Surgical Treatment of Pediatric Hypoplastic Left Heart Syndrome Workup

1. Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010 May 27. 362(21):1980-92. [Medline]. [Full Text].

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

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

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

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

6. Pigott JD, Murphy JD, Barber G, Norwood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg. 1988 Feb. 45(2):122-8. [Medline].

7. Sano S. New era in management of hypoplastic left heart syndrome. Asian Cardiovasc Thorac Ann. 2007 Apr. 15(2):83-5. [Medline].

8. Jacobs JP, O'Brien SM, Chai PJ, Morell VO, Lindberg HL, Quintessenza JA. Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007. Ann Thorac Surg. 2008 May. 85(5):1691-6; discussion 1697. [Medline].

9. Ashburn DA, McCrindle BW, Tchervenkov CI, et al. Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg. 2003 May. 125(5):1070-82. [Medline].

10. Daebritz SH, Nollert GD, Zurakowski D, et al. Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations. J Thorac Cardiovasc Surg. 2000 Feb. 119(2):358-67. [Medline].

11. Ishino K, Stümper 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. 1999 May. 117(5):920-30. [Medline].

12. Bove EL. Current status of staged reconstruction for hypoplastic left heart syndrome. Pediatr Cardiol. 1998 Jul-Aug. 19(4):308-15. [Medline].

13. Kishimoto H, Kawahira Y, Kawata H, Miura T, Iwai S, Mori T. The modified Norwood palliation on a beating heart. J Thorac Cardiovasc Surg. 1999 Dec. 118(6):1130-2. [Medline].

14. Sano S, Ishino K, Kado H, et al. Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: a multi-institutional study. Ann Thorac Surg. 2004 Dec. 78(6):1951-7; discussion 1957-8. [Medline].

15. Sano S, Ishino K, Kawada M, Honjo O. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004. 7:22-31. [Medline].

16. Malec E, Januszewska K, Kolcz J, Mroczek T. Right ventricle-to-pulmonary artery shunt versus modified Blalock-Taussig shunt in the Norwood procedure for hypoplastic left heart syndrome - influence on early and late haemodynamic status. Eur J Cardiothorac Surg. 2003 May. 23(5):728-33; discussion 733-4. [Medline].

17. Pizarro C, Malec E, Maher KO, et al. Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation. 2003 Sep 9. 108 Suppl 1:II155-60. [Medline].

18. Pizarro C, Norwood WI. Right ventricle to pulmonary artery conduit has a favorable impact on postoperative physiology after Stage I Norwood: preliminary results. Eur J Cardiothorac Surg. 2003 Jun. 23(6):991-5. [Medline].

19. Januszewska K, Kolcz J, Mroczek T, Procelewska M, Malec E. Right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig shunt in preparation to hemi-Fontan procedure in children with hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2005 Jun. 27(6):956-61. [Medline].

20. Rumball EM, McGuirk SP, Stümper O, et al. The RV-PA conduit stimulates better growth of the pulmonary arteries in hypoplastic left heart syndrome. Eur J Cardiothorac Surg. 2005 May. 27(5):801-6. [Medline].

21. Caspi J, Pettitt TW, Mulder T, Stopa A. Development of the pulmonary arteries after the Norwood procedure: comparison between Blalock-Taussig shunt and right ventricular-pulmonary artery conduit. Ann Thorac Surg. 2008 Oct. 86(4):1299-304. [Medline].

22. Ohye RG, Ludomirsky A, Devaney EJ, Bove EL. Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation. Ann Thorac Surg. 2004 Sep. 78(3):1090-3. [Medline].

23. Ballweg JA, Dominguez TE, Ravishankar C, et al. A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at Fontan completion. J Thorac Cardiovasc Surg. 2010 Sep. 140(3):537-44. [Medline].

24. Ballweg JA, Dominguez TE, Ravishankar C, et al. A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction. J Thorac Cardiovasc Surg. 2007 Aug. 134(2):297-303. [Medline].

25. Theis JL, Hu JJ, Sundsbak RS, et al. Genetic association between hypoplastic left heart syndrome and cardiomyopathies. Circ Genom Precis Med. 2020 Dec 16. [Medline].

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

27. McBride KL, Zender GA, Fitzgerald-Butt SM, et al. Linkage analysis of left ventricular outflow tract malformations (aortic valve stenosis, coarctation of the aorta, and hypoplastic left heart syndrome). Eur J Hum Genet. 2009 Jun. 17(6):811-9. [Medline]. [Full Text].

