梗阻性肥厚型心肌病的介入和外科手术治疗及进展

肥厚型心肌病是一种最为常见的心血管遗传性疾病,主要病因是编码肌小节蛋白或肌小节相关结构蛋白的基因变异,目前肥厚型心肌病已成为青少年猝死的头号病因,分为梗阻性肥厚型心肌病和非梗阻性肥厚型心肌病,梗阻性肥厚型心肌病相对非梗阻性肥厚型心肌病死亡率更高,诊治更加困难。现从梗阻性肥厚型心肌病的治疗角度出发,对以往及近期有关的治疗手段尤其是介入和手术治疗方面的进展做一综述。     

肥厚型梗阻性心肌病的药物治疗进展

肥厚型梗阻性心肌病的治疗目的主要是控制症状、预防猝死;治疗方法以药物治疗为主,包括β阻滞剂、维拉帕米、丙吡胺等.近年亦涌现出一些新的治疗肥厚型梗阻性心肌病的药物,但其效果尚待临床试验的验证.现对肥厚型梗阻性心肌病的药物治疗作一综述.     

肥厚型心肌病

肥厚型心肌病是一种常染色体显性遗传性心血管疾病。伴有左心室流出道梗阻的肥厚型心肌病是梗阻性肥厚型心肌病。现就肥厚型心肌病的病因和病理生理学、临床表现、诊断和治疗做一综述。     

心脏超声技术在肥厚型梗阻性心肌病化学消融中的运用

肥厚型梗阻性心肌病化学消融术是近年来发展起来的一项治疗肥厚型心肌病的新技术,由于和开胸手术相比具有创伤小,效果相当,因此该技术在临床上的使用呈逐年增加的趋势。该术式成功的关键在于准确的定位及可控性的消融梗阻部分心肌,其中心脏超声技术在肥厚型梗阻性心肌病化学消融术的术前诊断、术后随访中扮演了重要角色,特别是超声心肌造影技术在化学消融术的术中监测中起到了更为重要的作用,现就心脏超声技术在肥厚型梗阻性化学消融术中的运用做一综述。     

部分室间隔切除治疗肥厚型梗阻性心肌病

采用部分肥厚室间隔切除的方法治疗肥厚型梗阻性心肌病10例,1例同期行二尖瓣置换术。手术均获成功,无死亡者。认为部分肥厚室间隔切除治疗肥厚型梗阻性心肌病能有效解除左室流出道梗阻,效果满意。     

静脉注射美托洛尔对肥厚型心肌病左心室功能的影响

目的:应用频谱多普勒超声心动图技术,定量观测肥厚型梗阻性心肌病和肥厚型非梗阻性心肌病患者经静脉注射美托洛尔前后左心室功能和左心室流出道压力阶差的变化,并观察血流动力学的变化,探讨静脉注射美托洛尔对肥厚型心肌病左心室功能的影响。方法:应用PHILIPS-SONOS7500型彩色多普勒超声诊断仪,测量用药前和用药后10分钟肥厚型梗阻性心肌病组(n=33)和肥厚型非梗阻性心肌病组(n=26)患者左心室功能各指标,并监测用药过程中的血流动力学变化。结果:肥厚型梗阻性心肌病组患者用药后较用药前左心室舒张功能明显改善,左心室流出道(LVOT)明显增宽(P<0.05),左心室流出道压力阶差(LVOTPG)明显下降(P<0.05),EF值无明显变化(P>0.05);肥厚型非梗阻性心肌病组患者用药后较用药前上述各指标无明显变化(P>0.05)。两组的心率、收缩压、舒张压用药后较用药前均明显降低(P<0.05),有显著差异。结论:静脉注射美托洛尔能够快速改善肥厚型梗阻性心肌病组患者的左心室舒张功能,改善临床症状,明显减轻左心室流出道梗阻,降低压力阶差,明显降低两组的血压、心率,影响其血流动力学;而对肥厚型非梗阻性心肌病组患者无明显作用,对两组的收缩功能均无明显影响。     

