心脏血管肉瘤的临床及超声心动图特征

Clinical and echocardiographic features of cardiac angiosarcoma

目的探讨原发性心脏血管肉瘤的临床资料及超声心动图特点,以期为临床诊疗提供策略和依据。方法收集首都医科大学附属北京安贞医院2009年1月至2021年1月经病理证实的12例原发性心脏血管肉瘤患者的临床资料和超声心动图表现,包括一般资料、临床症状、超声心动图特点、临床检验结果、病理学特征以及治疗和预后,并进行回顾性分析。结果12例心脏血管肉瘤患者,男性8例,女性4例,平均年龄(44±15)岁,6例为局限性血管肉瘤,6例存在远处转移。其中10例超声心动图描述为心脏占位,均位于右心腔,基底较宽;其余2例,诊断为心包血管肉瘤,超声心动图表现为孤立性大量心包积液,未发现明确占位。心包受累是常见并发症,6例(6/12,50%)出现心包积液,1例患者心包积液细胞学检查为阴性。12例左、右心室功能均正常。部分患者血流动力学异常,三尖瓣口梗阻3例(3/12,25%),上腔静脉口阻塞2例(2/12,17%),另2例因肿瘤压迫出现三尖瓣后叶无力;右心室基底部运动不良1例。结论超声心动图对心腔内肿物检出较敏感,心脏血管肉瘤好发于右侧心腔,易侵犯邻近结构,以心包积液最常见。心脏血管肉瘤的超声心动图特征仍需进一步探索和明确。

Objective To investigate the clinical and echocardiographic manifestations of primary cardiac angiosarcoma(CA)in order to provide a basis for its clinical diagnosis and treatment.Methods The clinical data and echocardiographic features,including the tumor location and adjacent infiltration,of 12 cases of CA pathologically diagnosed at Anzhen Hospital from 2009 to 2021 were reviewed retrospectively.Results Twelve patients with CA were identified,including eight males and four females,with an average age of(44±15)years old.Of the 12 individuals included,six had distant metastases and six had localized CA.Ten of them were described by echocardiography as having cardiac space-occupying lesions,all located in the right heart cavity with a wide base;the remaining two cases were diagnosed with pericardial angiosarcoma,which showed an isolated massive pericardial effusion on echocardiography and no clear space-occupying lesion was found.Pericardial involvement was a common complication;six cases(6/12,50%)presented with pericardial effusion,and one patient had a negative pericardial effusion cytology.Both the left and right ventricles were functioning normally in all the 12 cases.Some patients had abnormal hemodynamics,three cases(3/12,25%)had tricuspid orifice obstruction,two(2/12,17%)had superior vena cava orifice obstruction,and another two had poor motion of posterior leaflet of tricuspid valve due to tumor compression.One case had right ventricular basal dyskinesia.Conclusion Echocardiography is sensitive in detecting intra-cardiac masses.CA tends to occur in the right side of the heart chambers and is prone to invade adjacent structures,with pericardial effusion being the most common.The echocardiographic features of CA remain to be further explored and determined.