28. McBride KL, Riley MF, Zender GA, et al. NOTCH1 mutations in individuals with left ventricular outflow tract malformations reduce ligand-induced signaling. Hum Mol Genet. 2008 Sep 15. 17(18):2886-93. [Medline]. [Full Text].

29. Eghtesady P, Brar A, Hall M. Seasonality of hypoplastic left heart syndrome in the United States: a 10-year time-series analysis. J Thorac Cardiovasc Surg. 2011 Feb. 141(2):432-8. [Medline].

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

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

32. 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. 1991 Apr. 17(5):1143-9. [Medline].

33. Li J, Zhang G, Holtby H, et al. Carbon dioxide--a complex gas in a complex circulation: its effects on systemic hemodynamics and oxygen transport, cerebral, and splanchnic circulation in neonates after the Norwood procedure. J Thorac Cardiovasc Surg. 2008 Nov. 136(5):1207-14. [Medline].

34. 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].

35. 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].

36. Lai L, Laussen PC, Cua CL, Wessel DL, Costello JM, del Nido PJ. Outcomes after bidirectional Glenn operation: Blalock-Taussig shunt versus right ventricle-to-pulmonary artery conduit. Ann Thorac Surg. 2007 May. 83(5):1768-73. [Medline].

37. Tanoue Y, Kado H, Shiokawa Y, Fusazaki N, Ishikawa S. Midterm ventricular performance after Norwood procedure with right ventricular-pulmonary artery conduit. Ann Thorac Surg. 2004 Dec. 78(6):1965-71; discussion 1971. [Medline].

38. Ohye RG, Gaynor JW, Ghanayem NS, et al. Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure. J Thorac Cardiovasc Surg. 2008 Oct. 136(4):968-75. [Medline]. [Full Text].

39. Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. 2005 Mar-Apr. 26(2):190-9. [Medline].

40. Galantowicz M, Cheatham JP, Phillips A, Cua CL, Hoffman TM, Hill SL, et al. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg. 2008 Jun. 85(6):2063-70; discussion 2070-1. [Medline].

41. Goldberg CS, Bove EL, Devaney EJ, Mollen E, Schwartz E, Tindall S, et al. A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: outcomes for infants with functional single ventricle. J Thorac Cardiovasc Surg. 2007 Apr. 133(4):880-7. [Medline].

42. Visconti KJ, Rimmer D, Gauvreau K, del Nido P, Mayer JE Jr, Hagino I. Regional low-flow perfusion versus circulatory arrest in neonates: one-year neurodevelopmental outcome. Ann Thorac Surg. 2006 Dec. 82(6):2207-11; discussion 2211-3. [Medline].

43. Dent CL, Spaeth JP, Jones BV, Schwartz SM, Glauser TA, Hallinan B, et al. Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1523-30. [Medline].

44. Zampi JD, Hirsch JC, Goldstein BH, Armstrong AK. Use of a pressure guidewire to assess pulmonary artery band adequacy in the hybrid stage I procedure for high-risk neonates with hypoplastic left heart syndrome and variants. Congenit Heart Dis. 2013 Mar-Apr. 8(2):149-58. [Medline].

45. Siehr SL, Norris JK, Bushnell JA, et al. Home monitoring program reduces interstage mortality after the modified Norwood procedure. J Thorac Cardiovasc Surg. 2014 Feb. 147(2):718-23.e1. [Medline].

46. Kanade R, Shazly S, Ruano R. Interventions and neonatal outcomes of fetuses with hypoplastic left heart syndrome and congenital diaphragmatic hernia: a systematic review. J Matern Fetal Neonatal Med. 2020 Nov 18. 1-12. [Medline].

47. Christmann M, Valsangiacomo Buchel ER, Dave H, Klauwer D, Cavigelli-Brunner A. Prognostic value of troponin in infants with hypoplastic left heart syndrome between Stage I and II of palliation. Ann Pediatr Cardiol. 2018 Jan-Apr. 11 (1):56-9. [Medline]. [Full Text].

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

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

50. 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.

51. 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].

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

53. 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. 1997 Mar 1. 29(3):665-70. [Medline].

54. Lee TM, Aiyagari R, Hirsch JC, Ohye RG, Bove EL, Devaney EJ. Risk factor analysis for second-stage palliation of single ventricle anatomy. Ann Thorac Surg. 2012 Feb. 93(2):614-8; discussion 619. [Medline].

55. Ohye RG, Schonbeck JV, Eghtesady et al. Cause, timing, and location of death in the Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012 Oct. 144(4):907-14. [Medline]. [Full Text].

