目的:观察分时段采用不同中药方剂治疗缺血性心肌病气虚血瘀阻络型心衰的远期临床疗效.方法:将缺血性心肌病气虚血瘀阻络型心衰患者120例按照随机对照原则分为治疗组(心肌活力饮、冠脉疏通饮+西药治疗)和对照组(西药治疗)各60例,治疗4周后评估疗效,随访3个月比较两组患者心血管事件发生率及生活质量.结果:总有效率治疗组为90.00％,高于对照组的66.67％;治疗组心衰分级轻度占63.33％,高于对照组的33.33％;治疗组心血管事件总发生率为6.67％,低于对照组的20.00％(P<0.05).治疗组躯体健康(physical component summary,PCS)总评分与心理健康(mental component summary,MCS)总评分均高于对照组(P<0.05).结论:根据人体阴阳消长,早晨服用心肌活力饮益气解毒,晚上服用冠脉疏通饮活血通络能降低缺血性心肌病气虚血瘀阻络型心衰患者心血管事件发生率,帮助提高患者生活质量.

目的 研究益气活血解毒法治疗酒精性心肌病的心功能疗效.方法 方便选择2015年3月—2018年3月心血管内科收入治疗的酒精性心肌病患者68例进行调研,按照治疗方法的不同分为两组,各34例,对照组采用常规西药治疗,观察组在对照组基础上加用自拟益气活血解毒法治疗;治疗1个疗程后比较两组心功能恢复情况以及疗效.结果 观察组治疗总有效率88.24％高于对照组70.59％,差异有统计学意义(χ2=8.822,P=0.003<0.05);两组治疗后左心室功能均恢复优于治疗前,但观察组治疗后LVEF(50.17±7.46)％高于对照组(42.57±6.89)％(t=4.364,P=0.000),LVEDD(53.73±4.64)mm、LVESD(39.86±3.71)mm低于对照组(58.87±4.95)mm、(43.19±3.64)mm,差异有统计学意义(t=4.418,P=0.000;t=3.736,P=0.000<0.05).结论 益气活血解毒法治疗酒精性心肌病缓解临床症状,有效改善患者左心室功能,疗效显著.

Objective Using whole exome sequencing (WES) to help to make a definite diagnosis for confusing cases at clinical practice. Methods A 29-year old proband with clinical manifestations of mild myocardial hypertrophy and skeletal myopathy, muscle biopsy suggested multiple minimal axonal disease. WES was applied to find the possible pathogenic gene mutations and verified in the proband and her relatives with Sanger sequencing. Results A likely pathogenic heterogeneous variant LAMP2 c. 974delT (p. Leu325fs) located on the X chromosome was detected in the proband, and LAMP2 encodes a type Ⅱ lysosome-associated membrane protein associated with Danon disease. Her younger brother with similar echocardiographic and muscle biopsy results also carried this hemizygous variant, but other healthy relatives including her father was negative for this variant. Therefore, there is a co-segregation of this variant with the disease in this family. Conclusions For those confusing cases which can′t be accurately diagnosed according to clinical manifestations and routine examination results, WES may be a more feasible and effective method for the differential diagnosis.

The present study reports on a family with two members affected by Danon disease but having different phenotypes. The clinical manifestations of Danon disease include cardiomyopathy, skeletal myopathy and different degrees of intellectual disability that varies greatly among patients. The present case study reports on two siblings, an older sister and a younger brother, with Danon disease from an affected pedigree, presenting with distinctly different phenotypes. The sister was diagnosed with dilated cardiomyopathy at the age of 26 years with an unfavorable outcome, while her younger brother presented with hypertrophic cardiomyopathy in a relatively stable state. The two probands shared the same mutation, c.974delTinsAA in exon 8, in the lysosomal-associated membrane protein-2 gene. Of note, the two patients had a pre-excitation pattern in the electrocardiogram on initial presentation and later developed atrial fibrillation (AF), which markedly aggravated heart failure. To the best of our knowledge, AF has not been widely reported in patients with Danon disease. The development of AF may have a prognostic value under these circumstances.

目的 研究炙甘草汤剂治疗糖尿病性心肌病的临床疗效和安全性.方法 研究时间为2016年3月—2018年3月,研究对象为该院收入治疗的78例糖尿病性心肌病患者,按照随机对照原则分为研究组和对照组,各39例,对照组采用西药治疗,研究组在对照组基础上加用炙甘草汤治疗,比较两组治疗后疗效及不良反应发生情况.结果 研究组临床治疗总有效率94.87％显著高于对照组82.05％,两组单项不良反应差异无统计学意义(P>0.05),但研究组总不良反应发生率17.95％低于对照组30.77％,组间比较,差异有统计学意义(P﹤0.05).结论 炙甘草汤用于治疗糖尿病性心肌病疗效显著,安全性高,可有效促进病情康复,值得推荐使用.