肥厚型梗阻性心肌病经皮心内膜射频消融治疗进展

肥厚型心肌病是一种常见的以室间隔不对称肥厚为特征的遗传性心脏病,目前无根治方法。室间隔肥厚常导致左心室流出道压力阶差升高,诱发左心室流出道梗阻,称为肥厚型梗阻性心肌病,梗阻严重者可发生晕厥、猝死。肥厚型梗阻性心肌病的治疗方法包括药物治疗、外科手术治疗及介入治疗。对于药物治疗效果不佳的患者,可选择外科手术及介入治疗。肥厚型梗阻性心肌病经皮心内膜射频消融术作为一种介入治疗,近10年开始在临床中应用,初步研究证实其可有效改善症状和心功能,降低左心室流出道压力阶差,安全性好,但同时仍有一些问题亟待解决。     

起搏治疗肥厚梗阻性心肌病的疗效观察

肥厚型心肌病是一种具有明显遗传倾向的心肌病,其中有左心室流出道梗阻者称为肥厚璎梗阻性心肌病.肥厚型梗阻性心肌病是严重威胁人类身体健康和造成心脏性猝死的心血管疾病之一.晕厥及猝死可以为该病的首发症状.预后不佳.药物治疗不能完全缓解症状.     

超声声学定量技术对肥厚型心肌病患者右心室功能的评价

目的应用声学定量技术(AQ)评价非对称性肥厚型心肌病(包括梗阻性和非梗阻性)患者右室收缩与舒张功能。方法应用声学定量技术分别对2004年7月至2005年10月辽宁省人民医院27例肥厚型梗阻性心肌病(HOCM)患者、35例肥厚型非梗阻性心肌病(HNCM)患者和30例正常对照组人群的标准心尖四腔心切面观进行分析。结果与对照组相比,肥厚型心肌病(包括梗阻型和非梗阻型)右室峰值快速充盈率与右房峰值快速充盈率之比(PRFR/PAFR)、右室快速充盈容积与右房收缩充盈容积之比(RF/AF)明显减低(P<0.05)。而右心室收缩功能指标EF与正常组相比差异无显著性。结论AQ为定量评价肥厚型心肌病患者的右室功能提供了新的方法。     

心尖肥厚型心肌病与典型肥厚型心肌病的临床特点比较

目的对心尖肥厚型心肌病与典型厚型心肌病的临床特点进行分析。方法选取我院2010年1月~2016年1月收治的典型肥厚型心肌病患者120例作为研究对象,对其临床资料采取回顾分析的方式进行分析。将其分为三组进行比较。心尖肥厚型心肌病患者40例,(一组)。然后根据典型肥厚型心肌病患者是否存在着流出道梗阻将剩下的患者分为梗阻性肥厚型心肌病患者35例(二组)以及非梗阻性肥厚型心肌病45例(三组)。将三组患者的心脏超声以及临床特点进行对比。结果一组患者的左心室尖部厚度为(21.2±3.5)mm,没有患者发生梗阻现象,室壁运动出现异常。结论心间肥厚型心肌病和典型肥厚型心肌病在临床上的特点是具有一定差异性的,而梗阻性肥厚型心肌病于非梗阻性肥厚型心肌病之间的差异不大,心脏超声检查阳性及心电图胸导联上典型的GNT可以确诊为心尖肥厚型心肌病。     

HLA-DRW4 antigen linkage in patients with hypertrophic obstructive cardiomyopathy

To determine the association of histocompatibility (HLA) genes in patients with hypertrophic cardiomyopathy, we determined HLA-A, HLA-B, HLA-C, and HLA-DR specificities in 33 Japanese patients (15 with the obstructive type of hypertrophic cardiomyopathy, and 18 with the nonobstructive type). HLA-DRW4 was found in 73% of patients with hypertrophic obstructive cardiomyopathy, as compared to 33% of 144 normal controls (p < 0.005). HLA-DRW4 occurred in 33% of those with hypertrophic nonobstructive cardiomyopathy, and there was no significant difference as compared with controls. Thus, hypertrophic obstructive cardiomyopathy is associated with genes in the HLA-DR region and immunogenetic factors linked to HLA appear to play a role in the pathogenesis. This work is the first attempt at demonstration of HLA-DR antigen in hypertrophic cardiomyopathy.     

