Prognosis in hypertrophic cardiomyopathy with asymmetric septal hypertrophy

Hypertrophic cardiomyopathy with asymmetric septal hypertrophy is not an uncommon disorder in the older adult population, and frequently masquerades as coronary artery disease. Most symptomatic patients with hypertrophic cardiomyopathy diagnosed as adults have a benign course with mild disability lasting for many years. Associated significant obstructive coronary artery disease was present in 22% of our patients and probably represents the cause of symptoms and, therefore, investigation in some of our patients rather than a high incidence of coronary disease in patients with asymmetric septal hypertrophy. Sudden death was not common in our patients (3.6%) and occurred at 11 years after diagnosis in a patient with associated coronary artery disease and 4 years after diagnostic study in a patient with uncomplicated hypertrophic cardiomyopathy.     

Sudden death associated with group A streptococcal infection in an 8-year-old girl with undiagnosed hypertrophic cardiomyopathy

An 8-year-old girl died suddenly without prior symptoms. Post-mortem examination identified both systemic group A streptococcal infection and hypertrophic cardiomyopathy. She had no history of cardiac symptoms and was not in a high-risk group for sudden death due to hypertrophic cardiomyopathy. We believe the disseminated but asymptomatic group A streptococcal infection precipitated her early death from hypertrophic cardiomyopathy. Sudden unexpected death during systemic infection should be followed by post-mortem examination to look for evidence of hypertrophic cardiomyopathy, as this diagnosis has genetic implications for other family members.     

Sudden cardiac death among competitive adult athletes: a review

Sudden cardiac death is the leading cause of mortality among young athletes with an incidence of 1-2 per 100,000 athletes per annum. It is described as 'an event that is non-traumatic, non-violent, unexpected, and resulting from sudden cardiac arrest within six hours of previously witnessed normal health'. Most predisposed athletes have no symptoms and there is no warning for the impending tragic event. The majority of cases are caused by an underlying structural cardiac abnormality, most commonly hypertrophic cardiomyopathy. More recently, the understanding of non-structural causes such as long QT syndrome and Brugada syndrome has grown and diagnostic criteria have been developed. This review presents the known aetiologies of sudden cardiac death among athletes and outlines their identification and management including implications for future sporting participation as laid out in the consensus documents produced by the European Society of Cardiology and the 36th Bethesda Conference.     

肥厚性心肌病心源性猝死的危险因素分析

目的:分析肥厚性心肌病心源性猝死的危险因素。方法:收集发生心源性猝死的肥厚性心肌病患者32例,所有患者均经临床表现、实验室检查、心电图及超声心动图检查确诊,回顾性分析患者的临床资料。结果:单因素分析显示心脏骤停(心室颤动),自发性持续性室速,猝死家族史(≤40岁的一级亲属),晕厥(≥2次/年),左室厚度≥30mm,负荷或运动后收缩压反应异常(无变化或降低>10mmHg或升高<25mmHg),非持续性室速,左室流出道梗阻(压力差>30mmHg)等因素为肥厚性心肌病患者发生心源性猝死的危险因素(P<0.01)。多因素logistic分析显示心脏骤停(心室颤动),自发性持续性室速,猝死家族史及晕厥≥2次/年是肥厚性心肌病心源性猝死重要的危险因素。结论:多种危险因素与肥厚性心肌病心源性猝死密切相关,临床工作中应该积极应对。     

肥厚型心肌病冠状动脉造影的特征及临床意义

目的分析肥厚型心肌病(HCM)患者冠状动脉造影的特征及临床意义。方法将64例经心脏彩色多普勒超声、左心室造影和冠状动脉造影检查确诊为HCM的患者分为梗阻型HCM组(HOCM组,44例)及非梗阻型HCM组(HNCM组,20例),比较两组患者的基线临床资料及冠状动脉造影检查结果,测量并比较冠状动脉各分支的内径。结果冠状动脉性心脏病的高危因素中,两组间仅年龄及糖尿病构成比的差异有统计学意义(P值均<0.05)。心脏彩色多普勒超声检查显示,两组间二尖瓣前叶收缩期前向运动(SAM)征的构成比、左心室流出道压差、室间隔厚度、左心房直径的差异均有统计学意义(P值均<0.05)。冠状动脉造影检查显示,两组间冠状动脉狭窄、左前降支肌桥的发生率及左侧冠状动脉优势型患者构成比的差异均无统计学意义(P值均>0.05),而冠状动脉粥样硬化、左间隔支肌桥发生率的差异均有统计学意义(P值均<0.01)。HOCM组的左主干、左前降支、左回旋支及左间隔支内径均显著大于HNCM组(P值均<0.01)。结论 HOCM患者的左间隔支肌桥发生率较HNCM患者高,且冠状动脉内径较粗大,尤其是左间隔支粗大,对临床上经皮导管化学消融有指导意义。     

