18例心肌致密化不全患者的临床特征  

Clinical characterization and outcome of patients with noncompaction of ventricular myocardium

作　　者：何涛[1] 曾和松[1] 乐伟波[1] 李小欢[1] 陆再英 

机构地区：[1]华中科技大学同济医学院附属同济医院心血管内科,武汉430030

出　　处：《中华心血管病杂志》2007年第6期548-551,共4页Chinese Journal of Cardiology

摘　　要：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 analyze the clinical features and outcome of patients with noncompaction of ventricular myocardium （NVM）. Methods Clinical manifestations, electrocardiograms and echocardiographies data were analyzed in 18 patients with NVM. Mean follow-up period was （ 11 ＋ 5 ） months. Results The patients aged from 1.5 to 71 years, 66. 7% patients were males, familial history was observed in 2 cases, congestive heart failure was present in 14 cases, thromboembolic event occurred in 1 patient, arrhythmia induced syncopes were diagnosed in 2 patients and 1 patient was asymptomatic. Abnormal electrocardiograms were observed in all patients, including premature ventricular beats （7 cases） , heart block （4 cases）, and atrial fibrillations （4 cases）. Echocardiographies showed that noncompaction of ventricular myocardium localized in the left ventricle in 17 patients, and right ventricle in 1 patient. The extension of noncompaction myocardium was predominantly at the apex （72%）. N/C was 2. 3-3. 1. EF was less than 50% in 15 patients. Hypokinetic movements were observed in both noncompacted and compacted segments. During the follow-up, 1 patient with congestive heart failure received heart transplantation. ICD was implanted in one patient due to ventricular tachycardia. One patient suffered from sudden cardiac death. Conclusions The most common clinical presentations of NVM are congestive heart failure, cardiac arrhythmias, and thromboembolism. Echocardiography is considered as the best tool for the diagnosis of NVM. ICD, heart transplantation and anticoagulation therapy could improve the prognosis of patients with NVM in selected cases.

关 键 词：心肌疾病 心力衰竭 充血性 心律失常 血栓栓塞 心肌致密化不全 

分 类 号：R541.1[医药卫生—心血管疾病]