肥厚型心肌病病人室性心律失常发生率的基因预测

目的 筛选肥厚型心肌病(HCM)高危病人,从而进行重点监护。方法 采用PCR技术对102例HCM病人(观察组)的血管紧张素转换酶(ACE)基因型进行分析,并与98例正常人(对照组)比较,同时比较分析观察组ACE基因型与室性心律失常的关系。结果 两组经电泳得到ACE基因的DD、ID、Ⅱ3种基因图谱,观察组ACE基因DD型及D等位基因频率显著高于正常对照组(均P相似文献   

实时三平面应变率成像评价肥厚型心肌病患者左心室长轴舒张功能

目的采用实时三平面应变率成像评价肥厚型心肌病(HCM)患者左心室长轴局部舒张功能。方法选取HCM患者37例(HCM组)与健康志愿者42名(正常对照组)。采用实时三平面应变率成像模式测定受检者左心室各壁基底段、中间段舒张早期(SRe)、舒张晚期(SRa)的峰值应变率,并进行统计学分析。结果HCM组各节段SRe、SRa、SRe/SRa与正常对照组比较均明显降低(P均0.05),HCM组室间隔及前壁的基底段、中间段SRe、SRe/SRa较左心室后壁均明显降低(P均0.05)。结论实时三平面应变率成像技术可快速、客观地定量分析HCM患者左心室长轴舒张功能的变化。     

超声三维斑点追踪成像评价肥厚型心肌病左心室扭转运动

目的应用超声三维斑点追踪成像(3D-STI)技术评价肥厚型心肌病(HCM)左心室扭转运动特征。方法收集非梗阻性HCM患者28例(HCM组)及正常志愿者25名(对照组),应用3D-STI获取并定量比较其左心室收缩及舒张末期容积、射血分数、质量和室壁基底水平及心尖水平收缩期旋转角峰值、局部扭矩峰值,左心室整体及局部收缩期扭转角峰值、左心室整体收缩期扭矩峰值等参数。结果 HCM组左心室收缩及舒张末期容积减小,左心室质量增加,左心室壁基底水平收缩期旋转角峰值和局部扭矩峰值、左心室整体收缩期扭转角峰值和扭矩峰值以及室间隔基底水平和心尖水平局部扭转角峰值均大于对照组(P均<0.05),而左心室射血分数、心尖水平收缩期旋转角峰值和局部扭矩峰值差异均无统计学意义(P均>0.05)。结论HCM患者左心室整体及部分节段(室间隔基底水平和心尖水平)收缩期扭转运动能力较正常人增强。左心室整体收缩期扭转角峰值能更好地反映HCM的异常心肌力学状态。     

含高钙和维生素D的强化奶能有效提高健康的绝经后的中国女性体内的维生素D水平并减少骨吸收

目的 钙摄入水平低以及体内维生素D不足是导致亚洲女性罹患骨质疏松的危险因素。此项研究的目的是评估高钙维生素D强化牛奶（HCM）对健康的中国绝经后妇女的体内维生素D状态和骨吸收标志物进行干预的影响。方法 本试验对象为55岁以上,绝经至少5年的女性。共有124名女性进行了总体健康状况和骨密度检查筛选。63名女性被随机分为对照组和试验组,试验组在12w内每日饮用两次高钙/维生素D强化奶（含900mg钙、96mg镁、2.4mg锌和6.4μg维生素D）。2组分别在试验开始、试验2w、8w和12w时测量血矿物质、25（OH）维生素D3和Ⅰ型胶原C端肽(CTX)水平。结果 对照组女性平均年龄为63±4.6岁,高钙强化奶（HCM）组女性平均年龄为62±3.8岁。所测得的每日钙摄入量的基线值为HCM组260~482mg,对照组为252~692mg。12w后HCM组的平均身体质量指数（BMI）显著降低（P<0.05）。HCM组的血25（OH）维生素D3水平显著提高（33.13~39.49 nmol/L）,而对照组则维持不变（29.27~28.21nmol/L）。HCM组的血CTX水平在0w和2w之间下降了25%,并保持不变直至第12w。从第2w开始HCM组和对照组相比血CTX水平就出现了显著差异（P<0.001）。结论 通过饮用含高钙和维生素D的强化奶（HCM）,在2至4w内能显著提高中国绝经妇女体内维生素D水平,并减少骨吸收破坏。如果长期持续地饮用含高钙和维生素D的强化奶（HCM）,能减少骨吸收和骨量丢失的风险,从而减少患骨质疏松和由此引起骨折的风险。     

