Surgical resection of a primary cardiac angiosarcoma and reconstruction of involved right atrium

Primary cardiac angiosarcoma is an extremely rare malignant neoplasm, typically showing terrible prognosis, the gold standard treatment is surgical resection. We present a case of a 41-year-old woman who was diagnosed with a large right atrium, presenting with complaints of chest pain and shortness of breath for 2 weeks. Multimodality imaging results suggest a cardiac angiosarcoma. The patient underwent successful radical resection and single-stage reconstruction of the right atrium and superior vena cava under normothermic cardiopulmonary bypass with a beating heart. Histopathology confirmed the diagnosis of primary cardiac angiosarcoma. Based on the genetic results, tiragolumab was used for postoperative immunotherapy. Fortunately, no evidence of recurrence of angiosarcoma was showed 4 year after surgery. Radical surgical resection with reconstruction of the resected heart structures was the only possible salvage option for giant angiosarcoma, which led to hemodynamic instability. Followed by chemotherapy, this radical approach may prolong survival.