Ann Thorac Surg 2007;83:306-308
© 2007 The Society of Thoracic Surgeons
Case Reports
Bridge to Transplantation With a Left Ventricular Assist Device for Systemic Ventricular Failure After Mustard Procedure
Robert S. George, BS, MRCSa,b,c,
Emma J. Birks, MRCP, PhDa,c,*,
Rosemary C. Radley-Smith, FRCPb,
Asghar Khaghani, FRCSa,
Magdi Yacoub, FRSc
a Departments of Transplantation and Mechanical Circulatory SupportHarefield, Middlesex, United Kingdom
b Department of Pediatric Cardiology, Royal Brompton and Harefield NHS TrustHarefield, Middlesex, United Kingdom
c National Heart and Lung Institute, Harefield, Middlesex, United Kingdom
Accepted for publication March 14, 2006.
* Address correspondence to Dr Birks, Royal Brompton and Harefield NHS Trust, Hill End Rd, Harefield, UB9 6JH Middlesex, UK (Email: e.birks{at}rbhnt.nhs.uk).
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Abstract
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After Mustard or Senning procedures, transplantation remains the only option for some patients who present at late stage with severe systemic (right) ventricular failure. In some circumstances these patients may require urgent mechanical circulatory support to bridge them to transplantation. The use of mechanical support poses considerable potential and actual specific problems both in terms of insertion and management of the device. We report the case of a 17-year-old patient who had a left ventricular assist device implanted from the right ventricle to the aorta for "end-stage" heart failure 15 years after the Mustard procedure. The specific problems are discussed and a management scheme is proposed.
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Introduction
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Late right (systemic) ventricular failure is well described after the Mustard procedure for transposition of the great arteries (TGA). The number of patients suffering from irreversible heart failure after atrial switch operations will likely rise in the next several years. Although transplantation remains the only definitive therapy, these patients could still deteriorate clinically prior to the availability of a donor. This report describes the successful use of the HeartMate I (Thoratec Corp, Pleasanton, CA) device as a bridge to transplantation while waiting for a suitable donor.
At 2 years of age our patient had a Mustard procedure for d-TGA using a trouser shaped autologous pericardial patch. Four years later he had surgical repair of superior baffle obstruction followed by balloon dilation of both superior and inferior baffle conduits. He remained well until the age of 15 when he presented with systemic ventricular heart failure. His echocardiogram revealed an estimated ejection fraction of 33%, systolic and diastolic diameters of 46 mm and 55 mm, respectively, and severe tricuspid regurgitation. Cardiac catheterization to assess his baffles status was performed, the results of which are shown in Fig 1. He was added to the transplant waiting list and discharged home while waiting for a donor.
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Fig 1. A diagram of d-transposition of the great arteries (d-TGA) corrected by the Mustard procedure. The numbers represent the angiographic results during catheterisation. (IVC = inferior vena cava; MP = mean arterial pressure; MPA = main pulmonary artery; Sats = saturation in percentage; SVC = superior vena cava.)
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His condition continued to deteriorate and he was readmitted 9 months later in end-stage heart failure with evidence of multiorgan dysfunction. His echocardiogram showed an extremely dilated globular systemic ventricle with ejection fraction less than 10%. The interventricular septum was akinetic and bulging toward the left ventricle, which was severely compressed (ie, banana shaped), and the right atrium was dilatated with turbulence in the systemic venous return baffle. He was in a low cardiac output state with apparent mild tricuspid regurgitation. Despite intensive inotropic support his condition worsened and a left ventricular assist device was inserted within 48 hours. The inflow cannula of a HeartMate I (Thoratec) was inserted in the right ventricle on the front of the right ventricular apex after division of some trabeculae with orientation toward the tricuspid valve while the outflow was inserted into the ascending aorta. The position of the inflow cannula was confirmed intraoperatively by transesophageal echocardiography after placement of the device. In spite of the moderately elevated pulmonary vascular resistance, pump filling and output were satisfactory. The pump was placed in the peritoneal cavity slightly nearer to the midline, and the driveline exited the right lower quadrant avoiding injury to the liver.
A year after implantation his on-off pump echocardiogram (protocol described elsewhere) [1] showed an ejection fraction of 71%; however, off-pump testing did not satisfy our criteria for explantation [2]. Transplantation was performed 1 month later and the donor was a 31-year-old man (body weight, 85 kg). Bypass was established using bi-caval cannulation as far away as possible from the atrium. The heart was removed with the whole of the right atrium and scar tissue as previously described [3]. The donor heart was then inserted using a modified bi-caval anastomosis. The total ischaemic time of the donor heart was 320 minutes and the HeartMate device was explanted from the abdomen.
