Hostname: page-component-7c8c6479df-24hb2 Total loading time: 0 Render date: 2024-03-28T12:25:49.377Z Has data issue: false hasContentIssue false

Neonatal surgical reconstruction and peri-operative care for hypoplastic left heart syndrome: current strategies

Published online by Cambridge University Press:  13 December 2011

Stephanie Fuller*
Affiliation:
Cardiac Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Nancy Ghanayem
Affiliation:
Division of Pediatric Critical Care Medicine, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Paul Chai
Affiliation:
The Congenital Heart Institute of Florida (CHIF), All Children's Hospital, Saint Petersburg and Tampa, Florida, United States of America
John Nigro
Affiliation:
Cardiac Surgery, Phoenix Children's Hospital and St. Joseph's Hospital and Medical Center, Phoenix, Arizona, United States of America
Thomas Kleisli
Affiliation:
Phoenix Children's Hospital and St. Joseph's Hospital and Medical Center, Phoenix, Arizona, United States of America
Sarah Tabbutt
Affiliation:
Division of Pediatric Critical Care Medicine, Benioff Children's Hospital, University of California, San Francisco, United States of America
*
Correspondence to: Dr Stephanie Fuller, MD, Cardiac Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America. Tel: 215-590-2708; Fax: 215-590-2715; E-mail: FULLERS@email.chop.edu

