We report the case of a 28-year-old male patient, active carabinero, with no relevant morbid history, who debuted on November 27, 2010 with syncope in the street.
The patient was admitted to the Hospital Institucional HOSCAR, where drainage was performed, with hemodynamic compromise and mediastinal widening in the chest X-ray confirming a cardiac tamponade by 2D echocardiography
A subsequent chest computed tomography (CT) study showed a solid tumor in the posterior and lateral area of the left ventricle (LV).
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On November 30, 2010 a thoracic magnetic resonance imaging (MRI) was performed, which showed a solid tumor lesion, in relation to the left atrioventricular groove, of approximately 9.3 x 6 x 5 cm in the left ventricle extensive contact.
The pulmonary veins displaced by the tumor mass, but permeable, the trunk of the pulmonary artery and its left branch also with close contact, may be located.
He also failed to control the fatty planes surrounding the involved circumflex artery.
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The patient underwent dissemination study with positron emission tomography (PET) which was negative and CT of the abdomen and pelvis that were normal.
On December 14, 2010 the patient underwent a midline laparotomy and conventional cardiopulmonary bypass, by arterial cannulation in the ascending aorta and bicaval venous cannulation.
The exploration of the heart revealed a large tumor located in the posterior and lateral regions of the left ventricle, which was unresectable with the heart in situ. For this reason, we proceeded to perform a cardiac explantation, with cardiac precaution
The examination of the heart revealed stenosis of the pulmonary artery and its left branch, but without stenosis of the left atrium, the circumflex artery and the mitral annulus.
With the heart on the operating table, the tumor was resected with a left ventricular wall margin, including in the surgical specimen the circumflex artery from birth, compromised mitral annulus and left atrial wall.
Subsequently, left ventricular wall and left atrium were repaired with bovine pericardium pretracted in glutaraldehyde and a mechanical mitral prosthesis was implanted Saint Jude® # 33.
Then, the heart was reimplanted in the same sequence of a conventional cardiac transplant with a bicaval technique.
Finally, two bypasses of the saphenous vein were performed at the distal circumflex territory.
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The rapid biopsy revealed a primary fusocellular sarcoma of the myocardium highly suggestive of rabies sarcoma and the delayed biopsy confirmed a primary embryonal rhabosarcoma of the myocardium.
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The postoperative evolution was satisfactory, presenting only low output syndrome that required transient use of inotropes, being discharged 16 days after surgery.
On January 23, 2011 began the first cycle of chemotherapy with PINDA protocol, Group 4, with doxorubicin, doxorubicin-doxyl (+)-vincristine plus cardioxan as cardiorubicin toxicity.
In April 2011, a tumor mass was researched in the left thigh. A subcutaneous metastasis of the primary sarcoma was diagnosed, without histological confirmation, so she was treated with radiotherapy plus chemotherapy with vincristine plus irine.
In August 2011, the patient post-chemotherapy cycle suffered respiratory distress secondary to pneumonia and acute renal failure, requiring hospitalization in the intensive care unit, use of mechanical ventilation, broad-spectrum intravenous antibiotics and transient hemodialysis, being new.
The patient has had echocardiographic follow-up and CT that ruled out local recurrence and recovery of left ventricular systolic function with ejection fraction VI of 57%.
At nine months of follow-up, the patient is in good condition and functional capacity II NYHA.
