Right sided reconstruction of the heart for invasive angiosarcoma of the right atrium

Abstract

Introduction Primary cardiac tumors are a rare entity (0.0017 to 0.033%, based on autopies). Most cardiac tumors are metastastic locations of other primary tumors. About 75% of the primary cardiac tumors are benign, and 75% of these benign tumors are myxomas. The remaining 25% of the primary tumors of the heart are malignant, with a majority of sarcomas (75%). Median survival for cardiac sarcomas is only six months. A 35-year-old male presented with new diagnosis of cardiac angiosarcoma. We describe the case, diagnosis, treatment modalities and first observations concerning survival. Case description A 35-year-old male presented with palpitations and progressive dyspnea since five weeks. ECG showed atrial fibrillation and transthoracic echocardiography (TTE) demonstrated a large right intra-atrial mass (7.8x5.2cm), infiltrating the interatrial septum and the tricuspid valve. There were no signs of right ventricular inflow obstruction. TEE and cardiac MRI confirmed a large intra-atrial mass (59x49x68 mm) and because of the density, the irregular shape and the localization, an angiosarcoma was suspected (Figure 1). In addition infiltration of the pericardium and pericardial effusion were found. A FDG-PET/CT scan did not show signs of metastatic disease. Coronary angiography showed normal coronary arteries. Results and conclusion After fourteen days, a resection was performed under general anesthesia and full extracorporeal circulation (Figure 2). A reconstruction of the fibrous part of the septum and tricuspid annulus allowed implantation of a tricuspid bioprosthesis (Mosaic 33). Right and left atrium and the basal part of the right ventricle were reconstructed using pericardial patches. The vena cava superior was reconstructed using a PTFE graft. CABG was performed on branches of the right coronary artery using a saphenous vein graft. A permanent pacemaker was implanted after ten days because of persistent total AV-block. Patient recovered uneventfully of the intervention. Take-home message Pathologic examination showed a classic angiosarcoma, with resection margins free of tumor. CD31 and CD34 immunohistochemical staining was positive, confirming vascular differentiation and the diagnosis of an angiosarcoma (Figure 3). After multidisciplinary consultation between oncologists, pathologists, radiologists and cardiac surgeons an intensive adjuvant chemotherapy treatment was initiated. Up until the moment of evaluation there is a survival of six months, with a NYHA classification I and with no arguments for metastatic liver disease on PET-CT. We therefore believe that a radical and aggressive resection combined with optimal medical treatment can improve survival in this devastating disease.