目的:探究阿托伐他汀钙联合依那普利治疗缺血性心肌病的临床疗效.方法:选取2014年7月至2015年7月在我院接受治疗的80例缺血性心肌病患者为考察对象,将80例患者按照不同治疗方法将他们分为对照组和研究组,各40例,对照组患者采用阿托伐他汀钙进行治疗,研究组在对照组的基础上添加依那普利进行治疗,对比两组治疗效果及不良反应发生情况.结果:治疗之后,对照组和研究组的总有效率分别为77.50％和95.00％,不良反应发生率分别为25.00％和7.50％,两组间数据差异较大,且P＜0.05,数据差异存在统计学意义.结论:阿托伐他汀钙联合依那普利治疗缺血性心肌病临床疗效显著,且不良反应发生率较低,值得推广应用.

目的:研究缺血性心肌病(ICM)的临床特点、治疗方法与效果.方法:选取我院于2010年1月～2016年1月间收治的ICM患者58例,回顾性分析其临床资料,总结其临床特点及治疗情况.结果:所有患者均有冠心病史,男性所占比例较大(70.7％),临床症状以气短、胸闷、心悸为主,均发生心衰竭;左心射血分数降低者占77.6％,左心室、心脏扩大者占84.5％,室壁运动呈节段性异常者占74.1％,左心室增大者占86.2％;存在程度不同的肺淤血者占53.4％,心律失常者占70.7％;病理Q波伴或不伴ST-T改变者占84.5％,冠状动脉均狭窄,2支或多支血管发生病变,平均在76％以上.给予抗心律失常、血管扩张剂、利尿剂等对症支持治疗,行经皮腔内冠状动脉成形术(PTCA)治疗者40例.最终4例患者死亡,其余54例患者均病情好转并出院.结论:ICM的发病人群以老年男性为主,临床表现大多为心律失常、心力衰竭和心脏扩大,与扩张型心肌病相似,可通过冠状动脉造影加以鉴别,采用介入治疗可取得良好效果.

Objective:to explore the curative effect of Buyuan Yangxin Tang on the heart and kidney insufficiency of dilated car-diomyopathy.Methods:200 patients with dilated cardiomyopathy-induced heart failure combined with kidney insufficiency from our de-partment were randomly divided into experimental group ( EG) and control group ( CG) .CG were given western conventional therapy under the guidance of heart failure while EG were added with our self-made Buyuan Yangxin Tang , for four weeks as a course.Com-pare the curative effect scores before and after treatment, and other indexes like LVDd, LVEF, cardio-thoracic ration, BNP, CRP, se-rum creatinine, urea nitrogen and uric acid, to observe the therapeutic effect of Buyuan Yangxin tang .Results:(1) before and af-ter therapy, the total effective rate of EG was 96%, CG 71.4%, the former superior to the latter ( P <0.05 ); ( 2 ) compared to pretherapy, both indexes of the heart improved greatly, LVEF increased to some degree ( P CG <0.05;P EG <0.01); LVDd and BNP decreased ( P CG <0.05;P EG <0.01);cardio-thoracic ration decreased ( P CG <0.05;P EG <0.01);CRP improved signifi-cantly ( P CG <0.05;P EG <0.01);compared to CG, the heart functional index improved more significantly ( P <0.05); (3) compared to pretherapy, kidney indexes of both group were improved, serum creatinine, urea nitrogen and blood uric acid decreased ( P CG <0.05;P EG <0.01); EG improved more significantly than CG in all indexes ( P <0.05) .Conclusion: western therapy together with Buyuan Yangxin Tang can improve not only clinical symptoms and heart function, but also kidney index, which makes up for the damage to the kidney during treatment of heart failure with western medicine.