Abstract

The management of newborns with hypoplastic left heart syndrome has changed significantly over the past three decades, with an associated dramatic improvement in outcomes. The aim of this paper is to discuss current peri-operative and palliative surgical strategies. Owing to the fact that comparative outcomes for these strategies have been addressed in a limited number of prospective trials and extractions from multi-centred databases, the primary focus of this review is descriptive.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Norwood, WI, Lang, P, Hansen, DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983; 308: 2326.CrossRefGoogle ScholarPubMed
2.Tweddell, JS, Ghanayem, NS, Mussatto, KA, et al. Mixed venous oxygen saturation monitoring after stage 1 palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2007; 84: 13011311.CrossRefGoogle ScholarPubMed
3.Sano, S, Huang, SC, Kasahara, S, Yoshizumi, K, Kotani, Y, Ishino, K. Risk factors for mortality after the Norwood procedure using right ventricle to pulmonary artery shunt. Ann Thorac Surg 2009; 87: 178186.CrossRefGoogle ScholarPubMed
4.Srinivasan, C, Sachdeva, R, Morrow, WR, et al. Standardized management improves outcomes after the Norwood procedure. Congenit Heart Dis 2009; 4: 329337.CrossRefGoogle ScholarPubMed
5.Ghanayem, NS, Hoffman, GM, Mussatto, KA, et al. Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 2010; 140: 857863.CrossRefGoogle ScholarPubMed
6.Furck, AK, Uebing, A, Hansen, JH, et al. Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12 year single center survey. J Thorac Cardiovasc Surg 2010; 139: 359365.CrossRefGoogle ScholarPubMed
7.Atz, AM, Travison, TG, Williams, IA, et al. Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 2010; 140: 16.CrossRefGoogle ScholarPubMed
8.Rychik, J, Szwast, A, Natarajan, S, et al. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5 year single institutional experience. Ultrasound Obstet Gynecol 2010; 36: 465470.CrossRefGoogle ScholarPubMed
9.Azakie, A, Merklinger, SL, McCrindle, BW, et al. Evolving strategies and improving outcomes of the modified Norwood procedure: a 10 year single-institution experience. Ann Thorac Surg 2001; 72: 13491353.CrossRefGoogle ScholarPubMed
10.Tabbutt, S, Ramamoorthy, C, Montenegro, LM, et al. Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 2001; 104: I-159I-164.CrossRefGoogle ScholarPubMed
11.Wernovsky, G, Ghanayem, N, Ohye, RG, et al. Hypoplastic left heart syndrome: consensus and controversies in 2007. Cardiol Young 2007; 17: 7586.CrossRefGoogle ScholarPubMed
12.Johnson, BA, Mussatto, K, Uhing, MR, Zimmerman, H, Tweddell, J, Ghanayem, N. Variability in the preoperative management of infants with hypoplastic left heart syndrome. Pediatr Cardiol 2008; 29: 515520.CrossRefGoogle ScholarPubMed
13.McElhinney, DB, Hedrick, HL, Bush, DM, et al. Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics 2000; 106: 10801087.CrossRefGoogle Scholar
14.Jeffries, HE, Wells, WJ, Starnes, VA, Wetzel, RC, Moromisato, DY. Gastrointestinal morbidity after Norwood palliation for hypoplastic left heart syndrome. Ann Thorac Surg 2006; 81: 982987.CrossRefGoogle ScholarPubMed
15.Jonas, RA, Lang, P, Hansen, D, Hickey, P, Castaneda, AR. First-stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size. J Thorac Cardiovasc Surg 1986; 92: 613.CrossRefGoogle ScholarPubMed
16.Brawn, W. Stage I Norwood: the Birmingham Children's Hospital Approach. Oper Tech Thorac Cardiovasc Surg 2005; 10: 286298.CrossRefGoogle Scholar
17.Meyer, DB, Spray, TL. Stage I – the Philadelphia Approach. Oper Tech Thorac Cardiovasc Surg 2005; 10: 299312.CrossRefGoogle Scholar
18.Sano, S, Ishino, K, Kawada, M, Honjo, O. Right ventricle-pulmonary artery shunt in first stage palliation of hypoplastic left heart-syndrome. Oper Tech Thorac Cardiovasc Surg 2005; 10: 141151.CrossRefGoogle Scholar
19.Tweddell, JS. The Norwood procedure with an innominate artery-to-pulmonary artery shunt. Oper Tech Thorac Cardiovasc Surg 2005; 10: 123140.CrossRefGoogle Scholar
20.Griselli, M, McGuirk, SP, Stumper, O, et al. Influence of surgical strategies on outcome after the Norwood procedure. J Thorac Cardiovasc Surg 2006; 131: 418426.CrossRefGoogle ScholarPubMed
21.Norwood, WI, Kirklin, JK, Sanders, SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol 1980; 45: 8791.CrossRefGoogle ScholarPubMed
22.Norwood, WI, Lang, P, Castaneda, AR, Campbell, DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1981; 82: 511519.CrossRefGoogle ScholarPubMed
23.Bu'Lock, FA, Stumper, O, Jagtap, R, et al. Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure. Br Heart J 1995; 73: 456461.CrossRefGoogle ScholarPubMed
24.Fraser, CDJ, Mee, RB. Modified Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 1995; 60: S546S549.CrossRefGoogle ScholarPubMed
25.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 1999; 117: 920930.CrossRefGoogle ScholarPubMed
26.Baker, CJ, Wells, WJ, Derby, CA, Rizi, S, Starnes, VA. Ascending aortic extension for enlargement of the aortopulmonary space in children with pulmonary artery stenosis. Ann Thorac Surg 2005; 80: 16471651.CrossRefGoogle ScholarPubMed
27.Bautista-Hernandez, V, Marx, GR, Gauvreau, K, et al. Coarctectomy reduces neoaortic arch obstruction in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2007; 133: 15401546.CrossRefGoogle ScholarPubMed
28.Hawkins, JA, Breinholt, JP, Lambert, LM, et al. Class I and class II anti-HLA antibodies after implantation of cryopreserved allograft material in pediatric patients. J Thorac Cardiovasc Surg 2000; 119: 324330.CrossRefGoogle Scholar