Causes of sudden cardiac death in young Australians

OBJECTIVES: To determine the causes of sudden cardiac death in people aged 35 years or younger. DESIGN AND SETTING: A review of all autopsies performed between 1 January 1994 and 31 December 2002 at a major Sydney forensic medicine department serving an area with over 2 million people. MAIN OUTCOME MEASURES: Incidence of various types of cardiac disease causing sudden death in those aged 相似文献   

肥厚型心肌病基因突变的临床意义

肥厚型心肌病（hypertrophic cardiomyopathy,HCM）是以心肌肥厚尤其是左心室不对称性心肌肥厚、心肌纤维肥大、排列紊乱为病理特征的原发性心肌病,是目前年轻人和运动员常见的猝死原因之一。目前HCM通常被认为是一种基因突变所导致的常染色体疾病,呈显性遗传。最近研究发现心脏肌节蛋白基因以及相关的线粒体基因与修饰基因的基因突变,超过900种不同的基因突变类型与HCM发生发展以及临床表型有关。本文主要就近年来关于HCM常见的基因突变与其临床意义的研究作一综述。     

Clinical and angiographic profile of congenital anomalous origin of coronary arteries

Coronary artery anomalies were found in 24(0.6%) patients undergoing coronary arteriography at University Cardiac Center, Bangabandhu Sheikh Mujib Medical University, Dhaka from 2004 to 2007. Of the 24 patient, 21(87.5%) had anomalies of origin and distribution and 3(12.5%) had coronary artery fistulae. Most coronary anomalies did not result in signs, symptoms or complications and usually were discovered as an incidental finding at the time of catheterization. Most of them were benign anomalies: i) separate origin of left anterior descending and circumflex from sinus of valsalva; ii) ectopic origin of circumflex from right sinus of valsalva; iii) Anomalous origin of left main coronary artery from right coronary artery; iv) anomalous coronary origin from ascending aorta; v) absent left circumflex; vi) double right coronary artery; vii) small coronary artery fistula. These anomalies may be associated with potentially serious sequelae such as angina pectoris, myocardial infarction, syncope, cardiac arrhythmias, congestive heart failure, or sudden death. In this study large coronary artery fistula was found as potentially serious anomaly. So coronary artery anomalies require accurate recognition for the appropriate management of the patients.     

84例肥厚型心肌病患者的临床特点和治疗分析

目的：分析肥厚型心肌病的临床特点和治疗方法。方法回顾性分析2008年1月至2013年12月在安徽医科大学第一附属医院就诊的肥厚型心肌病患者的临床资料。结果84例肥厚型心肌病患者常见的临床症状主要有胸闷气短、心悸、胸痛、呼吸困难、头晕黑曚、前兆晕厥或晕厥。常见的合并疾病有高血压病、冠心病和心律失常,心律失常中最常见的为房颤和各种传导阻滞。95．2%的肥厚型心肌病患者心电图有异常表现,最常见的异常表现为左心室肥厚和(或) ST-T改变。80例患者接受β受体阻滞剂和(或)非二氢吡啶类钙离子拮抗剂治疗,11例患者接受起搏器置入治疗,22例接受经皮室间隔心肌化学消融术( PTS-MA),2例患者接受外科心肌切除手术。院内死亡1例,死亡原因为恶性心律失常。余82例患者住院期间患者症状均有缓解。结论肥厚型心肌病患者临床表现多样,治疗应遵循个体化原则,选择合理的药物或手术方法。     

