Thorac Cardiovasc Surg 2008; 56(6): 348-352
DOI: 10.1055/s-2008-1038636
Original Cardiovascular

© Georg Thieme Verlag KG Stuttgart · New York

Evolving Strategies and Improving Outcomes of Norwood Stage One Reconstruction: A Decade Experience of a Single Institution in Taiwan

E.-T. Wu1 , S.-C. Huang2 , Y.-S. Chen2 , C.-I Chang2 , J.-K. Wang1 , M.-H. Wu1 , I.-S. Chiu2
  • 1Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan – Republic of China
  • 2Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan – Republic of China
Further Information

Publication History

received January 22, 2008

Publication Date:
14 August 2008 (online)

Abstract

Aim: The aim of this study was to define the improvement in short-term outcome and risk factors of Norwood stage one reconstruction for hypoplastic left heart syndrome (HLHS) in Taiwan, after implementing new perioperative management strategies. Methods: Data were retrieved from a retrospective chart review of patients with HLHS treated between July 1997 and July 2007. Since we implemented new perioperative strategies in 2004, we divided our patients into two groups, early era (1997 – 2003) and late era (2004 – 2007), and compared the outcome. Results: We enrolled 48 patients. In the early era group (n = 28), the diagnosis was confirmed by cardiac catheterization and controlled ventilation was used to manipulate the balance between systemic and pulmonary blood flow. The survival rate was only 17.9 % (5/28). Surgery was performed at 15.2 ± 10.7 days, which was significantly later than in the late era group (4.6 ± 4.0 days, n = 20). A lower preoperative shock and more prenatal diagnoses were recorded for the late era group. RV‐PA conduit was used in 17 patients in the late era group of which 12 (70.6 %) survived to be discharged from hospital. The risk factor was significant TR (triscupid regurgitation). Conclusions: With our contemporary perioperative management and change in surgical strategy, survival after first-stage palliation has improved. We believe that our HLHS experience is valuable for low volume centers and also for Asian cohorts.

References

  • 1 Allan L D, Sharland G, Tynan M J. The natural history of the hypoplastic left heart syndrome.  Intern J Cardiol. 1989;  25 341-343
  • 2 Norwood W I, Lang P, Hansen D D. Physiologic repair of aortic atresia-hypoplastic left heart syndrome.  N Engl J Med. 1983;  308 23-26
  • 3 Mahle W T, Spray T L, Wernovsky G, Gaynor J W, Clark 3rd B J. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution.  Circulation. 2000;  102 III136-III141
  • 4 Jacobs M L. Staged reconstructive surgery – the most appropriate therapy for hypoplastic left heart syndrome.  Cardiol Young. 2004;  14 (Suppl 1) 105-108
  • 5 Tweddell J S, Hoffman G M, Mussatto K A. et al . Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients.  Circulation. 2002;  106 I82-89
  • 6 Chiu I S, Chang J S, Lin S F. et al . [The first successful case or Norwood operation for hypoplastic left heart syndrome in Taiwan].  J Formos Med Assoc. 1995;  94 (Suppl 2) S156-S161
  • 7 Stieh J, Fischer G, Scheewe J. et al . Impact of preoperative treatment strategies on the early perioperative outcome in neonates with hypoplastic left heart syndrome.  J Thorac Cardiovasc Surg. 2006;  131 1122-1129.e2
  • 8 Sano S, Ishino K, Kawada M. et al . Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome.  J Thorac Cardiovasc Surg. 2003;  126 504-509 509-510
  • 9 Ungerleider R M, Shen I, Yeh T. et al . Routine mechanical ventricular assist following the Norwood procedure – improved neurologic outcome and excellent hospital survival.  Ann Thorac Surg. 2004;  77 18-22
  • 10 Wu K L, Lin M T, Wu E T. et al . Arterial switch operation for transposition of the great arteries: experience from 2000 – 2002 in Taiwan.  Acta Paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi. 2004;  45 19-22
  • 11 Chang R K, Chen A Y, Klitzner T S. Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988 – 1997.  Pediatrics. 2002;  110 292-298
  • 12 Bailey L L. Transplantation is the best treatment for hypoplastic left heart syndrome.  Cardiol Young. 2004;  14 (Suppl 1) 109-111 112-114
  • 13 Galantowicz M, Cheatham J P. Lessons Learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome.  Pediatr Cardiol. 2005;  26 190-199
  • 14 http://www.eactscongenitaldb.org
  • 15 Hirsch J C, Gurney J G, Donohue J E. et al . Hospital mortality for Norwood and arterial switch operations as a function of institutional volume.  Pediatr Cardiol. 2007;  2007;  DOI: 10.1007/s00246-007-9171-2
  • 16 Gutgesell H P, Gibson J. Management of hypoplastic left heart syndrome in the 1990s.  The Am J Cardiol. 2002;  89 842-846
  • 17 McGuirk S P, Griselli M, Stumper O F. et al . Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience.  Heart (British Cardiac Society). 2006;  92 364-370
  • 18 Pizarro C, Malec E, Maher K O. et al . Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome.  Circulation. 2003;  108 (Suppl 1) II155-II160
  • 19 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 1378-1384
  • 20 Ghanayem N S, Jaquiss R D, Cava J R. et al . Right ventricle-to-pulmonary artery conduit versus Blalock-Taussig shunt: a hemodynamic comparison.  Ann Thorac Surg. 2006;  82 1603-1609 1609-1610
  • 21 Daebritz S H, Nollert G D, Zurakowski D. et al . Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations.  J Thorac Cardiovasc Surg. 2000;  119 358-367
  • 22 Barber G, Helton J G, Aglira B A. et al . The significance of tricuspid regurgitation in hypoplastic left-heart syndrome.  Am Heart J. 1988;  116 1563-1567
  • 23 Weinstein S, Gaynor J W, Bridges N D. et al . Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome.  Circulation. 1999;  100 II167-II170
  • 24 Gaynor J W, Mahle W T, Cohen M I. et al . Risk factors for mortality after the Norwood procedure.  Eur J Cardiothorac Surg. 2002;  22 82-89
  • 25 Tworetzky W, McElhinney D B, Reddy V M. et al . Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome.  Circulation. 2001;  103 1269-1273

Dr. Ing-Sh Chiu

Department of Surgery
National Taiwan University Hospital

7th, Chung-Shan South Road

Taipei 100

Taiwan – Republic of China

Email: ingsh@ccms.ntu.edu.tw