心肌致密化不全患者的临床特点及预后

目的:通过分析心肌致密化不全患者的临床特点、诊断方法、治疗及预后,以提高临床诊治水平。方法:分析及随访2000年1月～2006年4月住院治疗的17例心肌致密化不全患者的临床资料、治疗及预后。结果:心肌致密化不全特征为海绵状心肌,多发生于左心室。临床表现主要为心力衰竭(纽约心功能分级Ⅱ～Ⅳ级者占88.2%)、心律失常及血栓形成(分别为88.2%与11.8%)。17例中14例为孤立性心肌致密化不全,3例合并其他心血管疾患。17例患者均行超声心动图及心脏磁共振检查,其中13例患者的超声心动图及心脏磁共振检查均明确诊断,另4例经心脏磁共振检查确诊。随访10例患者,3例行心脏移植手术,1例死亡。结论:心肌致密化不全临床表现各异,预后差。超声心动图检查是诊断主要方法,心脏磁共振检查有助于提高诊断水平。

Clinical Features and Prognosis in 17 Patients With Noncompaction of the Ventricular Myocardium

Objective:To investigate the clinical features,treatment and prognosis of the ventricular noncompaction in 17 patients in or- der to improve diagnosis and treatment. Methods:We analyzed clinical datum and followed up treatment and prognosis in 17 patients with the ventricular noncompac- tion from January 2000 to April 2006. Results:The most common presentations were congestive heart failure(88.2%),arrhythmias(88.2%)and thrombosis (11.8%).The noncompaction of ventricular myocardium(NVM)was isolated,or occurred with other cardiovascular malforma- tion.Eehocardiography was a main method for diagnosis. Conclusion:NVM has a long-term clinical course and diversity manifestations and usually has a poor progno- sis.Echocardiography is a principal means of diagnosis.Magnetic resonance imaging can contribute to the diagnosis.