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Ann Thorac Surg 2002;74:912-914
© 2002 The Society of Thoracic Surgeons

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Case report

Left ventricular assist device for right side assistance in patients with transposition

^a Department of Surgery, Division of Cardiothoracic Surgery, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA

Accepted for publication April 1, 2002.

* Address reprint requests to Dr Acker, Department of Surgery, Division of Cardiothoracic Surgery, Hospital of the University of Pennsylvania, 6 Silverstein Pavilion, 3400 Spruce St, Philadelphia, PA 19103, USA
e-mail: macker{at}uphs.upenn.edu

	Abstract

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Right (systemic) ventricular dysfunction is well described after Senning operations for transposition of the great arteries, and patients with congenitally corrected transposition of the great arteries. Transplantation remains the only definitive therapy for refractory heart failure, however patients may deteriorate clinically prior to the availability of a donor heart. This report details the implantation of a TCI Heartmate (Thoratec Corp., Pleaston, CA) as a morphologic right ventricular assist device to bridge these patients to transplantation.

	Introduction

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Right (systemic) ventricular dysfunction is well described after Senning operations for transposition of the great arteries (TGA), [1] and patients with congenitally corrected transposition of the great arteries (ccTGA). Patients initially do well, but often deteriorate with right (systemic) ventricular failure after the second decade of life [2]. Transplantation remains the only definitive therapy for refractory heart failure. The number of patients suffering from irreversible heart failure after atrial switch operations, and also after Fontan-like interventions will likely increase during the next several years. In the absence of a donor organ, these patients may expire secondary to end-stage heart failure. This report describes 2 patients, one with TGA post-Senning procedure, and the second with ccTGA postvalve replacement, who were each successfully bridged to transplantation with a TCI Heartmate left ventricular assist device (LVAD) placed in the morphologic right ventricle.

	Case reports

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Patient 1
D.M. is a girl, who at 4 months of age underwent a Senning procedure for D transposition of the great vessels. She did well until age 15, when she presented with cyanosis, secondary to an intraatrial baffle leak. She underwent repair of the leak, but on postoperative day 1, she experienced a cardiac arrest with sustained right ventricular failure, requiring emergent institution of extracorporeal membrane oxygenation (ECMO). Cardiac catheterization revealed a right coronary artery occlusion requiring the placement of a right internal mammary artery graft to the right coronary artery.

Two months following her baffle repair, she developed an acute right (systemic) ventricular failure due to occlusion of her right internal mammary artery. She underwent percutaneous atrial septoplasty to decompress her right ventricle and reinstitution of ECMO. Thrombocytopenia, coagulopathy, and a failure to wean from ECMO complicated her course. She was ultimately taken to the operating room, and a TCI Heartmate was inserted with the in-flow originating from the free wall of the right (systemic) ventricle. The pneumatic pump was placed intraperitoneally, in a more midline position than normal. The outflow graft ascended within the right pleural space and was anastomosed to the ascending aorta. Because of her small size (body surface area 1.6), the abdomen was closed with multiple relaxing incisions and mesh. She did well but, on postoperative day 17, she developed an abdominal dehiscence. She was taken back to the operating room and a rectus muscle flap was used to obtain a tension free closure of the abdomen. She was extubated postoperatively, and maintained without further incident for 12 weeks on VAD support.

She subsequently underwent uncomplicated removal of the device and placement of an allograft. She is currently 3 years posttransplant and continues to do well with good allograft function.

Patient 2
G.G. is a 30-year-old man with the diagnosis of congenitally corrected L transposition of the great vessels. He did well until 20 years of age, when he required replacement of his systemic atrial-ventricular valve tricuspid with a Carpentier-Edwards porcine valve and closure of an atrial septal defect.

At age 28, the patient presented with failure of his systemic ventricle. Cardiac catheterization revealed super-systemic pulmonary pressures (90 to 110 mmHg), a pulmonary vascular resistance of 8 Woods units, a 30 mm gradient across the prosthetic valve, and a systemic ejection fraction of 10% to 15%. On echocardiogram, the right ventricle was massively dilated and the porcine valve was heavily calcified with poor function. The patient was initially considered for a heart-lung transplant, however, the addition of nitric oxide decreased his pulmonary vascular resistance from 8 to 2.6. However, in addition to his systemic atrial-ventricular valvular stenosis, he had refractory failure of his systemic ventricle. It was concluded that the patient could not tolerate valve replacement and was taken to the operating room for valve removal and placement of a VAD.

