Ann Thorac Surg 2009;88:e1-e2. doi:10.1016/j.athoracsur.2009.03.071
© 2009 The Society of Thoracic Surgeons
Case Reports
Reconstruction of Two Independent Neo-Atria After Resection of Recurrent Leiomyosarcoma
Sandro Sponga, MDa,*,
Adriano Rizzi, MDb,
Piersilvio Gerometta, MDa,
Gaetano Rocco, MD, FRCSEdc,
Vincenzo Arena, MDa
a Department of Cardiac Surgery, Bergamo, Italy
b Department of Thoracic Surgery, Humanitas Gavazzeni Hospital, Bergamo, Italy
c Department of Thoracic Surgery and Oncology, National Cancer Institute, Pascale Foundation, Naples, Italy
Accepted for publication March 20, 2009.
* Address correspondence to Dr Sponga, Department of Cardiac Surgery, Humanitas Gavazzeni Hospital, Via Mauro Gavazzeni 21, Bergamo, 24125, Italy (Email: sandro.sponga{at}unipd.it).
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Abstract
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We describe a case of a patient with recurrent leiomyosarcoma involving both atria after a previous right pneumonectomy. The patient was treated with wide resection of the mass and separate reconstruction of the cardiac cavities with prosthetic material.
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Introduction
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Malignant neoplasms involving cardiac cavities are rare. Therefore, the diagnosis is difficult and the surgical technique is not well standardized. Leiomyosarcomas originating in the pulmonary vein represent a very rare condition. Typically this tumor can be locally aggressive, making it difficult to obtain complete excision. As a consequence, pulmonary vein leiomyosarcomas may portend a dismal mean survival of 6 months [1–3]. Given the previously listed features, consensus of the treatment sequence of this neoplasm has not been reached. Advances in surgical technique and chemotherapy may improve the poor prognosis of these patients.
A 48-year-old woman was admitted to our hospital for dyspnea, constrictive chest pain, and night sweating. Previous history included a primary pulmonary low-grade leiomyosarcoma resected 12 years earlier through a right pneumonectomy. Since then, follow-up chest roentgenograms were negative for tumor recurrence.
The patient underwent a computed tomographic chest scan that revealed an 8 x 8 cm mass involving both atria and the residual right pleural cavity (Fig 1). These findings were confirmed by transesophageal echocardiography. Furthermore, the mass revealed increased metabolic activity on positron emission tomography. The endoscopic, ultrasound-guided, fine-needle aspiration biopsy was not diagnostic, although it was suspicious for malignancy.
The selected surgical approach was a median sternotomy. When the pericardium was opened, a sizeable white and ligneous mass, apparently originating from the stumps of the right pulmonary veins and protruding into the right pleural space was demonstrated (Fig 2).
Femoral venous cannulation and standard aortic cannulation allowed to obtain clean field around the atria and the tumor. Cardiopulmonary bypass was instituted and an apparent dissection plane between the tumor and the left atrium was identified. However, the interatrial septum was macroscopically infiltrated, thus preventing complete excision. To avoid venous blood drainage due to opening of the right atrium, a vacuum-assisted extracorporal circulation and Carpentier bi-caval femoral cannula 24-French (Medtronic Inc, Minneapolis, MN) were used. The superior vena cava was cannulated too. This technique permitted a bloodless operative field to avoid circulatory arrest and deep hypothermia.
With the patient on moderate hypothermia (27°C), aortic cross clamping was performed and cardioplegic arrest induced. Through a right atriotomy, it was possible to incise the interatrial septum and remove the tumor originating from the vascular stumps of the previously divided right pulmonary veins. On inspection, the mass infiltrated the right-sided aspects of both atria.
The tumor was enucleated along with the tissue of left atrium around the vascular stumps of the right pulmonary veins between the left pulmonary veins and the interatrial septum.
A significant portion of the interatrial septum and the lateral wall of the right atrium was also resected.
Care was taken at not damaging the coronary sinus, the atrioventricular valves, the sinus node, the atrioventricular node area and the left pulmonary veins.
Reconstruction appeared difficult, and therefore it was decided to create two independent atrial cavities. The left atrium was reconstructed with a patch of equine pericardium. The lower part of the pericardium was sutured to the residual left atrium bordering the left pulmonary veins and the mitral annulus, and the upper part to the residual interatrial septum paying attention at ensuring an adequate atrial cavity volume (Fig 3).
The right atrium was reconstructed by using a gelatine impregnated woven vascular prosthesis (Gelweave No. 20; Vascutek Ltd, Inchinnan Renfrewshire, Scotland) sutured to both venae cavae and then opened lengthwise. The inferior border of this prosthesis was sutured to the residual septum and the upper border to the residual right atrial appendage (Fig 4). Clamping time and cardiopulmonary bypass time were 143 and 166 minutes, respectively. Weaning from cardiopulmonary bypass was uncomplicated, yielding a spontaneous sinus rhythm. No inotropes were used.
The postoperative course was uneventful. An echocardiographic evaluation confirmed good left ventricular function, correct positioning of the prosthesis, without stenosis or thrombosis, and minimal pericardial effusion. On postoperative day 6, the Holter study showed sinus rhythm without ventricular or supraventricular arrhythmias. Final pathology showed a Coindre grade II leiomyosarcoma.
The patient remained asymptomatic without chest pain or dyspnea and was discharged on postoperative day 7. He was then referred to the oncologists to receive systemic chemotherapy.
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Comment
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Leiomyosarcoma is an aggressive tumor with a poor prognosis due to of the infiltration of the surrounding structures [1, 2]. As a consequence, cardiotomy or division of the aorta and pulmonary artery are necessary to achieve surgical radicality [2]. Combined resection of the left atrium and left lung through a thoracotomy or sternotomy has already been reported [2, 4]. Although the goal of surgery is usually palliative, complete resection has also been shown to be the only way of improving survival [3].
When the leiomyosarcoma invades the right side of the heart the prognostic outlook is invariably dismal [2]. In addition, the extensive infiltration of both atria makes surgical clearance difficult.
Bi-caval isolation, vacuum-assisted venous drainage, and the use of a Carpentier cannula, all enable good exposure. To warrant acceptable hemodynamic function and adequate hemostasis, and to avoid a restrictive physiology, reconstruction of two independent neo-atria is recommended.
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References
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- Shimono T, Yuasa H, Yuasa U, et al. Pulmonary leiomyosarcoma extending into the left atrium or pulmonary trunk; complete resection with cardiopulmonary bypass J Thorac Cardiovasc Surg 1998;2:460-461.
- Evans JB, Haw PM. Surgical clearance of invasive cardiac leiomyosarcoma with concomitant pneumonectomy Eur J Cardiothorac Surg 2003;24:843-846.[Abstract/Free Full Text]
- Poule GV, Meredith JW, Breyer RH, Mills SA. Surgical implications of malignant cardiac disease Ann Thorac Surg 1983;36:484-491.[Abstract/Free Full Text]
- Gyhra AS, Santander CK, Alarcon EC, Mucientes FH, Carrillo H. Leiomyosarcoma of the pulmonary veins with extension to the left atrium Ann Thorac Surg 1996;61:1840-1841.[Abstract/Free Full Text]
- Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac leiomyosarcomas Ann Thorac Surg 1991;6:999-1001.
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Abstract
Introduction