The patient was discharged home within a few weeks on triple immunosuppressants. He is doing well with an ejection fraction of 65% at 41/2 years since transplantation, and he is employed full time.
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Comment
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Congenital heart defects are present in nearly 1% of all newborns, with TGA being one of the most common types of cyanotic congenital heart disease.
Atrial switch operations, namely the Senning and Mustard procedures, described in 1958 and 1964, respectively, are rarely performed today. However there remains a growing population of postoperative patients in their second or third decade of life. The incidence of right ventricular failure ranges from 8% to 44% [4, 5]. The absence of reservoir atrial function with or without baffle obstruction could interfere with the filling and possibly the function of the systemic ventricle leading to severe elevation of pulmonary venous pressure, which can result in progressive pulmonary vascular disease.
The patient required one invasive operation and transcutaneous balloon dilation of both baffles after his initial Mustard procedure for baffle obstruction. At the age of 15 he presented with severe heart failure refractory to medical management. The poor function and dilatation of the systemic ventricle resulted in compromise of his pulmonary ventricle leading to severe low cardiac output [6].
Other measures such as tricuspid repair or performing two-stage anatomical correction were considered to be nonviable options because of the advanced functional and structural changes of both ventricles.
Implantation of the left ventricular assist device in children or adults with congenital heart disease is not very common, and certainly the use of left ventricular assist devices in patients after atrial repair of TGA poses specific problems. Author Under and colleagues [7] reported that 3 of their 17 patients requiring extracorporeal life support had TGA. The only patient who was implanted with the HeartMate I had congenitally corrected TGA and died 106 days post-implantation.
The shape and size of the inflow cannula of the HeartMate I is designed for the left ventricle. The apex of the right ventricle is not as well developed and does not correspond to the apex of the heart. It also contains many trabeculae that could disturb the flow to the inflow cannula leading to inadequate drainage. These anatomical differences require special attention in placing the inflow cannula in the right ventricle to guarantee unobstructed flow in and out of the device, avoid injury to the liver and gut, and importantly to allow the chest to be closed.
Positioning of the device was addressed by Wiklund and colleagues [8] when they rotated the HeartMate I device back-to-front with the inflow to the right and the outflow cannula to the left in a patient with severe heart failure after a failing Mustard procedure [8]. They aimed to achieve optimal drainage with minimal disruption to the inflow or compromise to other organs. In our case this was unnecessary, and just placement of the device nearer to the midline was enough to achieve optimal drainage without any compromise to other organs, specifically the liver.
Explantation of the device from the peritoneal cavity was facilitated both by the absence of adhesions to the body of the device, and the fact that the Dacron velour (Thoratec Corp., Pleasanton, CA) covers part of the drive line excluding it from the peritoneal cavity during insertion.
Insertion of a left ventricular assist device in patients with congenital cardiac defects and those who had them repaired is technically demanding and time consuming. We show that insertion of a left ventricular assist device into the right ventricle of a patient with surgically corrected d-TGA is feasible and it served two purposes. First, it mechanically unloaded the systemic ventricle, thus reducing pulmonary hypertension that could have induced right heart failure in the transplanted heart, and second, the device acted as a bridge to transplantation. It is hoped that this report will help in optimizing the management of these patients.
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References
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- Yacoub M, Birks E, Tansley P, Henien M, Bowles C. Bridge to recovery: The Harefield approach J Congest Heart Failure & Circ Support 2001;2:27-30.
- Yacoub M. A novel strategy to maximise the efficacy of left ventricular assist devices as a bridge to recovery Eur Heart J 2001;22:534-540.[Free Full Text]
- Allard M, Assaad A, Bailey L, et al. Session IV: surgical techniques in paediatric heart transplantation J Heart Lung Transplant 1991;10:808-827.[Medline]
- Wilson N, Clarkson P, Barratt-Boyes B, et al. Long-term outcome after the Mustard repair for simple transposition of the great arteries J Am Coll Cardiol 1998;32:758-765.[Abstract/Free Full Text]
- Moons P, Gewillig M, Sluysmans T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium Heart 2004;90:307-313.[Abstract/Free Full Text]
- Yacoub M. Two hearts that beat as one Circulation 1995;92:156-157.[Free Full Text]
- Under A, McKenzie D, McGarry M, et al. Outcomes of congenital heart surgery patients after extracorporeal life support at Texas Children’s Hospital Artif Organs 2004;28:963-966.[Medline]
- Wiklund L, Svensson S, Berggren H. Implantation of a left ventricular assist device, back-to-front, in an adolescent with a failing mustard procedure J Thorac Cardiovasc Surg 1999;118:755-756.[Free Full Text]
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Abstract
Introduction
