Ann Thorac Surg 2008;86:1018-1020. doi:10.1016/j.athoracsur.2008.03.003
© 2008 The Society of Thoracic Surgeons
Case Reports
Right-Sided Univentricular Cardiac Assistance in a Failing Fontan Circulation
René Prêtre, MDa,*,
Achim Häussler, MDa,
Dominique Bettex, MDb,
Michele Genoni, MDa
a Cardiovascular Surgery, Department of Surgery, University Hospital, Zürich, Switzerland
b Cardiac Anesthesia, Department of Surgery, University Hospital, Zürich, Switzerland
Accepted for publication March 3, 2008.
* Address correspondence to Dr Prêtre, Department of Surgery, University Hospital, Rämistrasse 100, Zürich, 8006, Switzerland (Email: rene.pretre{at}kispi.uzh.ch).
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Abstract
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Fontan patients are doomed to a circulatory failure and many of them will require a circulatory assistance as a bridge to transplantation. The univentricular heart with a total cavopulmonary connection presents a special challenge for the insertion of an assist device. We report a patient in multiple organ dysfunction and failure who was supported by right-sided univentricular assistance. Technically, a new chamber was created between both vena cava for implantation of the inflow cannula, and the extracardiac conduit was used to set the outflow cannula. The patient dramatically recovered and is currently in the best condition for heart transplantation.
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Introduction
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The shortage of donors has prolonged the time on a waiting list for heart transplant candidates and increased pressure on transplantation teams to optimize candidates to become a low surgical risk. Mechanical assist devices have proven an ability to maintain candidates in life during the waiting time and to promote recovery of hemodynamically strained organs [1].
Patients with univentricular hearts palliatively treated with the Fontan principles will at some time have terminal heart failure develop. Most patients are poor transplantation candidates because of a wasted nutritional status and multiple organ dysfunction. The implantation of a cardiac assist device can reverse organ dysfunction and replenish these patients.
We report the implantation of an assist device in a terminally ill Fontan patient who recovered from multiple organ failure and is now in optimal condition for transplantation. This is the first report of successful support limited to the pulmonary side (the failing side) of Fontan circulation.
A 27-year-old man had undergone a Blalock-Taussig shunt and then a Björk type of atriopulmonary connection for a tricuspid atresia and intact ventricular septum. Over the years he had developed dilatation of the right atrium with supraventricular arrhythmia, and chronic renal and hepatic failure, but without protein-losing enteropathy. A Fontan conversion with a cavopulmonary anastomosis and a fenestrated extracardial conduit with a Gore-Tex tube of 22 mm (W. L. Gore & Associates, Flagstaff, AZ) and a cryoablation maze were performed (Fig 1, left panel). The main pulmonary artery was divided and closed with a patch on the pulmonary bifurcation side. The postoperative course was stormy, but the patient progressively recovered.
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Fig 1. Schematic view of the implantation of the Berlin heart on the right circulation. Left sketch shows the cavopulmonary anastomosis and the extracardial conduit with a Gore-Tex graft (W. L. Gore & Associates, Flagstaff, AZ). Right sketch shows the implantation of the arterial cannula in the proximal stump of the extracardiac conduit, the capacity chamber created with an enlarging patch of Dacron in a Dacron graft (Vascutek Terumo, Wädenswil, Switzerland) and the connection of the superior vena cava in the capacity chamber with enlargement patch of xenopericardium. The venous cannula is inserted in the capacity chamber. Both cannula are brought percutaneously and connected to a paracorporeal ventricle.
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He was re-addressed to us 16 weeks later in moribund condition with cardiac, renal, and hepatic failure. He had massive ascites (estimated at 12 L) and watery diarrhea. His central venous pressure was 30 mm Hg and his arterial saturation was 88%.
Because of the normal function of the left ventricle and mitral valve, it was decided to implant univentricular assistance on the pulmonary side for a long-term bridge-to-transplantation. This was performed with cardiopulmonary bypass on a beating and perfused heart. A new capacity chamber (the inflow site) was created and separated from the pulmonary artery (the outflow site). The extracardiac conduit was divided in its middle part and its distal stump was used for implantation of the arterial line. The proximal part was removed. A 30-mm Dacron graft (Vascutek Terumo, Wädenswil, Switzerland) was inserted on the inferior vena cava. The cavopulmonary anastomosis was taken down and the pulmonary stump was closed directly. The venous stump was connected with the Dacron graft (Vascutek Terumo) using a patch of xenopericardium cut as a doughnut to adapt diameters (Fig 1, right panel). The superior part of the Dacron graft was enlarged with a Dacron patch (cut in the redundant part of the graft) to increase its volume capacity (Figs 1 and 2).
