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目的探讨小儿先天性心脏病(CHD)伴心内膜弹力纤维增生症(EFE)和心肌致密化不全(NVM)的临床表现、诊断及治疗效果,以提高对该病的认识和诊疗水平。方法对2005年5月-2008年1月本院收治的6例CHD伴EFE和NVM患儿的临床表现、胸片、心电图、超声心动图及临床治疗情况进行分析。CHD伴EFE和NVM的诊断依据超声心动图表现,治疗方案以CHD及EFE的治疗方案为主。结果3例行手术治疗[行动脉导管未闭(PDA)结扎术1例,主动脉弓成形术2例(其中1例同时行PDA结扎术)]。5例(包括手术治疗的3例)在心功能不全和心律失常控制且稳定后好转出院;1例心力衰竭严重者自动出院。结论超声心动图对CHD伴EFE和NVM的诊断和鉴别诊断有重要价值;可通过手术治疗、强心、利尿、降低心脏前后负荷及免疫抑制治疗使该类疾病临床症状得到缓解。
Objective To investigate the clinical manifestations, diagnosis and treatment of children with congenital heart disease (CHD) with endocardial fibroelastosis (EFE) and myocardial degeneration (NVM) in order to improve their understanding and diagnosis of the disease. Methods The clinical manifestations, chest X-ray, electrocardiogram, echocardiography and clinical treatment of 6 CHD patients with EFE and NVM admitted to our hospital from May 2005 to January 2008 were analyzed. CHD with EFE and NVM diagnosis based on echocardiography, treatment options to CHD and EFE treatment based. Results Surgical treatment was performed in 3 cases [1 case of patent ductus arteriosus (PDA) ligation and 2 cases of aortic arch angioplasty (1 cases underwent concurrent PDA ligation)]. Five cases (including 3 cases of surgical treatment) were discharged after stable heart failure and arrhythmia control. One case of severe heart failure was discharged automatically. Conclusions Echocardiography is of great value in the diagnosis and differential diagnosis of CHD with EFE and NVM. The clinical symptoms of these diseases can be alleviated by surgery, cardiac, diuretic, cardiac load reduction and immunosuppressive therapy.