Primary malignant cardiac tumors, usually sarcomas, represent an infrequent subgroup among cardiac masses. However, they constitute processes with poor prognosis, and surgical treatment is the most favorable therapeutic alternative in terms of survival.

The literature regarding left-sided cardiac sarcomas reveals that patients are subjected to reinterventions for local recurrence, generally related to incomplete resections, probably due to a suboptimal anatomical exposure during surgery, which conditions inadequate resections and technically difficult recon structions.

Sometimes, to fulfill the objectives of a radical oncological resection and facilitate the reconstruction of the resected cardiac structures, it is necessary to explant the heart in order to resect the tumor with adequate margins and reconstruct the cavities or in volved structures, finally reimplanting the heart in bench surgery (cardiac autotransplantation).

This is the case is a female 73-year-old patient, without relevant clinical history who was admitted to hospital due to progressive dyspnea and anemia. In the diagnostic algorithm, the transthoracic echocardiogram showed a dilated left atrium occupied by a 4.8 cm × 2.8 cm immobile, heterogeneous mass, in timately associated with the mitral annulus, which completely filled the left atrial appendage, and severe mitral valve insufficiency with central jet.

A cardiac magnetic resonance performed to com plete the mass evaluation revealed the mentioned heterogeneous tumor in weighted T1 and T2 sequences, before and after contrast, as well as in perfusion and late enhancement sequences. No contrast capture was evidenced in the tumor sector protruding to the left atrium, which was interpreted as an added thrombotic component (Figure 1). The same study showed absence of pericardial and pulmonary vein involvement. Prophylactic anticoagulation was started, and a positron-emission computed tomography (PET-CT) of the whole body was performed for local evaluation and search of eventual metastasis.

Fig. 1 A. Nuclear magnetic resonance: left atrial sarcoma located in the left atrial appendage. B. Surgical specimen with complete lesion resection. The resection margins are evidenced on tissue not affected by the tumor. 

The PET study showed a hypermetabolic mass of 5.9 cm × 3.4 cm × 2.4 cm (SUV 8.5) in the already known location, focal liver lesions compatible with he mosiderosis and absence of secondaries.

A surgical treatment was decided due to the con dition and clinical characteristics of the patient, disease staging and prognosis without resection. Owing to the location of the lesion to resect, in close contact with the mitral annulus, the circumflex artery and the coronary sinus, it was inferred that to perform an ad equate oncological resection, the heart should be explanted and reconstructed in bench surgery (ex situ) with subsequent autotransplantation.

Surgery was performed with midline sternotomy and cannulation of both venae cava and aorta

The tumor was explored entering the left atrium by the interatrial sulcus as usually performed for a mitral valve procedure. Absence of pulmonary vein in volvement and tumor growth up to the vicinity of the mitral annulus were verified.

Considering that the oncological resection would involve resecting the mitral annulus and part of the mitral valve, and faced with the difficulty to define, through the mentioned approach, the external margin of the resection in relation to the interventricular sulcus structures, it was decided to explant the heart and perform a bench tumor resection.

The venae cava, aorta and pulmonary artery were sectioned and the atriotomy was extended leaving a hood that contained the pulmonary veins. The tumor was resected ex situ (bench surgery), which implied resecting a section of the mitral annulus at the P1 level exposing the atrioventricular sulcus vessels and the ventricular myocardium. The mitral annulus and the left atrium were reconstructed with a bovine pericardial patch and the mitral valve was replaced with a #25 porcine biological prosthesis.

The organ was reimplanted with autotransplanta tion technique (Figure 2).

Fig. 2 Cardiac autotransplantation showing the left atrium re paired with bovine pericardium and mitral prosthesis 

Extracorporeal circulation time was 232 min and cross-clamping time 175 min. The postopera tive course was in accordance with the magnitude of the procedure, requiring inotropic support for 72 h. Among other events, the patient presented an episode of atrial flutter which was controlled with amiodarone and isolated subfebrile records with negative cultures.

Anatomical pathology reported a grade III undif ferentiated pleomorphic sarcoma, which implies a maximum level of malignancy and undifferentiation.

The prevalence of primary cardiac tumors in au topsy series is 0.02%. Among them, 25 % are malignant and 75 % of these are sarcomas. ¹ Median survival in published series ranges between 9 and 33 months. ² Most are clinically silent until a very ad vanced stage and are often considered nonresectable due to the proximity to critical structures. However, surgical and imaging techniques have improved allowing more aggressive interventions, which aim to achieve a microscopically negative resection (R0), a situation in which there is clear benefit of survival. ³

Cardiac autotransplantation is a procedure described many years ago for the resection of tumors with difficult approach or complex intraoperative management. ⁴

Along time, the technique was reproduced for the management of this pathology in numerous patients, ⁵ and the initial results improved in terms of quality of the oncological resection and survival. ⁶

With adequate surgical training the technique is reproducible and should be considered a valuable alternative in the therapeutic arsenal to offer opportu nities to patients with severe oncological disease and poor prognosis without surgery.