变异型肥厚性心肌病一例的临床诊断解析

本文介绍1例心室中部肥厚性心肌病(HCM)的临床的诊断困惑,结合文献讨论多种心脏图像结合诊断和评估HCM及其变异。1基本资料患者女性,41岁,因"活动时胸闷3年"于2011年8月3日入院。病史:3年来活动时(登二楼)诱发胸闷、气短,休息缓解。1年前炒菜时晕厥1次,当时意识丧失,无抽搐,3分钟后清醒如常。2月前因胸闷当地医院急诊,心电图示:前壁导联ST段压低(0.1mv)伴T     

微创Morrow联合二尖瓣缘对缘修复技术治疗肥厚型梗阻性心肌病

<正>肥厚型心肌病(hypertrophic cardiomyopathy,HCM)是一种常见的以左心室壁不对称性肥厚为解剖特征的遗传性心肌病，在成年人群中HCM的发病率在0.2%左右，约70%的HCM会发生左心室流出道梗阻(left ventricular outflow tract obstruction,LVOTO)进展成肥厚型梗阻性心肌病(hypertrophic obstructive cardiomyopathy,HOCM)^[1-3]。左心室流出道梗阻和二尖瓣收缩期前向运动(systolic anterior motion,SAM)是HOCM病人的典型血流动力学表现^[4-5]。对于药物治疗无效的HOCM，室间隔心肌切除术(Morrow手术)是目前治疗的金标准^[6-9]。然而，大多数HOCM病人合并有不同程度的二尖瓣关闭不全(mitral regurgitation,MR)，单纯应用Morrow手术是否可以同时消除合并的MR，目前仍存在争议^[10-12]。     

肥厚型梗阻性心肌病外科治疗理念与技术浅析

<正>肥厚型心肌病(hypertrophic cardiomyopathy,HCM)是一种常见的遗传性心脏疾病，早前的数据认为人群中发病率为1/500，近年来由于基因检测和高敏感度心脏影像检查手段的大规模应用，估计HCM的发病率可能高达近1/200^[1-2]。约75%的HCM病人会出现左心室流出道梗阻(left ventricular outflow tract obstruction,LVOTO),LVOTO会导致活动能力下降、黑矇、晕厥、心衰甚至猝死，对病人的生活质量和生存产生不良影响^[3]。流出道梗阻的发生机制曾经被认为单纯是由室间隔肥厚引起，但随着影像学技术的进步，人们发现了更多的解剖学因素参与到这个病理过程^[4]。目前普遍认为肥厚型梗阻性心肌病(hypertrophic obstructive cardiomyopathy,HOCM)是一种异质性很强的疾病，在基因变异、解剖特征、临床表现及治疗策略等方面均存在较大的个体差异。     

梗阻性肥厚型心肌病的外科治疗：单中心5年421例经验总结

目的探讨外科治疗梗阻性肥厚型心肌病(HCM)的早中期效果并总结经验。方法回顾性收集2017年1月至2021年12月在复旦大学附属中山医院心脏外科接受手术治疗的421例梗阻性HCM患者的围手术期资料和术后随访结果。男性207例, 女性214例, 年龄(56.5±11.7)岁(范围:19～78岁)。术前心功能纽约心脏病学会分级Ⅱ级45例, Ⅲ级328例, Ⅳ级48例。58例为隐匿梗阻性HCM, 占13.8%(58/421)。257例合并中度及以上二尖瓣反流, 56例存在二尖瓣器质性病变。手术由多学科团队共同完成, 心脏超声医师参与术前规划和术中监测, 确定合理的二尖瓣处理策略。338例患者接受单纯室间隔心肌切除术, 59例同期接受二尖瓣手术。355例为单一经主动脉切口入路, 51例经右心房-房间隔/房间沟入路。术中选用长柄微创手术器械, 识别并处理二尖瓣瓣下异常结构。手术前后数据的比较采用配对t检验、配对秩和检验、χ2检验或Fisher确切概率法。结果单纯室间隔心肌切除术的主动脉阻断时间为(34.3±8.5)min(范围:21～94 min)。18例患者术中发生不良事件并即刻再次手术, 包...     

