Ann Thorac Surg 2007;83:1545-1547
© 2007 The Society of Thoracic Surgeons
Case Reports
Cardiac Autotransplantation and Radical Bi-Atrial Resection for Recurrent Atrial Myxoma
James S. Gammie, MD*,
A. Reza Abrishamchian, MD,
Bartley P. Griffith, MD
Division of Cardiac Surgery, University of Maryland, Medical Center, Baltimore, Maryland
Accepted for publication September 5, 2006.
* Address correspondence to Dr Gammie, Division of Cardiac Surgery, University of Maryland Medical Center, 22 S Greene St, Room N4W94, Baltimore, MD 21201 (Email: jgammie{at}smail.umaryland.edu).
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Abstract
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There have been sporadic reports of cardiac autotransplantation for ex-vivo resection of anatomically difficult cardiac tumors. We describe the first case of cardiac explantation, ex-vivo total biatrial resection, and reconstruction with bovine pericardial for recurrent cardiac myxoma.
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Introduction
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Carney complex is a rare entity characterized by recurrent atrial myxomas in association with skin hyperpigmentation and endocrine overactivity. We describe a patient with Carney complex facing a fourth operation for atrial tumor recurrence, autotransplantation facilitated total bi-atrial resection, and reconstruction with bovine pericardium. The patient remains well and free of tumor recurrence 3 years after operation.
A 47-year-old woman presented first in 1997 with exertional chest tightness associated with dyspnea, palpitation, and syncope. A 4.0-cm left atrial myxoma located on the fossa ovalis was diagnosed and resected uneventfully. She presented 2 years later with two myxomas at new locations in the left atrium. Standard resection was again successful. She was noted to have multiple lentigines and Carney complex (recurrent familial multifocal atrial myxomas and endocrine tumors) [1] was diagnosed. Two years later, surveillance echocardiography led to the diagnosis of an additional tumor again in a new location within the left atrium. Surgical excision was uneventful. A fourth recurrence consisting of a tumor arising in the right atrium was diagnosed 2 years later. Rather than performing a conventional fourth time reoperation, we reasoned that a more radical approach (ie, complete atrial resection) would minimize the likelihood of recurrence.
The heart was separated from dense adhesions through a redo median sternotomy. The superior and inferior cavae were cannulated directly, and cardiopulmonary bypass was established with femoral arterial return. Cardiectomy was performed in a fashion similar to that for transplantation.
The dome of the left atrium was entered and dissection was carried around the left atrium just above the level of the pulmonary veins. Both cavae were transected. The right atrium was excised and 1.5-cm gelatinous tumor was identified at the base of the septum, just above the coronary sinus.
The remaining left atrium in the patient’s chest was removed, leaving only orifices of the four pulmonary veins. Bovine pericardium was then fashioned with four circumferential openings each having a diameter of 1.5 to 2.0 cm. Each hole was sewn to the corresponding orifice of the pulmonary vein. The patch was then tailored to create a neo-posterior left atrium. A descending aortic homograft was interposed between the cut ends of the superior vena cava and inferior vena cava and was opened medially. The other end was then attached to superior vena cava (Fig 1). On the back table, near total excision of both atria was performed. Complete resection of atrial tissue was extended to the annulus of both atrioventricular valves. Pressurized retrograde infusion of cardioplegia into the coronary sinus helped to identify small venous bleeders that would not be visible after implantation; these were suture ligated. Both neo-atria were created by suturing bovine pericardium to each annulus. Each bovine patch was closed at the apex. The closure gathers the patch to appear similar to a bag with pursestring. The annular suturing was completed in two layers to optimize likelihood of hemostasis (Fig 2). At this point the heart and reconstructed atria were placed back into the chest. An oval hole was placed in the neo-posterior left atrium and the patch connecting the pulmonary veins was sutured to the edges. The systemic venous connection was made by anastomosing the opened homograft tube to a matching opening placed on the right lateral neo-posterior right atrium (Fig 3). Total ischemic time was 338 minutes; cardiopulmonary bypass time was 385 minutes. The patient’s hemodynamic function was excellent. The heart was paced with temporary epicardial leads. She was removed from ventilation support the following morning. A permanent pacemaker was inserted. She was anticoagulated with warfarin and discharged on postoperative day 12. Postoperative echocardiogram revealed a normal ejection fraction and no valvular abnormalities.
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Fig 1. Reconstruction of posterior left atrium with bovine pericardium and reconstruction of the posterior right atrium with an aortic homograft.
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After 7 months of gradual improvement the patient had progressive exertional dyspnea develop with peripheral edema resistant to diuretic therapy. Follow-up transesophageal echocardiogram suggested venous obstruction at the anastomoses of the pulmonary veins with the reconstructed atrium. A cardiac catheterization was performed that confirmed obstruction at the pulmonary vein orifices and at the inferior vena cava and right atrial junction with diastolic gradients of 20 mm Hg and 12 mm Hg, respectively. We returned her to the operating room and directly accessed the neo-left atrium through a right thoracotomy and right upper pulmonary vein. Four covered 8-mm stents were placed in the pulmonary ostia with fluoroscopic guidance (Fig 4). Thereafter the patient enjoyed clinical improvement for an additional 12 months, at which time she had dyspnea develop again. Repeat echocardiography suggested recurrent pulmonary vein stenosis. A transapical puncture was performed and recurrent pulmonary vein stenosis was diagnosed. The pulmonary vein stents were balloon dilated without difficulty, and the gradient dropped from 20 mm Hg to 4 mm Hg in the pulmonary vein and from 4 mm Hg to 1 mm Hg in the inferior vena cava. The patient is now able to walk 3 miles without difficulty.
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Comment
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While we have had experience with a similar approach to radical left atrial excision for tumor, we believe that this is the first report of cardiac autotransplantation with total bi-atrial excision and reconstruction for cardiac malignancy. The Carney complex is a syndrome consisting of cardiac myxomas, endocrine overactivity, and spotty pigmentation (lentiginosis) [1]. Recurrence of atrial myxomas is common (>30%) [2] in patients with the Carney complex, with some patients experiencing as many as 7 recurrences (J. A. Carney, personal communication). In this case, cardiac autotransplantation allowed easy ex-vivo resection of the tumor as well as essentially all atrial tissue, followed by construction of neo-atria with bovine pericardium. We faced the difficult problem of late pulmonary vein stenosis at the junction of the native pulmonary veins and the neo-posterior left atrium. This was treated by direct pulmonary vein access and stent deployment. This novel approach is a radical means to minimize the likelihood of tumor recurrence. Our patient remains free of tumor recurrence 3 years after operation. Future technical modifications will include attention to fashioning even larger neo-pulmonary vein orifices.
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References
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- Carney A, Gordon H, Carpenter P. The complex of myxomas, spotty pigmentation, and endocrine activity Medicine 1985;64:270-283.[Medline]
- Stergiopoulos S, Stratakis C. Human tumors associated with Carney complex and germline PRKAR1A mutations: a protein kinase disease FEBS Letters 2003:59-64.
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Abstract
Introduction
