Skip to main content Accessibility help
×
Home

Catheter interventions in the staged management of hypoplastic left heart syndrome

  • Zdenka Reinhardt (a1), Joseph De Giovanni (a1), John Stickley (a1), Vinay K. Bhole (a1), Benjamin Anderson (a1), Bari Murtuza (a1), Chetan Mehta (a1), Paul Miller (a1), Rami Dhillon (a1) and Oliver Stumper (a1)...

Abstract

Aim

To analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.

Methods

This study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years.

Results

A staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan–Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction – 84 balloon angioplasties – and augment pulmonary blood flow – 15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery – 73 procedures and abolishing systemic venous collaterals – 32 procedures. After Stage III, the focus was on manipulating the size of the fenestration – 42 interventions – and the left pulmonary artery −31 procedures.

Conclusion

Interventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.

Copyright

Corresponding author

Correspondence to: Dr O. Stumper, MD, PhD, Consultant Paediatric Cardiologist, Department of Paediatric Cardiology, Heart Unit, Birmingham Children's Hospital NHS Trust, Steelhouse Lane, Birmingham B6 4NH, United Kingdom. Tel: +44121 333 9999; Fax: +44121 333 9998; E-mail: oliver.stumper@bch.nhs.uk

References

Hide All
1. Norwood, WIJ, Jacobs, ML, Murphy, JD. Fontan procedure for hypoplastic left heart syndrome. Ann Thorac Surg 1992; 54: 10251029; discussion 1029–1030.
2. Maher, KO, Gidding, SS, Baffa, JM Jr, et al. New developments in the treatment of hypoplastic left heart syndrome. Minerva Pediatr 2004; 56: 4149.
3. 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: 504509.
4. Barron, DJ, Brooks, A, Stickley, J, et al. The Norwood procedure using a right ventricle-pulmonary artery conduit: comparison of the right-sided versus left-sided conduit position. J Thorac Cardiovasc Surg 2009; 138: 528537.
5. Stümper, O, Gewillig, M, Vettukattil, J, et al. Modified technique of stent fenestration of the atrial septum. Heart 2003; 89: 12271230.
6. Anderson, B, Bhole, V, Desai, T, et al. Novel technique to reduce the size of a Fontan Diabolo stent fenestration. Catheter Cardiovasc Interv 2010; 76: 860864.
7. McGuirk, SP, Griselli, M, Stumper, OF, et al. Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart 2006; 92: 364370.
8. 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 (Suppl 1): I82I89.
9. Alsoufi, B, Bennetts, J, Verma, S, et al. New developments in the treatment of hypoplastic left heart syndrome. Pediatrics 2007; 119: 109117.
10. Algra, SO, Breur, JM, Evens, FC, et al. Improving surgical outcome following the Norwood procedure. Neth Heart J 2011; 19: 369372.
11. Moszura, T, Mazurek-Kula, A, Dryzek, P, et al. Interventions complementing surgery as part of multistage treatment for hypoplastic left heart syndrome: one center's experience. Pediatr Cardiol 2009; 30: 106113.
12. Chessa, M, Dindar, A, Vettukattil, JJ, et al. Balloon angioplasty in infants with aortic obstruction after the modified stage I Norwood procedure. J Am Heart J 2000; 140: 227231.
13. Zeltser, I, Menteer, J, Gaynor, JW, et al. Impact of re-coarctation following the Norwood operation on survival in the balloon angioplasty era. J Am Coll Cardiol 2005; 45: 18441848.
14. Tworetzky, W, McElhinney, DB, Burch, GH, et al. Balloon arterioplasty of recurrent coarctation after the modified Norwood procedure in infants. Catheter Cardiovasc Interv 2000; 50: 5458.
15. Desai, T, Stumper, O, Miller, P, et al. Acute interventions for stenosed right ventricle-pulmonary artery conduit following the right-sided modification of Norwood-Sano procedure. Congenit Heart Dis 2009; 4: 433439.
16. Dähnert, I, Riede, FT, Razek, V, et al. Catheter interventional treatment of Sano shunt obstruction in patients following modified Norwood palliation for hypoplastic left heart syndrome. Res Cardiol 2007; 96: 719722.
17. McElhinney, DB, Reddy, VM, Hanley, FL, et al. Systemic venous collateral channels causing desaturation after bidirectional cavopulmonary anastomosis: evaluation and management. J Am Coll Cardiol 1997; 30: 817824.
18. Nakanishi, T. Cardiac catheterization is necessary before bidirectional Glenn and Fontan procedures in single ventricle physiology. Pediatr Cardiol 2005; 26: 159161.
19. Kanter, KR, Vincent, RN, Raviele, AA. Importance of acquired systemic-to-pulmonary collaterals in the Fontan operation. Ann Thorac Surg 1999; 68: 969974; discussion 974–975.
20. Bhole, V, Wright, JG, De Giovanni, JV, et al. Transcatheter interventions in the early postoperative period after the Fontan procedure. Catheter Cardiovasc Interv 2011; 77: 9298.
21. Tofeig, M, Walsh, KP, Chan, C, et al. Occlusion of Fontan fenestrations using the Amplatzer septal occluder. Heart 1998; 79: 368370.
22. Rao, PS. Catheter-based device closure of Fontan fenestrations. Catheter Cardiovasc Interv 2001; 52: 407408.

Keywords

Catheter interventions in the staged management of hypoplastic left heart syndrome

  • Zdenka Reinhardt (a1), Joseph De Giovanni (a1), John Stickley (a1), Vinay K. Bhole (a1), Benjamin Anderson (a1), Bari Murtuza (a1), Chetan Mehta (a1), Paul Miller (a1), Rami Dhillon (a1) and Oliver Stumper (a1)...

Metrics

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed