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目的探讨心肌致密化不全患者的临床特征。方法回顾分析了18例心肌致密化不全患者的临床表现、心电图、超声心动图等资料,随访(11±5)个月。结果心肌致密化不全占同期确诊的各类原发性心肌病的8.1%。发病年龄1.5～71岁,男性占66.7%,家族性发病2例。14例患者表现为程度不等的心力衰竭,2例患者因心律失常表现为黑矇、晕厥,1例患者发生血栓栓塞,1例患者无症状。心电图均有异常,室性期前收缩7例,心脏传导阻滞和心房颤动各4例。所有患者均通过心脏超声检查确诊,其中17例患者病变局限于左心室,1例局限于右心室,心尖部最常受累(72%),收缩末期非致密化心肌层/致密化心肌层2.3～3.1,15例患者左室射血分数<50%,室壁弥漫性运动障碍。随诊期间13例患者反复出现心力衰竭,1例患者于确诊后16个月发生心脏性猝死,1例患者因顽固性心力衰竭于确诊后10个月接受心脏移植,1例患者因药物难以控制的心律失常置入埋藏式自动心脏复律除颤器,口服抗凝药的患者未再发血栓栓塞。结论心肌致密化不全的病情迁延,男性多见,有家族遗传倾向,在原发性心肌病中并不罕见。临床表现主要是心力衰竭、心律失常、血栓栓塞。心脏超声为确诊的首选检查。预后较差,顽固性心力衰竭和致死性心律失常是主要死因,心脏移植、置人埋藏式自动心脏复律除颤器能降低致死(残)率,口服抗凝药能减少血栓栓塞的发生。
Objective To investigate the clinical features of patients with myocardial densification. Methods The clinical manifestations, electrocardiogram (ECG) and echocardiography were retrospectively analyzed in 18 cases of patients with myocardial densification. The follow-up was (11 ± 5) months. Results Myocardial densification incomplete accounting for the same period identified all kinds of primary cardiomyopathy 8.1%. Age 1.5 to 71 years of age, 66.7% of men, familial onset in 2 cases. 14 patients showed varying degrees of heart failure, 2 patients showed arrhythmia due to amaurosis, syncope, 1 patient thromboembolism, 1 patient asymptomatic. ECG abnormalities, ventricular contraction in 7 cases, heart block and atrial fibrillation in 4 cases. All patients were diagnosed by echocardiography, of which 17 patients were limited to the left ventricle, 1 to the right ventricle, most often to the apical portion (72%), 2.3 to 2.3% in the non-demyelinating myocardium / densified myocardium 3.1,15 patients with left ventricular ejection fraction <50%, wall diffuse motor dysfunction. During follow-up, 13 patients experienced recurrent heart failure, 1 patient developed sudden cardiac death 16 months after diagnosis, 1 patient underwent heart transplantation 10 months after diagnosis due to refractory heart failure, and 1 patient was refractory to medication Of cardiac arrhythmias were placed in a buried AED cardiograph and patients without oral anticoagulation had no further thromboembolism. Conclusions Myocardial densification is a persistent condition that is more common in men and has a family genetic predisposition. It is not uncommon in primary cardiomyopathy. The main clinical manifestations of heart failure, arrhythmia, thromboembolism. Echocardiography is the first choice of diagnosis. Poor prognosis, refractory heart failure and fatal arrhythmia are the main causes of death. Cardiac transplantation, placement of buried automatic cardioverter-defibrillator can reduce the rate of death (residual), oral anticoagulants can reduce the occurrence of thromboembolism.