1例合并肥厚型心肌病患者whipple手术的麻醉处理及体会

<正>肥厚型心肌病(hypertrophic cardiomyopathy,HCM)是一种病因未明的常染色体遗传病,以心肌非对称性肥厚、左室充盈受阻及舒张期顺应性下降等为基本病征~([1])。由于其特殊的病理生理改变,使此类患者围术期脏器功能衰竭的风险显著     

左室肥厚的血透患者左心室舒张功能的观察

透析(HD)患者左室肥厚(LVH)发生率高达75％,我们采用超声心动图(UCG)对伴左室肥厚的尿毒症维持血透患者和其它引起LVH的高血压性心脏病和肥厚性心肌病的左室舒张功能作系统比较。 对象与方法 1.对象:(1)HD组30例,经UCG诊断符合LVH(室间隔 左室后壁≥24mm),20例男女各10例,年龄53±14岁。(2)高血压性心脏病(HHD)组11例,男10例,女1例,年龄57±10岁。(3)肥厚性心肌病(HCM)组11例,男6例,女5例,年龄51±12岁。(4)正常对照(N)组11例,男8例,女3例,年龄46±16岁。     

Becker muscular dystrophy-related cardiomyopathy: a favorable response to medical therapy.

Patients with Becker muscular dystrophy-related cardiomyopathy typically survive into their 30s, when they succumb to complications of cardiomyopathy or receive heart transplants. The cardiomyopathy is related to an abnormality in the protein dystrophin; no successful medical responses have been reported to date. We describe a patient with Becker muscular dystrophy-related cardiomyopathy in whom we noted improvement of cardiac status with carefully titrated medical therapy. Successful therapy suggests a trial of medical therapy may be beneficial when patients with Becker muscular dystrophy-related cardiomyopathy are evaluated for transplant.     

Two cases of left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve due to valvular cardiomyopathy during operation

We report two cases of left ventricular outflow tract (LVOT) obstruction with systolic anterior motion (SAM) of anterior mitral valve due to specific (secondary) cardiomyopathy during operation. The LVOT obstructions occurred and led to hypotension just after the induction of anesthesia in one case, and following the administration of nicardipine in another case. In both cases, preoperative diagnosis of the specific cardiomyopathy was not made. We revealed the LVOT obstruction with SAM using a transesophageal echocardiography (TEE) when the unstable hemodynamics developed. After the operations the valvular cardiomyopathy without LOVT obstruction was diagnosed by a cardiologist. Careful management is required including the TEE monitoring when we anesthetize a patient who is complicated not only with idiopathic cardiomyopathy but also with specific cardiomyopathy such as valvular or hypertensive cardiomyopathy because both types of cardiomyopathy develop LVOT obstruction with SAM in some pathophysiological conditions during operation.     

Levosimendan im septischen Schock bei Tako-Tsubo-Kardiomyopathie

As a stress-induced disease, takotsubo cardiomyopathy can also occur in septic syndromes; however, the hemodynamic management is fundamentally different from the treatment approaches for classical septic cardiomyopathy, as beta mimetics can increase the heart failure symptoms in takotsubo cardiomyopathy. This article reports the case of an 82-year-old female patient who presented with acute abdomen due to adhesion ileus and takotsubo cardiomyopathy, developed severe septic shock with peritonitis and could be successfully hemodynamically stabilized with levosimendan.     

Gestante a término con miocardiopatía no compactada

Non-compaction cardiomyopathy, a genetic primary cardiomyopathy, is being increasingly diagnosed. Pregnant women with non-compaction cardiomyopathy are more susceptible to complications, such as heart failure, arrhythmias and embolic events. This paper reports the case of a pregnant woman with non-compaction cardiomyopathy under treatment and asymptomatic, who received epidural analgesia during labor and delivery. The clinical course is described and a brief review is presented.     

Hypertensive heart disease and cardiomyopathy in blacks. Diagnostic confusion.

