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目的探讨多脾综合征患者合并心脏畸形的特点,为超声诊断及外科治疗提供思路。方法采用超声心动图显示45例多脾综合征患者心脏畸形并分析其特点,回顾分析34例多脾综合征患者外科手术方式特点。结果 45例多脾综合征患者均存在房间隔发育异常;40例(88.9%)合并室问隔发育异常;房室瓣发育异常发生率较高,共25例(55.6%);21例(46.7%)患者存在动脉-心室连接异常;32例(71.1%)患者存在右室流出道及肺动脉发育异常;39例(86.7%)存在体静脉及肺静脉引流异常。34例患者接受外科手术,包括双心室矫治及Fontan类手术。结论多脾综合征多合并复杂心脏畸形,超声心动图可以较准确诊断多脾综合征及其心脏畸形情况,决策外科手术方式。
Objective To investigate the characteristics of patients with polysplex syndrome complicated with cardiac malformations and to provide ideas for ultrasonic diagnosis and surgical treatment. Methods Echocardiography was used to show the cardiac malformations in 45 patients with splenomegaly and its characteristics were analyzed retrospectively. The surgical characteristics of 34 patients with polysplex syndrome were retrospectively analyzed. Results Forty-five cases (88.9%) had dysplasia of atrial septum. Forty-five cases (55.6%) had abnormal atrioventricular valve dysplasia. Twenty-one cases (46.7% %) Patients had abnormal arterial-ventricular connections; 32 patients (71.1%) had right ventricular outflow tract and pulmonary artery dysplasia; 39 patients (86.7%) had abnormalities of venous and pulmonary venous drainage. Thirty-four patients underwent surgery, including biventricular surgery and Fontan class surgery. Conclusion Multiple splenic syndrome complicated with complicated cardiac malformation, echocardiography can be more accurate diagnosis of splenic syndrome and cardiac deformity, decision-making surgical approach.