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A novel and unique treatment of right ventricular inflow obstruction in a patient with a Bjork modification of the Fontan palliation before pregnancy

Published online by Cambridge University Press:  29 March 2010

Charles J. T. Butcher ,
Carla M. Plymen  and
Fiona Walker
Charles J. T. Butcher
Affiliation:
The Heart Hospital, University College London Hospitals NHS Foundation Trust, London
Carla M. Plymen
Affiliation:
The Heart Hospital, University College London Hospitals NHS Foundation Trust, London
Fiona Walker*
Affiliation:
The Heart Hospital, University College London Hospitals NHS Foundation Trust, London
*
Correspondence to: Dr F. Walker, Consultant Adult Congenital Cardiology, The Heart Hospital, University College London Hospitals NHS Foundation Trust, 16-18 Westmoreland Street, London W1G 8PH. Tel: +0207 573 8888; Fax: +0207 573 8807; E-mail: Fiona.walker@uclh.nhs.uk
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Abstract

Introduction

Patients with complex congenital cardiac disease are increasingly surviving to adulthood and many are keen to consider pregnancy. Haemodynamic status should be optimal prior to embarking on pregnancy and for some this may mean surgical intervention to alleviate haemodynamic residua.

Methods

We report the successful implantation of a percutaneous pulmonary stent valve into a right atrial to right ventricular conduit in a young woman with a Bjork modification of the Fontan palliation to improve haemodynamics prior pregnancy.

Discussion

Catheter interventions offer a low-risk option for the treatment of haemodynamic residua and innovative use of new technologies such as the pulmonary stent valve presents a novel, safe, and effective treatment for such conduit problems.

Keywords

Percutaneous valvecongenital heart diseasematernity
Type
Brief Reports
Information
Cardiology in the Young , Volume 20 , Issue 3 , June 2010 , pp. 337 - 338
Copyright
Copyright © Cambridge University Press 2010

A young woman with a Bjork modification of the Fontan operation (Fig 1) was referred for pre-pregnancy counselling. Although reportedly New York Heart Association Class II, on objective exercise testing, she completed 6 minutes of the Bruce protocol, maximum workload eight metabolic equivalents (METs), stopping due to fatigue. Cardiac magnetic resonance imaging provided three-dimensional visualisation of the right atrium to right ventricular conduit, which measured 14 × 8 millimetres. It was freely regurgitant and moderately obstructed, mean pull-back gradient of 7 millimetres of mercury at cardiac catheterisation.

Figure 1 Line drawing showing a Bjork modification of the Fontan operation, in this patient for palliation of tricuspid atresia.

The Fontan circulation has a finely balanced physiology, which under normal circumstances, no arrhythmias, non-dilated right atrium, good ventricular function, low left ventricular end diastolic pressure, low pulmonary artery pressures, provides a good functional status. However, pregnancy is associated with a significant increase in cardiac workload and for good maternal outcome haemodynamics should be optimal.Reference Robson, Hunter, Boys and Dunlop1 It was therefore decided that the conduit obstruction and regurgitation should be alleviated before pregnancy, but because re-do surgery in the context of two prior sternotomies is associated with not insignificant risk, a percutaneous catheter intervention approach was chosen, with an attempt to implant a Melody valve within the conduit. Although never previously used for this indication, prior cardiac magnetic resonance imaging had defined the anatomy and morphology of the conduit, which looked suitable for Melody valve deployment.

The procedure was performed under general anaesthesia without complication. The conduit was balloon-sized, pre-dilated (22 millimetres × 40 millimetres BIB; BVM Medical, NuMED Inc.) and pre-stented (EV3® MAX-LD IntraStent, Paris) to provide additional support and rigidity for the Melody valve. The Melody valve, 18 millimetres, was then implanted and post-dilated with a 20 millimetre high pressure balloon (EnsembleTM, Medtronic Inc.; MullinsTM, NuMed Inc). Post-implantation, there was no pull-back gradient across the conduit and right ventricular angiography demonstrated trivial regurgitation only (Fig 2).

Figure 2 Coronal view angiogram of right ventricle. Valved stent can be seen clearly in situ within the RA–RV conduit. The stenosis has been relieved across the conduit and there remains only trivial regurgitant flow.

The patient was discharged home the following day and at 6 months follow-up functional status had improved (New York Heart Assocation Class I) and echocardiography demonstrated a competent pulmonary stent valve within the conduit, with a peak velocity of 1.2 metres per second, trivial regurgitation and a mean gradient of 3.6 millimetres of mercury. On repeat exercise testing (Bruce protocol) she completed 7 minutes and 50 seconds, workload nine METS, and she is now planning a pregnancy.

Conclusion

We report the first case of Melody stent valve deployment within a Bjork Fontan connection, which successfully treated conduit obstruction and regurgitation. The improvement in haemodynamics resulted in a better exercise performance and functional class, which highlights its potential use for these patients both within and outside the context of pregnancy planning. Cardiac magnetic resonance imaging provided three-dimensional imaging, which enabled accurate definition of the right atrial to the right ventricular conduit anatomy for the Melody valve deployment, while the percutaneous catheter approach offered a low-risk treatment option for what was potentially a high-risk problem.Reference Khambadkone, Coats and Taylor2 This case also demonstrates that innovative thinking and teamwork are essential for the optimal management of adults with congenital heart disease.Reference Lutter, Ardehali, Cremer and Bonhoeffer3

Acknowledgements

The authors thank Professor P. Bonhoeffer, Great Ormond Street Hospital, London, Dr Marina Hughes, Consultant Cardiologist, Great Ormond Street Hospital, London, and Dr Dominic Abrams, Consultant Cardiologist, creator and designer of line image.

References

1. Robson, SC, Hunter, S, Boys, RJ, Dunlop, W. Serial study of factors influencing changes in cardiac output during human pregnancy. Am J Physiol 1989; 256: 1060–1065.Google ScholarPubMed
2. Khambadkone, S, Coats, L, Taylor, A, et al. Percutaneous pulmonary valve inmplantation in humans: results in 59 consecutive patients. Circulation 2005; 112: 1189–1197.CrossRefGoogle Scholar
3. Lutter, G, Ardehali, R, Cremer, J, Bonhoeffer, P. Percutaneous valve replacement: current state and future prospects. Ann Thorac Surg 2004; 78: 2199–2206.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1 Line drawing showing a Bjork modification of the Fontan operation, in this patient for palliation of tricuspid atresia.

Figure 1

Figure 2 Coronal view angiogram of right ventricle. Valved stent can be seen clearly in situ within the RA–RV conduit. The stenosis has been relieved across the conduit and there remains only trivial regurgitant flow.