The venous cannula was inserted in the Dacron graft. Both cannula were brought through the skin and connected to a 60 mL Berlin Heart ventricle (Berlin Heart GMBH, Berlin, Germany). The postoperative course went extremely smooth with mobilization of the patient already during the first postoperative day. His nutritional and physical status improved dramatically. Ascites, which had recurred shortly after the operation, had spontaneously disappeared. Liver and renal functions recovered dramatically within a few weeks. Two months after implantation, the patient was in excellent condition and involved in a home fitness training program. He has been waiting for heart transplantation for 11 months.
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Fig 2. In vivo picture of the montage. The capacity chamber out of Dacron (Vascutek Terumo, Wädenswil, Switzerland) is clearly seen as well as the connection of the superior vena cava. The arterial cannula is directly inserted in the stump of the extracardiac conduit (right panel). Note the minimal opening of the sternum and the dissection limited to the right side of the heart.
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Comment
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Univentricular patients treated with Fontan circulation are probably doomed to premature cardiopulmonary failure. The ultimate therapy is cardiac transplantation. The shortage of donors not only prolongs the waiting time but also poses a question as to whether it is wise to allocate hearts to high-risk patients. Due to its chronic low cardiac output and elevated venous and lymphatic pressure, the failing Fontan patient presents often with advanced systemic organ damage. Survival time is short at the stage of wasting protein-losing enteropathy, and even an emergency transplantation is unlikely to be successful in such depleted patients.
Circulatory support with a paracorporeal assist device was the only chance of this terminally ill patient. The implantation of the device after total cavopulmonary connection requires the reconstruction of a capacity chamber separated from the pulmonary circulation. This was done with an enlarged Dacron graft. The outflow cannula was connected directly to the proximal stump of the extracardiac Gore-Tex conduit (W. L. Gore & Associates). This connection avoided additional surgery on the pulmonary arteries.
The implantation of the univentricular assist device on the right side has never been described in a failing Fontan. One group inserted biventricular assistance after failing cavopulmonary anastomosis in a Norwood-Fontan pathway [2]. This group was able to reconnect the superior vena cava to the right atrium, and implanted the cannulas in the usual fashion. Two other groups inserted a paracorporeal assist device in the apex of the left ventricle after a failing conversion to a total or partial cavopulmonary connection [3, 4]. In both situations, the main problem was a failing driving ventricle after surgery. A left ventricular assist device reduces the pressure in the left atrium and indirectly the venous and lymphatic congestion. Even though any reduction of the venous pressure has a significant impact in a Fontan circulation, the benefit of this approach remains limited in failing pulmonary circulation and would probably not have rescued our extremis patient. The only drawback of our montage is the impossibility to measure the evolution of the pulmonary vascular resistance, which is typically elevated in failing Fontan patients [5]. The more physiological distribution of blood within the lungs with pulsatility and the reduction of the lymphatic congestion are expected to reduce the resistance.
Because of the good function of the left ventricle, we decided to implant the assist device only on the pulmonary side. The hemodynamic situation improved dramatically in this patient, even though the mean venous pressure required approximately 10 days to fall under 8 mm Hg. The patient made a fantastic recovery with correction of his metabolic and organ dysfunctions and is now a suitable transplantation candidate.
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Addendum
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The patient underwent successfully the heart transplantation on the 28th April 2008, 13 months after implantation of the device. The procedure was performed by Professor Ludwig von Segesser in Lausanne, Switzerland.
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References
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- Rose EA, Moskowitz AJ, Packer M, et al. Long-term use of a left ventricular assist device for end-stage heart failure N Engl J Med 2001;345:1435-1443.[Abstract/Free Full Text]
- Nathan M, Baird C, Fynn-Thompson F, et al. Successful implantation of a Berlin heart biventricular assist device in a failing single ventricle J Thorac Cardiovasc Surg 2006;131:1407-1408.[Free Full Text]
- Frazier OH, Gregoric ID, Messner GN. Total circulatory support with an LVAD in an adolescent with a previous Fontan procedure Tex Heart Inst J 2005;32:402-404.[Medline]
- Newcomb AE, Negri JC, Brizard CP, d'Udekem Y. Successful left ventricular assist device bridge to transplantation after failure of a Fontan revision J Heart Lung Transplant 2006;25:365-367.[Medline]
- 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.[Abstract/Free Full Text]
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M. E. Mitchell
Invited commentary.
Ann. Thorac. Surg.,
July 1, 2009;
88(1):
176 - 176.
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Abstract
Introduction