Four Black South African patients, representative of a larger group, are described in detail. The common features were long periods of observation, multiple hospital admissions in both normotensive and hypertensive cardiac failure, hypertensive retinopathy and good renal function. All had been diagnosed as having cardiomyopathy. Two of the patients in a normotensive phase became hypertensive after responding to therapy for heart failure. One patient with malignant hypertension showed the features of idiopathic cardiomyopathy at necropsy. These cases are regarded as evidence in favour of the hypothesis that many cases of cryptogenic heart disease (cardiomyopathy, congestive cardiomyopathy, idiopathic cardiomegaly) are in fact cases of hypertension presenting with normotensive cardiac failure.     

Arrhythmic cardiomyopathy. Case report

An 11 year-old boy was admitted with incessant sinus node reentrant tachycardia and secondary dilated arrhythmic cardiomyopathy, treated by radiofrequency ablation. Two years later he was admitted with incessant automatic atrial tachycardia and arrhythmic cardiomyopathy; a second catheter ablation procedure failed, but the third one, performed four month later, was successfully and resulted in a restoration of a normal sinus rhythm and a complete regression of arrhythmic cardiomyopathy.     

Sulprostone for postpartum hemorrhage in a parturient with a history of Tako-tsubo cardiomyopathy

A 32 year old parturient with a history of Tako-tsubo cardiomyopathy, who suffered from postpartum hemorrhage for which sulprostone was administered without any adverse events, is reported. Anesthetic considerations related to the management of patient with a history of Tako-tsubo cardiomyopathy, especially triggers that may cause a recurrence, are described. The potential deleterious effects of sulprostone in a patient with a history of Tako-tsubo cardiomyopathy are discussed.     

Anaesthetic management of a parturient with dilated cardiomyopathy: A case report

Dilated cardiomyopathy (DCM) is a form of cardiomyopathy characterized by left ventricular or biventricular dilatation and impaired ventricular contractility. This results in systolic dysfunction of the heart with decreased left ventricular ejection fraction and progressive congestive cardiac failure. Pregnancy in patients with pre-existing dilated cardiomyopathy can flare up the disease and can be fatal. This case report describes the successful anaesthetic management of a parturient with dilated cardiomyopathy undergoing caesarean section under spinal anaesthesia.     

Œdème pulmonaire deux jours après une césarienne réglée

Two days after an elective Caesarean section, a 30-year-old woman suffered a pulmonary oedema. The diagnosis of cardiomyopathy was obtained with echocardiography, right heart catheterization and a myocardial biopsy. Peripartum cardiomyopathy is defined as a heart failure occurring in the last trimester of pregnancy or up to the third postpartum month and the lack of another etiology. The cause of myocarditis resulting in peripartum cardiomyopathy remains yet unknown. An immunosuppressive therapy can be associated with the treatment of the congestive heart failure.     

Mitral valve surgery in non-ischemic cardiomyopathy

Dilated cardiomyopathy is a disorder of the cardiac muscle in which myocyte cytoskeletal weakness leads to ventricular dilatation and congestive cardiac failure. Most commonly, the etiology of non-ischemic cardiomyopathy is unknown (idiopathic) and, in our practice, the second most common cause is advanced valvular heart disease. Functional mitral valve regurgitation occurs in up to 40% of patients with heart failure due to dilated cardiomyopathy and contributes to a vicious cycle of volume overload, further left ventricular dilatation, and worsening mitral valve regurgitation and heart failure. Surgical management of mitral valve regurgitation in dilated cardiomyopathy may carry a high risk and can be very challenging. However, operative risk is mitigated by continued vigorous medical management and judicious perioperative care. For example, at our Clinic, mortality for mitral valve repair or replacement in 43 patients with non-ischemic cardiomyopathy having operation between 1993 and 2002 was 2.3%. Additional procedures to reverse cardiac remodeling have not proven to be uniformly successful and continue to undergo scientific scrutiny. Clinical outcome of mitral valve surgery in non-ischemic dilated cardiomyopathy compares well with cardiac transplantation in the early-to-intermediate term, but the long-term results are less satisfactory. For our patients having mitral valve repair, the 1-, 3-, and 5-year survivorships were 84%, 80% and 33%. Evolving technology and research that focus on methods of altering or reversing cardiomyopathy; e.g., cell transplant, may have significant impact on the future management of this debilitating illness.