目的 观察平肝通脉汤治疗不稳定性心绞痛合并高血压的疗效,并评价其安全性.方法 将120例不稳定性心绞痛合并原发性高血压患者随机分为2组,对照组60例予常规西药治疗,治疗组60例在对照组西药方案基础上,予平肝通脉汤辅助治疗.4周后比较治疗前后血压、心电图及中医证候改变情况,并统计不良反应发生情况.结果 治疗组治疗后心绞痛发作次数及发作持续时间、收缩压及舒张压均降低(P＜0.05),心电图ST段及T波倒置均明显改善(P＜0.05),且改善情况均优于对照组(P＜0.05).治疗组心绞痛改善总有效率88.3％,优于对照组(60.0％,P＜0.05).治疗组心电图改善总有效率86.7％,优于对照组(50.0％,P＜0.05).治疗组降压疗效总有效率88.3％,优于对照组(68.3％,P＜0.05).治疗组中医证候疗效总有效率90.0％,优于对照组(68.3％,P＜0.05).治疗组不良反应发生率8.33％,对照组13.3％,2组比较差异无统计学意义(P＞0.05).结论 平肝通脉法用于不稳定性心绞痛合并高血压的治疗,能够在不影响安全性的前提下提高心绞痛及原发性高血压的治疗效果,值得在临床上推广研究.

目的:观察益气解毒活血通络法对缺血性心肌病患者心功能的影响.方法:选取缺血性心肌病患者100例,按随机数字表法分为观察组和对照组,每组50例.对照组给予常规西药对症治疗,观察组在常规治疗基础上给予心肌活力饮半剂和冠脉疏通饮半剂治疗.结果:治疗后观察组心功能疗效、中医证候疗效、左心室射血分数、6 min步行距离均明显优于对照组,差异有统计学意义(P＜0.05).结论:益气解毒活血通络法治疗缺血性心肌病疗效显著,能改善患者心功能.

目的 对比益气活血法和常规西药法治疗缺血性心肌病的疗效.方法 从本院2013年3月~2015年3月住院的缺血性心肌病患者中挑选符合研究要求的50名患者,分成治疗组(口服中药心肌活力饮、冠脉疏通饮+常规西药)和对照组(口服常规西药)各25例.对比两组疗效和心功能.结果 观察组疗效为Ⅰ级36.00%(9/25),Ⅱ级为28.00%(7/25),均高于对照组的12.00%(3/25),16.00%(4/25),差异均有统计学意义(均P<0.05).结论 因缺血性心肌病具有冠心病心绞痛和扩张型心肌病两种疾病的特点,所以受此启发,结合人体的阴阳消长,秉承中医"整体观念、辩证论治"的原则我们采用"益气活血"法治疗缺血性心肌病.

目的:观察益气解毒活血通络法治疗缺血性心肌病的临床疗效.方法:选取缺血性心肌病患者100例,采用随机数字表法分为治疗组和对照组,每组50例.治疗组采用益气解毒活血通络法治疗,对照组采用常规西药口服治疗.结果:治疗组有效率为92.0％,对照组有效率为76.0％,治疗组优于对照组(P＜0.05);治疗组治疗后6 min步行距离优于对照组(P＜0.05).结论:益气解毒活血通络法治疗缺血性心肌病临床疗效显著.

　　扩张型心肌病是一种以左心室或双心室扩张并伴收缩功能受损为特征，常表现为进行性心力衰竭、室性和室上性心律失常、血栓栓塞、猝死，可见于病程中任何阶段。西医对该病治疗目前仍无有效确切的治疗手段从根本上逆转心肌细胞损害，改善心功能，仅是针对心衰、心律失常、栓塞等对症治疗。因此中西医结合治疗DCM 在此情况下不失为一种可行性选择。

1病例资料 患者男性，40岁，入院诊断：扩张型心肌病，心力衰竭，心功能Ⅲ级，心律失常、心房纤颤。查心脏彩超示：左房前后径53mm、左室舒张末内经65mm、右房（四腔心切面）58*56mm、右室前后径32mm、左室射血分数20%，入院后常规使用洋地黄、血管紧张素转换酶抑制剂、利尿剂和硝酸酯类、β受体阻滞剂等药物治疗，病情一度好转，但于住院期间晨起活动后突发晕厥，呼之不应，大动脉搏动消失，心脏骤停，就地实施心肺复苏术，心电监护示：反复室速、室颤，血压难以维持。行持续心脏按压，反复电除颤，给予积极纠正心衰、酸碱失衡、抗心律失常等抢救治疗约4个小时，共除颤22次，患者恢复窦性心律及自主呼吸，但因患者原发扩张型心肌病，心功能较差，惊恐焦虑，予芬太尼、咪达唑仑以镇静，继续给与呼吸机辅助呼吸，三天后脱机。后经过半个月的巩固治疗，患者病情平稳，好转出院。

Objective:To investigate the clinical efficacy of warming yang to promote diuresis tonifying Qi circulating blood treatment of cardiorenal syndrome.Methods:100 cases of patients with renal insufficiency in patients with heart failure of dilated cardiomyopathy,based on Combination of traditional Chinese and Western medicine plus complement Yangxin Decoction,observe the change of cardiac and renal function in patients before and after treatment.Results:Before and after treatment in patients with left ventricular diameter(LVD),ejection fraction(LVEF),cardiothoracic ratio and heart failure markers-brain natriuretic peptide(BNP),CRP were significantly improved(P<0.01).4 weeks after the treatment,the serum creatinine than before treatment,after 1 weeks of treatment decreased significantly(P<0.01),compared with 2 weeks also decreased(P<0.05);urea nitrogen treatment for 2 weeks,4 weeks after treatment were significantly decreased(P<0.01),1 weeks after treatment than before treatment has decreased(P<0.05);blood uric acid after 4 weeks of treatment than before treatment,1 weeks after treatment was significantly lower(P<0.01),2 weeks after treatment than before treatment also decreased(P<0.05).Conclusion:Onyang diuresis Yiqihuoxue treatment of heart and kidney significantly syndrome.

扩张型心肌病(dilated cardiomyopathy,DCM)是以左室、右室或双心腔扩大和收缩功能障碍等为特征的心肌病,进行性心力衰竭、心律失常、血栓栓塞和猝死是其特点.约1/3充血性心力衰竭(congestive heart failure,CHF)由DCM发展而来[1].随着心功能的下降,全身各系统均会受到影响,其中,心衰和肾功能损害之间的交互作用可使病情加重且与心衰症状的进展相关[2].

Objective To assess the influence of Xinjihuoli granule on the treatment of Qi deficiency to blood stasis type dilated cardiomyopathy with cardiac failure.Methods 120 cases of Qi deficiency to blood stasis type dilated cardiomyopathy with cardiac failure were randomly divided into two groups.60 cases in control group were given conventional western medicine therapy.60 patients in treatment group were treated by combination of Xinjihuoli granule and basis of control group treatment.The course was a month in two groups.The changes palpitations,shortness of breath,wheezing,chest tightness(pain),irritability,cough and spontaneous syndromes,cardiothoracic ratio,left ventricular end diastolic diameter and left ventricular ejection fraction(LVEF) were recorded.Cardiac function efficacy and effect of traditional Chinese medicine syndromes,and safety evaluation were completed.Results Traditional Chinese medicine syndrome points and total score after treatment were significantly decreased as compared with those before treatment in two groups(P0.01).Palpitations,shortness of breath,wheezing,chest tightness(pain),irritability syndrome score and total score in treatment group were lower than those in control group before treatment(P0.01).After treatment,cardiothoracic ratio compared with this group before treatment were significantly decreased(P0.05),left ventricular end diastolic diameter narrowing(P0.05) and LVEF increased(P0.05),and there was statistically significant between groups(P0.05),the treatment group was better than the control group.Efficacy of cardiac function,total effective rate of traditional Chinese medicine syndromes in treatment group(70.0%,96.7%) was higher than those in control group(50.0%,55.0%,P0.05).Conclusion Xinjihuoli granule can significantly improve cardiac function and clinical symptoms and signs of Qi deficiency to blood stasis type dilated cardiomyopathy and no obvious side effects.

<正>1病历介绍患者,女,44岁,主因间断心悸1个月于2009年6月13日11:42入我院,既往3 a前行子宫肌瘤摘除术。患者缘于1个月前无明显诱因出现心悸,当时心率90次/min左右,无胸闷气短,无胸痛,无发热,口服普萘洛尔症状可缓解。之后上述症状时轻时重,就诊多家医院,未予明确诊治,近日上述症

扩张型心肌病是一种难治性心脏病,家族性扩张型心肌病的治疗难度更大.因该病属常染色体显性遗传,致残率、病死率都很高,直到目前还没有有效的办法使病变的基因恢复正常,故多数学者认为家族性扩张型心肌病目前的治疗不可能使扩大的心脏明显缩小,而且心脏逐年增大,心力衰竭越来越严重,生存期越来越短.笔者用自拟中药方"心肌活力饮"加减,同时用对抗神经内分泌过度激活的西药,可使心力衰竭明显改善,心脏明显缩小,有的大心脏可恢复至正常大小.现将中西药治疗一家系扩张型心肌病的体会介绍如下.

<正>扩张型心肌病是指原因不明的心脏扩大,多伴有心力衰竭的一组疾病。因常出现心律失常、血栓栓塞、心室颤动或心脏骤停,治疗异常棘手,如果控制不佳,心衰会反复发作,生存期