心脏淀粉样变误诊为原发性肥厚型心肌病11例临床分析  

The analysis of 11 cases with cardiac amyioidosis misdignosised as idiopathic hypertrophic cardio-myopathy

作　　者：时向民[1] 王玉堂[1] 杨庭树[1] 

机构地区：[1]解放军总医院南楼心内科,北京100853

出　　处：《临床内科杂志》2012年第2期112-114,共3页Journal of Clinical Internal Medicine

摘　　要：目的分析11例心脏淀粉样变误诊为原发性肥厚型心肌病患者的临床特征，探讨误诊原因及诊断要点。方法1l例患者均为男性，平均年龄（56．3±21．5）岁，在外院诊断为原发性肥厚型心肌病，通过分析患者心电图、超声心动图和心力衰竭的特征及伴随的。肾脏、肝脏、呼吸系统及甲状腺受累的临床特点及相关实验室检查，分析心脏淀粉样变与原发性肥厚型心肌病的鉴别要点。结果所有患者均表现为进行性充血性心力衰竭，超声心动图显示左心室肥厚伴颗粒样闪光现象，6例患者心电图肢导联QRS波低电压，5例患者V1-V4导联呈Qs型，所有患者心电图电压（SV5＋RV1）与左室横截面积比值〈1．0，5例患者伴心房静止。患者均伴有蛋白尿，7例患者表现为肾病综合征，6例患者伴有肝肿大，4例患者伴呼吸睡眠暂停低通气综合征、原发性甲状腺功能减低及皮肤紫癜。腹壁脂肪活检及刚果红染色提示淀粉样变性。结论心脏淀粉样变有其特有的心电图及超声心动图改变，同时多伴有其他系统受累的临床表现，是其与原发性肥厚型心肌病鉴别的关键。Objective To analyze the clinical characters of 11 patients with cardiac amyloidosis （CAL） who were misdiagnosised as idiopathic hypertrophic cardiomyopathy（ IHC）, and to investigate the reason of misdiagnosis and the key points of diagnosis. Methods 11 male patients were diagnosised as IHC in other hospital, mean age 56.3 ＋ 21.5, clinical data including electrocardiogram（ ECG）, ultrasonic cardiogram （ UCG）, character of heart failure, manifestation of involved renal,liver, thyroid, respiratory sys- tem and relevant laboratory test were investigated. Results All patients manifested progressive congestive heart failure, as well as hypertrophy of left ventricular with the phenomenon ＂granular sparkling＂ was de- tected by UCG. 6 cases with low voltage of QRS in limb leads and 5 cases presented QS in V1-V4 leads of ECG, the parameter（ SV5 ＋ RV1 ）/CSA 〈 1 was positive in all patients,5 cases presented atrial stand- still. 11 cases were detected with proteinuria and 7 cases were confirmed with nephrotic syndrome,6 pa- tients suffered from hepatomegaly, 4 cases contracted sleep apnea syndrome （ SAS）, in addition, 4 cases with the diagnosis of idiopathic hypothyroidism and skin purpura, biopsy of fat tissue in abdominal wall and congo red stain confirmed the diagnosis of amydosis. Conclusion Patients with CAL had unique clin- ical characters in ECG and UCG, simultaneously other system could get involved, which could be the key points for differentiation between CAL and IHC.

关 键 词：心脏淀粉样变 肥厚型心肌病 

分 类 号：R541[医药卫生—心血管疾病]