56. Tabbutt S, Ghanayem N, Ravishankar C, et al. Risk factors for hospital morbidity and mortality after the Norwood procedure: A report from the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2012 Oct. 144(4):882-95. [Medline].

57. Frommelt PC, Guey LT, Minich LL, et al. Does initial shunt type for the Norwood procedure affect echocardiographic measures of cardiac size and function during infancy?: the Single Vventricle Reconstruction trial. Circulation. 2012 May 29. 125(21):2630-8. [Medline]. [Full Text].

58. Aiyagari R, Rhodes JF, Shrader P, et al. Impact of pre-stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg. 2013 Dec 11. [Medline].

59. 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. 2000 Jun. 119(6):1110-8. [Medline].

60. Tabbutt S, Nord AS, Jarvik GP, et al. Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome. Pediatrics. 2008 Mar. 121(3):476-83. [Medline].

61. Puosi R, Korkman M, Sarajuuri A, et al. Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart. J Int Neuropsychol Soc. 2011 Nov. 17(6):1094-103. [Medline].

62. Sarajuuri A, Jokinen E, Puosi R, Mildh L, Mattila I, Lano A, et al. Neurodevelopment in children with hypoplastic left heart syndrome. J Pediatr. 2010 Sep. 157(3):414-20, 420.e1-4. [Medline].

63. Blitzer D, Nguyen S, Farooqi KM, Takeda K. Orthotopic heart transplantation and concomitant aortic arch replacement in an adult Fontan patient with hypoplastic left heart syndrome. Interact Cardiovasc Thorac Surg. 2020 Nov 24. [Medline].

64. Pasquali SK, He X, Jacobs JP, Jacobs ML, O'Brien SM, Gaynor JW. Evaluation of failure to rescue as a quality metric in pediatric heart surgery: an analysis of the STS Congenital Heart Surgery Database. Ann Thorac Surg. 2012 Aug. 94(2):573-9; discussion 579-80. [Medline]. [Full Text].

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

66. Takabayashi S, Kado H, Shiokawa Y, Fukae K, Nakano T. Comparison of hemodynamics between Norwood procedure and systemic-to-pulmonary artery shunt for single right ventricle patients. Eur J Cardiothorac Surg. 2005 Jun. 27(6):968-74. [Medline].

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

68. Cunningham TW, Tan Y, Krawczeski CD, et al. Incidence and impact of acute kidney injury in patients with hypoplastic left heart syndrome following the hybrid stage 1 palliation. Cardiol Young. 2020 Dec 2. 1-7. [Medline].

Ming-Sing Si, MD Assistant Professor of Cardiac Surgery, University of Michigan Medical School

Ming-Sing Si, MD is a member of the following medical societies: American Heart Association, Biomedical Engineering Society, Society of Thoracic Surgeons, Tissue Engineering and Regenerative Medicine International Society, International Society for Stem Cell Research

Disclosure: Nothing to disclose.

Jennifer C Hirsch-Romano, MD, MS, FACS, FACC Assistant Professor of Cardiac Surgery, Pediatric Cardiac Surgery, Department of Cardiac Surgery, Assistant Professor, Pediatric Cardiac Intensive Cre, Department of Pediatrics, Surgical Director, Pediatric Cardiothoracic Intensive Care Unit, Co-Director, Pediatric Cardiac Extracorporeal Life Support, Pediatric Cardiac Surgeon, Fetal Diagnosis and Treatment Center, University of Michigan Medical Center

Jennifer C Hirsch-Romano, MD, MS, FACS, FACC is a member of the following medical societies: American College of Cardiology, American College of Surgeons, Association for Academic Surgery, Congenital Heart Surgeons Society, Society of Thoracic Surgeons, Association of Women Surgeons, European Association for Cardio-Thoracic Surgery, John Alexander Society, Frederick A Coller Surgical Society, Midwest Pediatric Cardiology Society

Disclosure: Nothing to disclose.

Edward L Bove, MD Associate Director, PICU, CS Mott Children's Hospital; Director, Division of Pediatric Cardiovascular Surgery, Professor, Department of Surgery, Section of Thoracic Surgery, 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, Society of University Surgeons

Disclosure: Nothing to disclose.

Richard G Ohye, MD Head, Section of Pediatric Cardiovascular Surgery, Associate Professor of Cardiac Surgery, Program Director, Pediatric Cardiac Surgery Fellowship, University of Michigan Medical Center

Richard G Ohye, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Thoracic Surgery, Congenital Heart Surgeons Society, Society of University Surgeons, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD, MMM 

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, 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, 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, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

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.

• Print this section
• Print the entire contents of
• Print the entire contents of article