales of Infant Development, the Vineland Adaptive Behavior Scales, and the Child Behavior Checklist.

Results indicated that children with HLHS scored statistically lower than children without HLHS with single ventricles. However, neither group scored significantly differently than population standards. As has been seen in children with congenital heart disease in general, patients in this study scored significantly better on tests of verbal intelligence than on tests of motor skills. Socioeconomic status, hypothermic circulatory arrest, and perioperative seizures were significant risk factors for impaired neurodevelopmental outcome. Duration of CPB, cardiac arrest requiring resuscitation, and clinical shock or pH less than 7.1 did not correlate with a poor neurodevelopmental result.

Future and Controversies

Several groups have begun to use the techniques of regional cerebral perfusion for aortic arch reconstruction in lieu of deep hypothermic circulatory arrest. For this technique, the proximal anastomosis of the modified Blalock-Taussig shunt is performed prior to arresting the heart. Then, the arterial cannula can be placed into the shunt, and perfusion is administered to the innominate artery. Whether these techniques will improve perioperative survival rates or long-term neurodevelopmental outcomes has yet to be determined. Several recent abstracts have failed to demonstrate any improvement in outcome with regional cerebral perfusion.

Currently, numerous groups are advocating the use of an extracardiac conduit to complete the Fontan procedure. This technique may offer significant advantages; however, patients may be exposed to the risks of thromboembolic complications inherent in prosthetic conduits in the venous system. Lack of growth is also of concern. The literature does not have a consensus that favors one technique over the other.

Future considerations for the Fontan procedure in this subgroup of patients include minimization of thromboembolic events, preservation of right ventricular and tricuspid valve function, and prevention of arrhythmias.

 


More on Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure

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Follow-up: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Multimedia: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
References
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References

  1. Norwood WI, Lang P, Casteneda AR, Campbell DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. Oct 1981;82(4):511-9. [Medline].

  2. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. Jan 6 1983;308(1):23-6. [Medline].

  3. Bailey LL, Nehlsen-Cannarella SL, Doroshow RW, et al. Cardiac allotransplantation in newborns as therapy for hypoplastic left heart syndrome. N Engl J Med. Oct 9 1986;315(15):949-51. [Medline].

  4. Bailey LL, Nehlsen-Cannarella SL, Concepcion W, Jolly WB. Baboon-to-human cardiac xenotransplantation in a neonate. JAMA. Dec 20 1985;254(23):3321-9. [Medline].

  5. Noonan JA, Nadas AS. The hypoplastic left heart syndrome; an analysis of 101 cases. Pediatr Clin North Am. Nov 1958;5(4):1029-56. [Medline].

  6. Lev M. Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes. Lab Invest. 1952;1:61.

  7. Lev M, Arcilla R, Rimoldi HJA. Premature narrowing or closure of the foramen ovale. Am Heart J. 1963;65:638.

  8. Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg. Jul 1984;88(1):97-101. [Medline].

  9. Barber G, Helton JG, Aglira BA, et al. The significance of tricuspid regurgitation in hypoplastic left-heart syndrome. Am Heart J. Dec 1988;116(6 Pt 1):1563-7. [Medline].

  10. Chang AC, Farrell PE Jr, Murdison KA, et al. Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure. J Am Coll Cardiol. Apr 1991;17(5):1143-9. [Medline].

  11. Sano S, Ishino K, Kawada M, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. Journal of Thoracic & Cardiovascular Surgery. 2003;126:504-509. [Medline].

  12. Tabbutt S, Dominguez TE, Ravishankar C, et al. Outcomes after the stage I reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock Taussig shunt. Annals of Thoracic Surgery. 2005;80:1582-1590. [Medline].

  13. Bove EL, Lloyd TR. Staged reconstruction for hypoplastic left heart syndrome. Contemporary results. Ann Surg. Sep 1996;224(3):387-94; discussion 394-5. [Medline].

  14. Norwood WI Jr. Hypoplastic left heart syndrome. Ann Thorac Surg. Sep 1991;52(3):688-95. [Medline].

  15. Jonas RA, Hanson D, Cook N. Anatomical subtype of hypoplastic left heart syndrome influences survival after palliative reconstruction. Paper presented at: The American Association for Thoracic Surgery; 1992;Los Angeles, Calif.

  16. Tweddell JS, Hoffman GM, Mussato KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002;106:I82-I89. [Medline]. [Full Text].

  17. Douglas WI, Goldberg CS, Mosca RS, et al. Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan. Ann Thorac Surg. Oct 1999;68(4):1361-7; discussion 1368. [Medline].