The valve was removed, creating a single chamber that included the left atrium and right ventricle. An apical core was removed from the right (systemic) ventricle and a TCI Heartmate was placed with the outflow originating from the right ventricle. The pneumatic pump was placed intraperitoneally.

After 8 months of VAD support, the patient underwent transplantation. He did well postoperatively and was discharged to home on posttransplant day 20. He is currently 17 months posttransplant with normal allograft function.

	Comment

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Late right ventricular failure in patients with TGA post-Senning or Mustard procedures or in those with ccTGA, is a difficult problem. Examination of right ventricular function after Senning or Mustard procedure has demonstrated decreased ejection fractions, symptomatic myocardial dysfunction, and abnormal right ventricular response to methoxamine challenge. Tricuspid valve surgery is not successful in arresting the progression of right ventricular failure [2]. Anatomic correction is advocated in patients who have deteriorated to New York Heart Association class III or IV, however there is debate over the operative indications for patients with compensated right ventricular failure [2]. In patients with severe right ventricular failure, with coexisting left ventricular dysfunction, orthotopic heart transplantation is recommended [2, 3]. Several reports of heart transplantation in adolescents and adult patients with congenital disease demonstrate that long-term survival is equivalent to adult acquired heart disease patients, but that the perioperative risk varies between 27.2% and 60%.

Both cases discussed above, presented with irreversible right heart failure (systemic ventricle) and an inability to survive without support until a donor heart became available. At the time of VAD insertion, case 1 had severe, irreversible heart failure along with the adverse effects of prolonged ECMO support; namely coagulopathy, thrombocytopenia, and pulmonary dysfunction. Case 2 also had severe right ventricular dysfunction along with left prosthetic atrial-ventricular valve (morphologic tricuspid, functional mitral) insufficiency and a pulmonary vascular resistance of 8. Given this severe pulmonary hypertension, immediate transplantation may have induced right heart failure in the transplanted heart. Since the TCI Heartmate can adequately unload the left ventricle (systemic) in patients with acquired heart failure, we attempted to sustain mechanical unloading and support by placement of the inflow valve in the systemic (right) ventricle of these 2 patients. The outflow is placed in the usual position in the ascending aorta. Postinsertion flow rates were not impaired by placement of the inflow in the right ventricle. The absence of a systemic atrial-ventricular valve in this patient was tolerated well for 9 months of ventricular support.

Supporting the systemic ventricle with an implantable TCI LVAD required placement of the pneumatic pump closer to the midline, secondary to the position of the right ventricle in the chest. In addition, the driveline exit site is placed in the right lower quadrant instead of the left. This midline placement positions the device directly under the midline incision, which in both cases led to wound dehiscence and required extensive revisions of the wound. Both patients had low body surface area, which contributed to the subsequent abdominal wound problems.

This report describes supporting the right (systemic) ventricle of patients with TGA as a bridge to transplantation with a TCI LVAD. It is expected that an increased number of patients who are post-Senning or Mustard procedures or with ccTGA will present with late right ventricular failure in the future. With the paucity of donor hearts and the lack of definitive surgical alternatives, supporting the systemic ventricle with an implantable TCI LVAD is a viable means of bridging to transplantation.

	References

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1. Trusler G.A., Williams W.G., Izukawa T., Olley P.M. Current results with the Mustard operation in isolated transposition of the great arteries. J Thorac Cardiovasc Surg 1980;80:381-389.[Medline]
2. Chang A.C., Wernovsky G., Wessel D.L., et al. Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation 1992;86(suppl 2):140.
3. Benson L.N., Bonet J., Maclaughlin P., et al. Assessment of right ventricular function during supine bicycle exercise after Mustard operation. Circulation 1982;65:1052-1061.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Robert C. Gorman Bruce R. Rosengard Michael A. Acker Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Stewart, A. S. Articles by Acker, M. A. Search for Related Content PubMed PubMed Citation Articles by Stewart, A. S. Articles by Acker, M. A. Related Collections Mechanical Circulatory Assistance