36例左室心肌致密化不全的临床特征及中期预后分析

目的:分析中国人左室心肌致密化不全的临床特征及中期预后.方法:回顾性分析36例左室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(30±10)个月.进行生存分析,运用Cox风险回归模型分析临床不良事件(死亡或心脏移植)的预测因素.结果:左室心肌致密化不全占同期确诊的各类型原发性心肌病的9.4％,仅次于扩张性心肌病(67.3％)和肥厚性心肌病(20.9％).发病年龄1.5-72岁,男性占66.7％.心尖部及左室中段侧壁和下壁最常受累及.随访期间,25例患者出现心力衰竭,8例患者出现室性心动过速,2例患者出现血栓栓塞,8例患者死亡,1例患者接受心脏移植.临床不良事件(死亡或心脏移植)的独立预测因素包括:心功能NYHAⅢ/Ⅳ级(RR:6.139,95％CI:1.072-35.157,P=0.042)和室性心动过速(RR:1.676,95％CI:1.25022.846,P=0.024).结论:左室心肌致密化不全在原发性心肌病中并不罕见.心力衰竭、心律失常和血栓栓塞是主要并发症.心功能NYHAⅢ/Ⅳ级和室性心动过速是预后不良(死亡或心脏移植)的独立预测因素.     

冠状动脉变异对动脉调转手术的影响

目的 观察冠状动脉变异对动脉调转手术结果的影响.方法 2001年1月至2008年12月中国医学科学院北京协和医学院阜外心血管病医院共有280例患者行动脉调转手术,73例(26.1%)合并冠状动脉变异(变异组),其中男54例,女19例,年龄7 d～6岁,平均(0.6±1.1)岁,体重3.0～18.0(5.8±2.6)kg.合并室间隔缺损的完全性大动脉转位21例,室间隔缺损完整的完全性大动脉转位30例,Taussig-Bing畸形22例.其余207例冠状动脉正常(正常组).手术方法包括动脉调转和冠状动脉移植以及其他心内畸形的矫治.结果 全组早期死亡29例,总病死率10.4%,其中变异组死亡12例,病死率为16.4%,而正常组死亡17例,病死率为8.2%,两组患者病死率差异有统计学意义(P＜0.05),2001至2005年间冠状动脉异常的患者手术病死率是冠状动脉正常的患者手术病死率的2倍.冠状动脉变异组平均体外循环时间(229±84)min,主动脉阻断时间(146±48)min,均长于冠状动脉正常组(206±59)min和(137±40)min,两组患者体外循环时间的差异有统计学意义(P＜0.05).冠状动脉变异组6例患者术中证实有冠状动脉受压或梗阻,早期死亡的12例患者中包括单冠畸形5例,冠状动脉走行异常4例,其他少见畸形3例.结论 冠状动脉变异增加手术难度并影响手术结果,手术技术的成熟可以减少病死率.     

Clinical and histologic studies of a Qatari family with myofibrillar myopathy

The current study reports the first family with confirmed myofibrillar myopathy MFM in the Middle East and the third family worldwide. This study highlights the importance of considering MFM in young patients presenting with idiopathic cardiomyopathy, arrhythmia or atrioventricular block in the Gulf states. This is the first report that presented 2 different types of cardiomyopathy and 2 different indications of permanent pacemaker placement in the same generation of a family with MFM. This report studies a Qatari family consisting of one brother and 3 sisters. The brother had restrictive cardiomyopathy at the age of 16 years. One sister underwent heart transplantation for severe hypertrophic cardiomyopathy at the age of 15 years, the other sister had permanent pacemaker for complete heart block at the age of 21 years. This report is focused mainly on the clinical presentation and investigations carried out for the brother including echocardiogram, cardiac catheterization, cardiac and skeletal muscle biopsy, and electromyography and electrophysiology studies. The study findings support the diagnosis of MFM.     

双源CT诊断心肌桥及心肌桥相关冠状动脉病变的临床分析

目的:探讨双源CT(DSCT)对心肌桥的临床诊断价值及心肌桥合并冠状动脉粥样斑块与年龄的相关性。方法:应用双源CT对417例有心绞痛或类似心绞痛症状的可疑冠心病者行CT冠状动脉血管成像;并按患者年龄不同分为青年、中年和老年组,分析组间合并冠状动脉斑块及斑块性质的差异。结果:青年、中年和老年组分别为137例、197例和83例,所有患者检出心肌桥病变者76例(28.1%),其中合并有冠状动脉斑块者41例(53.9%)。青年组心肌桥10例(13.2%),合并冠状动脉斑块者2例(20.0%);中年组心肌桥36例(47.4%),合并冠状动脉斑块者17例(47.2%);老年组心肌桥30例(39.5%),合并冠状动脉斑块者22例(73.3%)。组间心肌桥冠状动脉斑块发生率及斑块性质差异有显著性统计学意义(P<0.01)。结论:双源CT对心肌桥病变检出率高。心肌桥合并冠状动脉粥样斑块与年龄增长有关,不同年龄组合并冠状动脉斑块的性质有显著差异。     

