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Sildenafil in the management of the failing Fontan circulation

Published online by Cambridge University Press:  02 June 2010

Zdenka Reinhardt ,
Orhan Uzun ,
Vinay Bhole ,
Victor Ofoe ,
Dirk Wilson ,
Obed Onuzo ,
John G. C. Wright  and
Oliver Stumper
Zdenka Reinhardt
Affiliation:
The Heart Unit, Birmingham Children’s Hospital, NHS Foundation Trust, Birmingham, United Kingdom
Orhan Uzun
Affiliation:
Department of Paediatric Cardiology, University Hospital Wales, Cardiff, United Kingdom
Vinay Bhole
Affiliation:
The Heart Unit, Birmingham Children’s Hospital, NHS Foundation Trust, Birmingham, United Kingdom
Victor Ofoe
Affiliation:
The Heart Unit, Birmingham Children’s Hospital, NHS Foundation Trust, Birmingham, United Kingdom
Dirk Wilson
Affiliation:
Department of Paediatric Cardiology, University Hospital Wales, Cardiff, United Kingdom
Obed Onuzo
Affiliation:
Department of Paediatric Cardiology, University Hospital Wales, Cardiff, United Kingdom
John G. C. Wright
Affiliation:
The Heart Unit, Birmingham Children’s Hospital, NHS Foundation Trust, Birmingham, United Kingdom
Oliver Stumper*
Affiliation:
The Heart Unit, Birmingham Children’s Hospital, NHS Foundation Trust, Birmingham, United Kingdom
*
Correspondence to: O. Stumper, Birmingham Children’s Hospital, NHS Foundation Trust, Steelhouse Lane, Birmingham, B4 6NH, United Kingdom. Tel: 44 (0)121 333 9999; Fax: 44 (0)121 333 9441; E-mail: oliver.stumper@bch.nhs.uk and oliverstumper@hotmail.com
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Abstract

Background

Sildenafil is increasingly being used in the management of pulmonary arterial hypertension in the newborn. Its role in patients with congenital cardiac disease is less well defined and as yet has only been reported sporadically.

Aim

Present our experience with sildenafil treatment in patients with a failing Fontan circulation.

Patients and methods

Retrospective review of 13 symptomatic patients after Fontan palliation who received treatment with sildenafil between January, 2006 and July, 2008.

Results

Three patients suffered from protein-losing enteropathy, four patients presented with bronchial casts, two had severe cyanosis after fenestrated Fontan procedure, two had prolonged chylous effusions, one had a previous failure of Fontan and take-down, and one patient had arrhythmias and end-stage cardiac failure requiring conversion to an extra-cardiac Fontan. Sildenafil was used in the dosage of 1–2 milligrams per kilogram 3–4 times per day. Protein-losing enteropathy and α-1-antitrypsin levels improved in all three patients on sildenafil treatment. One of these patients had a concomitant catheter creation of a fenestration, as did two patients presenting with bronchial casts and both patients with persistent chylous effusions. All four patients with bronchial casts and two patients with cyanosis improved significantly on sildenafil treatment. Chylous effusions decreased after sildenafil and stent enlargement of a fenestration. There were no significant side effects requiring sildenafil withdrawal over a treatment period ranging from 2 months to 2 years.

Conclusions

Sildenafil can be used safely and effectively in the treatment of patients with a failing Fontan circulation.

Keywords

Fontan fenestrationprotein-losing enteropathypulmonary vascular resistance
Type
Original Articles
Information
Cardiology in the Young , Volume 20 , Issue 5 , October 2010 , pp. 522 - 525
Copyright
Copyright © Cambridge University Press 2010

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References

1. Redington, A. The physiology of the Fontan circulation. Prog Pediatr Cardiol 2006; 22: 179–186.CrossRefGoogle Scholar
2. Gewillig, M. The Fontan circulation. Heart 2005; 91: 839–846.CrossRefGoogle ScholarPubMed
3. Ghanayem, NS, Berger, S, Tweddell, JS. Medical management of the failing Fontan. Pediatr Cardiol 2007; 28: 465–471.CrossRefGoogle ScholarPubMed
4. Mertens, L, Hagler, DJ, Sauer, U, Somerville, J, Gewillig, M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115: 1063–1073.CrossRefGoogle ScholarPubMed
5. Rychik, J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007; 2: 288–300.CrossRefGoogle ScholarPubMed
6. Hosein, RB, Clarke, AJ, McGuirk, SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The ‘Two Commandments’? Eur J Cardiothorac Surg 2007; 31: 344–352; discussion 353.CrossRefGoogle ScholarPubMed
7. Khambadkone, S, Li, J, de Leval, MR, Cullen, S, Deanfield, JE, Redington, AN. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003; 107: 3204–3208.CrossRefGoogle ScholarPubMed
8. Baquero, H, Soliz, A, Neira, F, Venegas, ME, Sola, A. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded study. Pediatrics 2006; 117: 1077–1083.CrossRefGoogle ScholarPubMed
9. Humpl, T, Reyes, JT, Holtby, H, Stephens, D, Adatia, I. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation 2005; 111: 3274–3280.CrossRefGoogle Scholar
10. Haseyama, K, Satomi, G, Yasukochi, S, Matsui, H, Harada, Y, Uchita, S. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006; 132: 1232–1233.CrossRefGoogle Scholar
11. Stümper, O, Gewillig, M, Vettukattil, J, et al. Modified technique of stent fenestration of the atrial septum. Heart 2003; 89: 1227–1230.CrossRefGoogle ScholarPubMed
12. Uzun, O, Wong, JK, Bhole, V, Stumper, O. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg 2006; 82: e39–e40.CrossRefGoogle ScholarPubMed
13. Teixeira, CE, Priviero, FB, Webb, RC. Differential effects of the phosphodiesterase type 5 inhibitors sildenafil, vardenafil, and tadalafil in rat aorta. J Pharmacol Exp Ther 2006; 316: 654–661.CrossRefGoogle ScholarPubMed
14. Colle, I, De Vriese, AS, Van Vlierberghe, H, Lameire, NH, DeVos, M. Systemic and splanchnic haemodynamic effects of sildenafil in an in vivo animal model of cirrhosis support for a risk in cirrhotic patients. Liver Int 2004; 24: 63–68.CrossRefGoogle Scholar
15. Giardini, A, Balduci, A, Specchia, S, Gargiulo, G, Bonvicini, M, Picchio, FM. Effect of Sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008; 29: 1681–1687.CrossRefGoogle ScholarPubMed