  18. Forbess JM, Cook N, Serraf A, et al. 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. Mar 1 1997;29(3):665-70. [Medline].

  19. Mosca RS, Kulik TJ, Goldberg CS, et al. Early results of the fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. Jun 2000;119(6):1110-8. [Medline].

  20. Goldberg CS, Schwartz EM, Brunberg JA, et al. Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr. Nov 2000;137(5):646-52. [Medline].

  21. Amodeo A, Galletti L, Marianeschi S, et al. Extracardiac Fontan operation for complex cardiac anomalies: seven years' experience. J Thorac Cardiovasc Surg. Dec 1997;114(6):1020-30; discussion 1030-1. [Medline].

  22. Bando K, Turrentine MW, Sun K, et al. Surgical management of hypoplastic left heart syndrome. Ann Thorac Surg. Jul 1996;62(1):70-6; discussion 76-7. [Medline].

  23. Bove EL. The case for operative intervention. Ann Thorac Cardiovasc Surg. 1997;3.

  24. Charpie JR, Dekeon MK, Goldberg CS, et al. Postoperative hemodynamics after Norwood palliation for hypoplastic left heart syndrome. Am J Cardiol. Jan 15 2001;87(2):198-202. [Medline].

  25. Charpie JR, Dekeon MK, Goldberg CS, et al. Serial blood lactate measurements predict early outcome after neonatal repair or palliation for complex congenital heart disease. J Thorac Cardiovasc Surg. Jul 2000;120(1):73-80. [Medline].

  26. Douville EC, Sade RM, Fyfe DA. Hemi-Fontan operation in surgery for single ventricle: a preliminary report. Ann Thorac Surg. Jun 1991;51(6):893-9; discussion 900. [Medline].

  27. Fyler D, Nadas A. Report of the New England Regional Infant Cardiac Program. Pediatrics. Feb 1980;65(2 Pt 2):375-461. [Medline].

  28. Gentles TL, Gauvreau K, Mayer JE Jr, et al. Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg. Sep 1997;114(3):392-403; discussion 404-5. [Medline].

  29. Gentles TL, Mayer JE Jr, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg. Sep 1997;114(3):376-91. [Medline].

  30. Gundry SR, Razzouk AJ, del Rio MJ, et al. The optimal Fontan connection: a growing extracardiac lateral tunnel with pedicled pericardium. J Thorac Cardiovasc Surg. Oct 1997;114(4):552-8; discussion 558-9. [Medline].

  31. Hehrlein FW, Yamamoto T, Orime Y, Bauer J. Hypoplastic left heart syndrome: "Which is the best operative strategy?". Ann Thorac Cardiovasc Surg. Jun 1998;4(3):125-32. [Medline].

  32. Hsu DT, Quaegebeur JM, Ing FF, et al. Outcome after the single-stage, nonfenestrated Fontan procedure. Circulation. Nov 4 1997;96(9 Suppl):II-335-40. [Medline].

  33. Iannettoni MD, Bove EL, Mosca RS, et al. Improving results with first-stage palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. Mar 1994;107(3):934-40. [Medline].

  34. Ishino K, Stumper O, De Giovanni JJ, et al. The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg. May 1999;117(5):920-30. [Medline].

  35. Kaulitz R, Ziemer G, Luhmer I, Kallfelz HC. Modified Fontan operation in functionally univentricular hearts: preoperative risk factors and intermediate results. J Thorac Cardiovasc Surg. Sep 1996;112(3):658-64. [Medline].

  36. Koutlas TC, Gaynor JW, Nicolson SC, et al. Modified ultrafiltration reduces postoperative morbidity after cavopulmonary connection. Ann Thorac Surg. Jul 1997;64(1):37-42; discussion 43. [Medline].

  37. Lamberti JJ, Spicer RL, Waldman JD, et al. The bidirectional cavopulmonary shunt. J Thorac Cardiovasc Surg. Jul 1990;100(1):22-9; discussion 29-30. [Medline].

  38. Lardo AC, Webber SA, Friehs I, et al. Fluid dynamic comparison of intra-atrial and extracardiac total cavopulmonary connections. J Thorac Cardiovasc Surg. Apr 1999;117(4):697-704. [Medline].

  39. Meliones JN, Snider AR, Bove EL, et al. Longitudinal results after first-stage palliation for hypoplastic left heart syndrome. Circulation. Nov 1990;82(5 Suppl):IV151-6. [Medline].

  40. Mosca RS, Bove EL, Crowley DC, et al. Hemodynamic characteristics of neonates following first stage palliation for hypoplastic left heart syndrome. Circulation. Nov 1 1995;92(9 Suppl):II267-71. [Medline].

  41. Natowicz M, Chatten J, Clancy R, et al. Genetic disorders and major extracardiac anomalies associated with the hypoplastic left heart syndrome. Pediatrics. Nov 1988;82(5):698-706. [Medline].

  42. Norwood WI Jr. Hypoplastic left heart syndrome. In: Baue AF, Geha AS, Hammond GL, et al, eds. Glenn's Thoracic and Cardiovascular Surgery. Appleton & Lange; 1991.

  43. Petrossian E, Reddy VM, McElhinney DB, et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg. Apr 1999;117(4):688-96. [Medline].

  44. Pigula FA, Nemoto EM, Griffith BP, Siewers RD. Regional low-flow perfusion provides cerebral circulatory support during neonatal aortic arch reconstruction. J Thorac Cardiovasc Surg. Feb 2000;119(2):331-9. [Medline].

  45. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol. Apr 15 1993;71(11):959-62. [Medline].

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

  47. Rossi AF, Sommer RJ, Lotvin A, et al. Usefulness of intermittent monitoring of mixed venous oxygen saturation after stage I palliation for hypoplastic left heart syndrome. Am J Cardiol. Jun 1 1994;73(15):1118-23. [Medline].

  48. Sinha SN, Rusnak SL, Sommers HM, et al. Hypoplastic left ventricle syndrome. Analysis of thirty autopsy cases in infants with surgical considerations. Am J Cardiol. Feb 1968;21(2):166-73. [Medline].

  49. Stamm C, Anderson RH, Ho SY. The morphologically tricuspid valve in hypoplastic left heart syndrome. Eur J Cardiothorac Surg. Oct 1997;12(4):587-92. [Medline].

  50. Starnes VA, Griffin ML, Pitlick PT, et al. Current approach to hypoplastic left heart syndrome. Palliation, transplantation, or both?. J Thorac Cardiovasc Surg. Jul 1992;104(1):189-94; discussion 194-5. [Medline].

  51. Tweddell JS, Hoffman GM, Fedderly RT, et al. Phenoxybenzamine improves systemic oxygen delivery after the Norwood procedure. Ann Thorac Surg. Jan 1999;67(1):161-7; discussion 167-8. [Medline].

  52. Watson DG, Rowe RD. Aortic-valve atresia report of 43 cases. JAMA. 1962;179:14.

  53. Zales VR, Backer CL, Lynch P. Management of neonates with the hypoplastic left heart syndrome prior to cardiac transplantation. Paper presented at: The American Academy of Pediatrics Annual Meeting;. 1991;Boston, Massachusetts.

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Further Reading

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Keywords

hypoplastic left heart syndrome, staged Norwood procedure, HLHS, left ventricular hypoplasia, absence of left ventricle, ascending aorta hypoplasia, staged orthoterminal correction, cardiac anomaly, cardiac disease, cardiac surgery, heart surgery, heart disease, orthotopic cardiac transplantation, cardiac transplantation, staged reconstructive cardiac surgery, right ventricle–to–pulmonary artery conduit, RVPAC, heart transplantation, aortic atresia, mitral atresia, congenital heart disease, patent ductus arteriosus, metabolic acidosis, renal failure, aortic stenosis, mitral stenosis, tricuspid regurgitation, cardiomegaly, obstructed pulmonary venous return, atrial septal defect, Fontan procedure

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Contributor Information and Disclosures

Author

Richard G Ohye, MD, Director, Pediatric Cardiac Transplantation, Fellowship Program Director, Pediatric Cardiac Surgery, Assistant Professor, Department of Surgery, Section of Cardiac Surgery, University of Michigan Medical Center
Richard G Ohye, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Ralph S Mosca, MD, Director, Pediatric Cardiac Surgery, Associate Professor, Department of Surgery, New York Presbyterian Medical Center
Ralph S Mosca, MD is a member of the following medical societies: American College of Surgeons, Central Surgical Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, Michigan State Medical Society, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Edward L Bove, MD, Associate Director, PICU, CS Mott Children's Hospital; Director, Department of Surgery, Section of Thoracic Surgery, Division of Pediatric Cardiovascular Surgery, Professor, University of Michigan Medical Center
Edward L Bove, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, American Heart Association, American Medical Association, American Surgical Association, Central Surgical Association, Congenital Heart Surgeons Society, Medical Society of the State of New York, Society of Thoracic Surgeons, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Jonah Odim, MD, PhD, MBA, Senior Medical Officer, Transplantation Immunology Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health
Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physician Executives, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, Canadian Cardiovascular Society, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting; Pfizer Honoraria Consulting

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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