Perioperative anesthetic risk of noncardiac surgery in hypertrophic obstructive cardiomyopathy

To determine their perioperative risk, we reviewed the records of 35 patients with hypertrophic cardiomyopathy diagnosed by cardiac ultrasound and/or catheterization who underwent general (52) or spinal (four) anesthesia--a total of 56 major surgical procedures. There were no operative or related perioperative deaths and no significant ventricular tachyarrhythmias. Intraoperative or postoperative complications included: myocardial infarction with heart failure in one patient who also had coronary artery disease and was one of three patients who had spinal anesthesia, arrhythmia requiring therapy in eight, and angina during supraventricular tachycardia in one. We conclude that the risk of general anesthesia and major noncardiac surgery is low in patients with hypertrophic obstructive cardiomyopathy. Spinal anesthesia, which decreases systemic vascular resistance and increases capacitance, may be relatively contraindicated. Concomitant coronary artery disease may increase the risk.     

Congenital heart disease, maternal age and parental consanguinity in children with Down's syndrome

OBJECTIVE: Analyze the role of parental consanguinity and/or maternal age on the occurrence of congenital cardiac anomalies in infants with Down's syndrome (DS). MATERIAL AND METHODS: A large group of patients with or without congenital heart disease, registered between May 1st, 1999 and June 30th, 2006, at the National Register of Paediatric and Congenital Heart Disease, Lebanese Society of Cardiology. 217 children with DS have been enrolled over this period. Group I: DS with cardiac anomalies (n:119). Group II: DS without cardiac anomalies (n:98). The consanguinity rate was compared to a study made in 2000 by UNICEF in Lebanon, in which the consanguinity rate was 14.9% of 1st degree cousins and 25% of all degrees together. RESULTS: Congenital cardiac anomalies were found in 54.2% of babies with DS. First degree cousins in both groups I and II was 143% (close to the general population), and it was lower in group I compared to group II (11.8% and 17.3% respectively ; p = 0.205) ; this difference was not statistically significant for all degrees together. Maternal age ranged from 20 to 47 years, with a mean of 29.5 +/- 5.3 years. The risk of congenital cardiac anomaly was greater (p < 0.05) when maternal age was equal or below 32 years. As in the international literature, complete atrioventricular canal and ventricular septal defect were the most frequent cardiac anomalies. CONCLUSION: In children with DS, the risk of congenital cardiac anomalies was not associated with the parents consanguinity. Maternal age above 32 years seem to be associated with a lesser occurrence of congenital cardiac anomaly in children with DS.     

冠状动脉畸形64层螺旋CT血管成像24例分析

目的分析先天性冠状动脉畸形64层螺旋CT血管成像诊断价值。方法回顾性分析423例患者行64层螺旋CT血管成像影像学资料,并分析冠状动脉畸形者的CT影像表现。结果423例行64层螺旋CT血管成像患者中,发现冠状动脉畸形24例,其中左前降支与左旋支并行7例;左旋支缺如2例;冠状动脉开口于升主动脉10例;单支冠状动脉1例;冠状动脉瘘4例。结论64层螺旋CT血管成像在冠状动脉畸形的诊断有重要价值。     

先天性冠状动脉变异的双源CT分析

目的探讨双源CT冠状动脉成像对先天性冠状动脉变异的检出率及诊断价值。方法对2007年5月~2008年9月行心脏双源CT冠状动脉成像的资料（2314例）进行分析,总结先天性冠状动脉变异的发生率及其CT表现。结果2314例中发现先天性冠状动脉变异170例,检出率7.35%,冠状动脉主支起源异常27例,冠状动脉瘘6例,副冠状动脉9例,冠状动脉发育不良7例,左窦房结动脉起源异常121例。结论双源CT冠状动脉成像能准确全面诊断先天性冠状动脉变异,可作为先天性冠状动脉变异的首选检查方法。     