Mechanocardiographic evaluation of left ventricular diastolic function in hypertrophic cardiomyopathy and hypertensive cardiomyopathy

Usefulness of computerized mechanocardiography and echocardiography in the diagnosis of impaired left ventricular diastolic function is shown in a study comparing 17 hypertrophic obstructive cardiomyopathy and 17 hypertensive cardiomyopathy patients to 20 normal subjects. Mechanocardiography allows the evaluation of three different parameters of diastolic function: isovolumic relaxation evaluated by S2a-O or better by t-dr/dt and dr/dt/A2 ratio, left ventricular compliance by the A/H ratio and time of rapid filling. All the parameters are impaired in both pathological populations. Isovolumic relaxation being more depressed in hypertensive cardiomyopathy and duration of rapid filling being prolonged especially in the hypertrophic obstructive cardiomyopathy patients. Although the differences are small indicating only trends computerized mechanocardiography gives some evidence for difference in the alteration of diastolic function in hypertrophic obstructive cardiomyopathy and hypertensive cardiomyopathy.     

Echocardiography in the diagnosis of hypertrophic obstructive cardiomyopathy

The role of echocardiography in the diagnosis of hypertrophic obstructive cardiomyopathy is reviewed. Salient features include a narrowed left ventricular outflow space, the increased thickness of the interventricular septum and the systolic anterior motion of the anterior mitral leaflet. The last may be present at rest, or it may be provoked with appropriate maneuvers. When all three features are demonstrated, a diagnosis of hypertrophic obstructive cardiomyopathy can be made with confidence. In the absence of resting or provocable systolic anterior motion, a diagnosis of nonobstructive cardiomyopathy may be entertained. An additional important echocardiographic feature of hypertrophic obstructive cardiomyopathy consists of an interventricular septal to posterior wall ratio in excess of 1.5, suggesting the asymmetric distribution of hypertrophy. Inert interventricular septum, aortic valve preclosure and a reduced diastolic slope of the anterior mitral leaflet are frequently present, but not specific for the condition. When hypertrophic obstructive cardiomyopathy is associated with other unrelated diseases, the index of suspicion may be raised even in the absence of all the “classic” echo signs.     

Provocation of left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy

Several provocation maneuvers are described in hypertrophic cardiomyopathy to Doppler echocardiographically distinguish the obstructive from the non obstructive type. No data are available about the value of orthostasis testing in comparison with nitrate application in this disease. In this study, 16 consecutive patients with hypertrophic cardiomyopathy were examined. 11 patients with hypertrophic cardiomyopathy were classified as obstructive, 5 patients with hypertrophic cardiomyopathy as non obstructive. Normal left ventricular outflow tract velocities as detected by the Doppler method were defined as < 2,0 m/s.Doppler echocardiographic measurements were performed after 10 minutes in supine position, within 10 minutes after head-up tilt and again, within 10 minutes in supine position. If systolic blood pressure during this examination exceeded 100 mm Hg 2,5 mg isosorbiddinitrate were sprayed sublingually. Measurements were done after 20 minutes in supine position and within 10 minutes after head-up tilt.Only in 7 of the 11 patients with hypertrophic obstructive cardiomyopathy maximal left ventricular outflow tract velocity in supine position measured > 2,0 m/s (2,2 ± 0,8). During head-up tilt, all patients showed increased values (3,8 ± 1,2 m/s). No differences in maximal left ventricular outflow tract velocity between head-up tilt and nitrate application in supine position (3,5 ± 1,4 m/s) were present. All patients with hypertrophic non obstructive cardiomyopathy showed maximal left ventricular outflow tract velocities < 2,0 m/s in every step of the examination.Consequently, orthostasis testing was able to identify all patients with hypertrophic obstructive cardiomyopathy and demonstrated a diagnostic value similar to nitrate application.     

Hypertrophic obstructive cardiomyopathy and Friedreich's ataxia. Report of a case and review of literature

A case of Friedreich's ataxia with evidence of hypertrophic obstructive cardiomyopathy involving both ventricles is reported. A review of the previously reported cases leads us to believe that this association is not merely accidental, but that Friedreich's ataxia and hypertrophic obstructive cardiomyopathy are parts of the same syndrome.     

