心肌致密化不全患者临床及预后分析

目的分析心肌致密化不全(NVM)患者的临床特点及预后。方法回顾性分析2002年9月～2010年2月在苏州大学附属第一医院就诊的34例NVM患者的临床表现、心电图、超声心动图、治疗过程等资料,并进行随访。结果 34例患者中,家族性患病者2例,14例曾被误诊,临床表现为心功能不全者29例,发现心律失常者31例,发生血栓栓塞3例,无症状者3例。所有患者均行心电图和超声心动图检查,1例行心脏磁共振检查。26例患者接受了随访,平均随访31.46个月,随访期间,3例患者死亡,2例经冠脉行自体干细胞移植术,1例因合并冠状动脉畸形至外科手术矫治,1例因合并严重二尖瓣狭窄行二尖瓣机械瓣置换术,16例因心力衰竭需反复住院治疗。结论心肌致密化不全患者临床表现个体差异较大,预后较差,超声心动图检查是最重要的筛查和确诊手段。

Clinical and prognosis analysis of the patients with noncompaction of ventricular myocardium

Aim To analyze the clinical characters and prognosis of patients with noncompaction of ventricular myocardium.Methods The clinical manifestations,electrocardiogram,echocardiogram and course of treatment of 34 patients with noncompaction of ventricular myocardium in our hospital,were retrospectively analyzed,and they all received follow-up visit.Results Among all the patients,2 sufferers had family history,14 misdiagnosis,29 cardiac insufficiency,31 arrhythmia,3 embolism,3 no symptoms.And all 34 patients received ECG and UCG,one of whom had magnetic resonance.26 patients had follow-up visit of averagely 31.46 months,and in this period,3 patients died,2 having autogeneic peripheral stem cells transplantation by coronary artery,1 having surgical diorthosis because of combined with malformation of coronary artery,1 having mitral valve replacement because of severe mitral stenosis,16 hospitalized repeatedly because of heart failure.Conclusions There were evident individual difference about clinical characters of patients with noncompaction of ventricular myocardium,and the prognosis was bad.UCG was an important instrument of screening and final diagnosis.