儿童冠状动脉起源异常的临床分析（附7例报告）

目的 总结冠状动脉起源异常(coronary artery origin anomalies,CAOA)的临床及检查特点，为及时诊断及治疗提供一定的依据。方法 对我院2000-2008年经心脏彩超及选择性冠状动脉造影确诊的冠状动脉起源异常7例患者的临床症状，体征，辅助检查，诊断及治疗方法进行回顾性分析。结果 左旋支起源于肺动脉1例，左前降支开口于右冠状动脉窦1例，左前降支异常起源于肺动脉1例，左冠状动脉异常起源于肺动脉前窦1例，左冠状动脉异常起源于肺动脉2例，右冠状动脉起源于肺动脉1例。误诊为扩张型心肌病3例，误诊为心内膜弹力纤维增生症2例，误诊为心肌致密化不全1例，误诊为冠状动脉-肺动脉漏1例。结论 儿童冠状动脉起源异常极易误诊为心内膜弹力纤维增生症及心肌病，提高临床及超声心动图医师对该病的认识，及时诊断及治疗对于提高患儿生存率有重要意义     

不同年龄段急性心肌梗死病人临床和冠状动脉病变特点分析

目的探讨不同年龄段急性心肌梗死（AMI）病人的临床和冠状动脉（冠脉）病变特点。方法选择201例行急诊冠脉造影检查的AMI病人,根据年龄分为中青年组（< 60岁,n=70）和老年组（≥60岁,n=131）,比较2组病人的临床资料和冠脉病变资料。结果AMI老年病人较中青年病人更容易发生院内死亡和心力衰竭（P < 0.05）;中青年组病人的红细胞计数、血红蛋白、血细胞比容、网织红细胞计数、淋巴细胞计数、嗜酸性粒细胞计数、丙氨酸氨基转移酶、胆碱酯酶、总蛋白、白蛋白、尿酸、总胆固醇、低密度脂蛋白和载脂蛋白B水平均高于老年组（P < 0.05~P < 0.01）,脂蛋白a低于老年组（P < 0.05）。中青年组病人冠脉以单支血管病变、低Gensini积分为主,术中发生无/慢复流的概率低;老年组病人冠脉以多支血管、钙化病变、高Gensini积分为主,术中容易出现无/慢复流,2组比较差异有统计学意义（P < 0.05~P < 0.01）。结论中青年AMI病人的血压、血糖、血脂水平更高,冠脉病变以单支、低Gesini积分病变为主。老年AMI病人冠脉病变以弥漫性长病变、钙化病变为主,术中容易发生无/慢复流。     

多器官病变在心力衰竭死亡患者中的发生状况和趋势

目的 了解急性失代偿心力衰竭住院死亡患者中多器官病变的发生状况和趋势.方法 回顾性分析2006年1月至2007年阜外心血管病医院重症监护病房30例住院死亡的急性失代偿心力衰竭患者的临床资料,对呼吸衰竭、肾功能不全、肝功能不全、肺部感染和贫血在入院时和死亡前的情况进行分析和比较.结果 30例死亡患者平均年龄(55±22)岁.男19例,女11例,NYHA心功能Ⅲ级者7例、Ⅳ级者23例.病因包括心脏瓣膜病17例、扩张型心肌病6例、缺血性心肌病4例、心脏瓣膜病合并陈旧性心肌梗死2例、限制型心肌病1例.平均住院时间(8.8±7.4)d.入院时合并肺部感染、肾功能不全、呼吸衰竭、肝功能不全和贫血者分别有15、6、7、13和7例,合并多器官(2个以上器官功能)病变者15例.死亡前,上述器官病变者分别增加至27、24、20、19例(P＜0.05或0.01)和9例(P＞0.05),合并多器官病变者增至29例(97%、P＜0.01).在发生致命性心律失常的11例患者中,3例在入院时合并多器官病变,另7例患者在心律失常事件后发生了多器官病变.而在未发生致命性心律失常的19例患者中,12例在入院时即合并多器官病变,7例在死亡前发生多器官病变.在发生致命性心律失常而早期心肺复苏成功且存活12 h以上的6例患者,均在心肺复苏之后发生了多器官病变,主要是肾功能不全、肺部感染、呼吸衰竭.结论 急性失代偿心力衰竭患者在心衰恶化到死亡的过程中普遍发生了多器官病变,以肺郎感染、肾功能不全和呼吸衰竭的增加最显著.