Acute myocardial infarction in a case of obstructive cardiomyopathy of the left ventricle (author's transl)

A case of acute myocardial infarction in 64 year old man with idiopathic hypertrophic obstructive cardiomyopathy of left ventricle is described. The Authors emphasize the rarity of association and that the diagnosis of obstructive cardiomyopathy in the elderly is always almost misinterpreted. This depends on the poor specificity of clinical and phonocardiographic findings, both basal and under pharmacological tests. The Authors point out that in adult patients with left ventricular idiopathic obstructive cardiomyopathy who must be operated also selective coronary angiography should be performed.     

Stetho-acoustic cardiomyopathy--characteristics in cardiomyopathies studied by echocardiography

In 4 different forms of cardiomyopathy which had been diagnosed echocardiographically we performed stethoacoustic investigations. Hereby each of these 4 different forms of cardiomyopathy developed a specific noise. In the patients with hypertrophic non-obstructive cardiomyopathy we found a mesosystolic to protomesosystolic interval murmur in 2 L 1 and called it hypertrophic murmur. In the apical hypertrophic cardiomyopathy we found a mesosystolic murmur above the apex and called it apical hypertrophic murmur. In the obstructive cardiomyopathy (hypertrophic obstructive cardiomyopathy) we found a mesotelesystolic murmur in 4 L 3 and called it obstruction murmur. In the dilatative cardiomyopathy (COCM) existed an immediate systolic murmur above the apex and we called it dilatation murmur.     

Mid-ventricular obstructive hypertrophic cardiomyopathy associated with an apical aneurysm and sustained ventricular tachycardia: a case report]

A 60-year-old woman presented with mid-ventricular obstructive hypertrophic cardiomyopathy associated with an apical aneurysm and sustained ventricular tachycardia. She was admitted because of drug refractory ventricular tachycardia. She had been treated with several antiarrhythmic agents, including amiodarone, but symptomatic episodes had continued. Echocardiography, magnetic resonance imaging, and left ventriculography showed mid-ventricular obstructive hypertrophic cardiomyopathy with an apical aneurysm. Electrophysiological study easily reproduced sustained pleomorphic ventricular tachycardia, polymorphic ventricular tachycardia, and ventricular fibrillation. The patient underwent implantation of a cardioverter-defibrillator. The relationship between mid-ventricular hypertrophic cardiomyopathy and apical aneurysm is unknown, but mid-ventricular hypertrophic cardiomyopathy is one of the causes of severe ventricular arrhythmias and sudden death.     

Polyarteritis nodosa and hypertrophic obstructive cardiomyopathy. A true association?

A 60-year-old man with polyarteritis nodosa under treatment presented with syncope. Echocardiography demonstrated hypertrophic obstructive cardiomyopathy; coronary arteriography revealed normal findings, and Holter monitor showed episodes of non-sustained ventricular tachycardia. This is the first report of hypertrophic obstructive cardiomyopathy developing in a patient with polyarteritis nodosa. Further studies should examine whether a true association exists.Abbreviations ESR Erythrocyte sedimentation rate - PAN Polyarteritis nodosa     

Staphylococcus hominis native mitral valve bacterial endocarditis (SBE) in a patient with hypertrophic obstructive cardiomyopathy

There are several species of coagulase-negative Staphylococci (CoNS) that are part of the normal skin flora and are relatively noninvasive/low virulence organisms. CoNS are important pathogens in patients with prosthetic devices and are the most common pathogen associated with prosthetic valve endocarditis. CoNS native valve infective endocarditis (IE) is rare. Patients with hypertrophic obstructive cardiomyopathy and an outflow pressure gradient greater than 30 mm Hg are predisposed to IE. There has been only one reported case of non-mitral valve IE due to CoNS in a patient with hypertrophic obstructive cardiomyopathy. To the best of our knowledge, we report the first case of Staphylococcal hominis mitral valve endocarditis in a patient with hypertrophic obstructive cardiomyopathy.