目的　探讨心脏K-M综合征的临床特点、诊断及治疗。方法　回顾分析1例心脏K-M综合征患儿的临床 资料，并复习相关文献。结果　患儿，男，出生33天。表现为呼吸急促、颈静脉充盈、心音低顿等。彩色多普勒超声心动 图及胸部CT提示大量心包积液。血常规显示血小板计数下降。急诊行心包部分切除引流术，升主动脉根部、上腔静脉、 右心耳上缘范围可见6 cm×5 cm硬质包块，固定。右室流出道及肺动脉处见长条形凸起包块，质硬，固定。右心室表面 部分瘤体及左侧壁层心包病理显示为Kaposi血管内皮瘤。确诊为心脏K-M综合征。给予类固醇激素治疗。结论　心脏 K-M综合征是一种罕见疾病，主要根据临床表现、影像学及病理学检查诊断，可给予口服类固醇激素等治疗，必要时手 术治疗。

　Objective　To the clinical characteristics, diagnosis and treatment of cardiac K-M syndrome. Method　The clinical data of cardiac Kasabach-Merritt syndrome (K-M syndrome) in an infant were retrospectively analyzed and the related literature was reviewed. Results　A 33-day-old boy presented with polypnea, jugular venous distention and low cardiac sound. Color doppler echocardiography and chest CT indicated massive pericardial effusion. Blood routine showed a decrease in platelet count. Patient was treated with acute partial resection of pericardium and drainage. Intraoperative findings included a 6 cm × 5 cm fixed firm mass on the surface of ascending aorta root, superior vena cava, the upper edge of right atrial appendage, and long strip protruding hard fixed mass seen in the right ventricular outflow tract and main pulmonary artery. Pathological findings in partial tumor of right ventricular surface and parietal pericardium showed Kaposi's hemangioendothelioma. The infant was diagnosed with cardiac K-M syndrome. The steroid therapy was administered. Conclusion　Cardiac K-M syndrome is a rare disease and is primarily diagnosed by clinical manifestations, imaging and pathological examination. It can be treated by oral steroid hormones and surgery if necessary.