29.Meyer, SR, Campbell, DN, Rutledge, JM, et al. Use of an allograft patch in repair of hypoplastic left heart syndrome may complicate future transplantation. Eur J Cardiothorac Surg 2005; 27: 554560.CrossRefGoogle ScholarPubMed
30.Laing, BJ, Ross, DR, Meyer, SR, et al. Glutaraldehyde treatment of allograft tissue decreases allosensitization after the Norwood procedure. J Thorac Cardiovasc Surg 2010; 139: 14021408.CrossRefGoogle ScholarPubMed
31.Morell, VO, Wearden, PA. Experience with bovine pericardium for the reconstruction of the aortic arch in patients undergoing a Norwood procedure. Ann Thorac Surg 2007; 84: 13121315.CrossRefGoogle ScholarPubMed
32.Burkhart, HM, Ashburn, DA, Konstantinov, IE, et al. Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure. J Thorac Cardiovasc Surg 2005; 130: 6165.CrossRefGoogle ScholarPubMed
33.Ohye, RG, Sleeper, LA, Mahony, L, et al. Pediatric Heart Network Investigators. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 19801992.CrossRefGoogle ScholarPubMed
34.Tabbutt, S, Dominguez, TE, Ravishankar, C, et al. Outcomes after the stage 1 reconstruction comparing the right ventricular to pulmonary artery conduit with the modified Blalock–Taussig shunt. Ann Thorac Surg 2005; 80: 15821590.CrossRefGoogle ScholarPubMed
35.Azakie, A, Martinez, D, Sapru, A, et al. Impact of right ventricle to pulmonary artery conduit on outcome of the modified Norwood procedure. Ann Thorac Surg 2004; 77: 1172711733.CrossRefGoogle ScholarPubMed
36.Mahle, WT, Cuadrado, AR, Tam, VK. Early experience with a modified Norwood procedure using right ventricle to pulmonary artery conduit. Ann Thorac Surg 2003; 76: 10841088.CrossRefGoogle ScholarPubMed
37.Mair, R, Tulzer, G, Sames, E, et al. Right ventricular to pulmonary artery conduit instead of modified Blalock–Taussig shunt improves postoperative hemodynamics in newborns after the Norwood operation. J Thorac Cardiovasc Surg 2003; 126: 13781384.CrossRefGoogle ScholarPubMed
38.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; 108 Suppl 1: II-155II-160.CrossRefGoogle Scholar
39.Tabbutt, S, Goldberg, C, Ohye, RG, et al. Can randomized clinical trials impact the surgical approach for hypoplastic left heart syndrome? World J Pediatr Congenit Heart Surg 2011; 2: 445456.CrossRefGoogle ScholarPubMed
40.Newburger, JW, Jonas, RA, Wernovsky, G, et al. A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery. N Engl J Med 1993; 329: 10571064.CrossRefGoogle ScholarPubMed
41.Bellinger, DC, Wypij, D, duDuplessis, AJ, et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 13851396.CrossRefGoogle ScholarPubMed
42.Fuller, S, Rajagopaian, R, Jarvik, GP, et al. J Maxwell Chamberlain Memorial Paper for congenital heart surgery. Deep hypothermic circulatory arrest does not impair neurodevelopmental outcome in school-age children after infant heart surgery. Ann Thorac Surg 2010; 90: 19851994.CrossRefGoogle Scholar
43.Tabbutt, S, Nord, AS, Jarvik, GP, et al. Neurodevelopmental outcomes after staged palliation for hypoplastic left heart syndrome. Pediatrics 2008; 121: 476483.CrossRefGoogle ScholarPubMed
44.Goldberg, CS, Bove, EL, Devaney, EJ, et al. A randomized clinical trial of regoinal cerebral perfusion versus deep hypothermic circulatory arrest: outcomes for infants with functional single ventricle. J Thorac Cardiovasc Surg 2007; 133: 880887.CrossRefGoogle ScholarPubMed
45.Ohye, RG, Goldberg, CS, Donohue, J, et al. The quest to optimize neurodevelopmental outcomes in neonatal arch reconstruction: the perfusion techniques we use and why we believe in them. J Thorac Cardiovasc Surg 2009; 137: 803806.CrossRefGoogle Scholar
46.Jobes, DR, Nicolson, SC, Steven, JM, Miller, M, Jacobs, ML, Norwood, WI. Carbon dioxide prevents pulmonary overcirculation in hypoplastic left heart syndrome. Ann Thorac Surg 1992; 54: 150151.CrossRefGoogle ScholarPubMed
47.Furck, AK, Hansen, JH, Uebing, A, Scheewe, J, Jung, O, Kramer, HH. The impact of afterload reduction on the early postoperative course after the Norwood operation – a 12 year single centre experience. Eur J Cardiothorac Surg 2010; 37: 289295.Google ScholarPubMed
48.Tweddell, JS, Hoffman, GM, Mussatto, KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002; 106: I-82I-89.CrossRefGoogle ScholarPubMed
49.Bradley, SM, Atz, AM, Simsic, JM. Redefining the impact of oxygen and hyperventilation after the Norwood procedure. J Thorac Cardiovasc Surg 2004; 127: 473480.CrossRefGoogle ScholarPubMed
50.Hickey, PR, Hansen, DD, Wessel, DL, Lang, P, Jonas, RA, Elixson, EM. Blunting of stress responses in the pulmonary circulation of infants by fentanyl. Anesth Analg 1985; 64: 11371142.Google ScholarPubMed
51.Li, JS, You, E, Berezny, KY, et al. Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: does aspirin make a difference? Circulation 2007; 116: 293297.CrossRefGoogle Scholar
52.O'Connor, MJ, Ravishankar, C, Ballweg, JA, et al. Early systemic-to-pulmonary shunt intervention in neonates with congenital heart disease. J Thorac Cardiovasc Surg 2011; 142: 106112.CrossRefGoogle ScholarPubMed
53.Ravishankar, C, Dominguez, TE, Kreutzer, J, et al. Extracorporeal membrane oxygenation after stage 1 reconstruction for hypoplastic left heart syndrome. Pediatr Crit Care Med 2006; 7: 319323.CrossRefGoogle ScholarPubMed
54.Allan, CK, Thiagarajan, RR, del Nido, PJ, Roth, SJ, Almodovar, MC, Laussen, PC. Indication for initiation of mechanical circulatory support impacts survival of infants with shunted single-ventricle circulation supported with extracorporeal membrane oxygenation. J Thorac Cardiovasc Surg 2007; 133: 660667.CrossRefGoogle ScholarPubMed
55.Ghanayem, NS, Hoffman, GM, Mussatto, KA, et al. Home surveillance program prevents interstage mortality after the Norwood procedure. J Thorac Cardiovasc Surg 2003; 126: 13671377.CrossRefGoogle ScholarPubMed