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目的:总结婴幼儿原发性心脏肿瘤的诊断和治疗经验。方法:回顾性分析2013年8月至2019年2月大连医科大学附属大连市儿童医院心脏中心收治的7例疑似原发性心脏肿瘤婴幼儿的诊治过程，患儿均行超声心动图等检查明确诊断，根据肿瘤的大小和位置选择治疗方案。出院后行随访观察。结果:7例患儿均经超声心动图诊断为原发性心脏肿瘤，其中2例诊断合并结节性硬化症未行手术，5例选择手术治疗，其中1例手术患儿行自体心脏移植术切除肿瘤，1例术后出现心律失常，1例术后出现二尖瓣反流，再次手术纠正。其他患儿无并发症且均顺利出院。组织学检查显示横纹肌瘤4例，纤维瘤1例。随访2～66个月，手术患儿均无肿瘤复发，未手术的2例患儿心脏肿瘤有消退趋势。结论:婴幼儿原发性心脏肿瘤诊断首选超声心动图，该肿瘤组织学类型以良性横纹肌瘤为主，诊断过程中注意排除结节性硬化症。对于合并结节性硬化症的患儿可选择保守治疗，对于伴有明显症状的患儿应选择手术治疗，手术方案应根据肿瘤大小、位置及性质等选择，自体心脏移植术切除肿瘤是一个良好的手术治疗选择。“,”Objective:To summarize the experience in diagnosis and treatment of primary cardiac tumors in pediatric patients.Methods:Retrospectively analyzing 7 pediatric patients who were suspected as primary cardiac tumors and diagnosed and treated in Department of Heart Center, Children′s Hospital of Dalian Medical University from August 2013 to February 2019.All patients underwent echocardiography and other examinations, so as to confirm the diagnosis and the treatment plan was chosen based on the size and location of the tumor.All patients were followed up after discharge.Results:A total of 7 patients were diagnosed as primary cardiac tumors by echocardiography, among which 5 cases underwent surgical treatment, and 2 cases were diagnosed with tuberous sclerosis without surgery.In children undergoing surgery, 1 patient underwent autologous heart transplantation to remove the tumor, 1 patient had arrhythmia, 1 patient had mitral regurgitation after surgery, and the mitral regurgitation was corrected again.The remaining children had no adverse complications and were discharged successfully.Histologic examination revealed rhabdomyoma in 4 patients, and fibroma in 1 patient.The patients were followed up for 2-66 months after discharge, and no tumor recurrence was observed in the children who performed surgery.There was a trend of spontaneously regress of cardiac tumor in 2 patients without surgery.Conclusions:Echocardiography is the first choice for the diagnosis of primary cardiac tumors in Pediatric patients.Rhabdomyoma is the most prevalent histologic type of primary cardiac tumors, and tuberous sclerosis should be excluded during the diagnosis process.Patients with tuberous sclerosis selected conservative treatment, and surgical treatment was selected for children with obvious symptoms.According to the location and size of lesion, therapy strategies should be chosen and autologous heart transplantation can be adopted to remove the tumor for children with large tumors.Autologous heart transplantation to remove the tumor is a good surgical treatment.