Ex situ resection of recurrent left atrial sarcoma

Introduction: Cardiac sarcomas are a rare entity with poor prognosis. Only 10% of surgically resected heart tumors are primary sarcomas. Survival after surgical resection ranges from seven to forty seven months.

Aims: We report on a 31 year old female patient who had undergone surgical resection of a left atrial tumor in march 2011 in another heart surgery center. It had not been possible to resect the entire tumor mass, because it was adjacent to the back wall of the left atrium (LA). Nevertheless she recovered well from surgery. Histology revealed myxomatous tissue, but also tumor cells of grade 2–3 sarcoma. With this background she was referred to our center for radical resection of cardiac sarcoma in August 2011.

For preoperative staging and planning of the surgical procedures PET CT scan and computed tomography of the heart were performed, showing tumor infiltration of the posterior mitral leaflet, the left upper pulmonary vein and close neighborhood to the circumflex artery. Surgical access was gained via median sternotomy. The tumor infiltrated the superior caval vein (SCV), the interatrial septum, the back wall of the LA and the posterior mitral leaflet. In order to radically resect the extensive tumor mass ex situ surgery had to be performed. The heart was excised, including the complete LA, remaining only the apertures of the pulmonary veins. Ex situ preparation consisted in resection of the tumor-carrying mitral leaflet, the LA and the interatrial septum. After reconstruction of the annulus the mitral valve was replaced by a 25mm biological prosthesis. The LA and the SCV were reconstructed using bovine and textile prosthetic material. Thereafter the tumor-free heart was reimplanted. Postoperative course was uneventful; the patient was referred to the normal warden on postoperative day (POD) four. On POD thirteen she left the hospital. Histology revealed grade 1–2 sarcoma. Further treatment will include adjuvant chemotherapy and in case of local recurrence high dose radiation therapy.

Discussion: As treatment options for sarcoma of the heart, radical resection and heart transplantation have been described. With scarce donor organs and long waiting times, heart transplantation is no option in tumor patients with limited life expectancy of a few months without surgical treatment. Radical resection, including extensive surgical procedures like ex situ resection and autotransplantation remain as the only treatment option for those patients.