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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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