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。     

乙醇室间隔消融术治疗肥厚型梗阻性心肌病研究进展

肥厚型梗阻性心肌病是青年人心源性猝死的最常见原因。严重者应及时治疗,最适病人的筛选、影像技术的辅助、并发症的控制和操作技术的提升将使乙醇室间隔消融术成为治疗肥厚型梗阻性心肌病一种很有前途的方法。     

肥厚型梗阻性心肌病中二尖瓣前叶收缩期前向运动的机制与处理策略

二尖瓣前叶收缩期前向运动(systolic anterior motion,SAM)常出现于肥厚型心肌病中,但其机制十分复杂.室间隔肥厚、瓣叶冗长、异常乳头肌、腱索松弛、二尖瓣对合点至室间隔距离短以及高流速均被认为是肥厚型梗阻性心肌病中SAM的独立影响因素.目前国内外不同肥厚型心肌病治疗中心对SAM的处理策略各有千秋,...     

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。     

间隔消融术对比心肌切除术治疗梗阻性肥厚型心肌病研究进展

先前认为室间隔心肌部分切除术是治疗药物难治性梗阻性肥厚型心肌病的金标准,近年来,经皮经腔间隔心肌消融术由于创伤小,操作简单,获得广泛开展并取得了较好的疗效。但究竟哪种是更好的治疗方法,一直存在争论。现就目前药物难治性梗阻性肥厚型心肌病的两种方法疗效比较作一综述。     

环球要刊巡览

<正>梗阻性肥厚型心肌病室间隔切除术后生存率的性别差异为研究梗阻性肥厚型心肌病(HCM)患者室间隔切除术后生存率的性别差异,梅奥诊所的Meghji教授等对1961年1月至2016年4月接受室间隔切除术的2 506例成年患者进行单中心回顾性研究,主要终点为生存率。研究发现,与男性     

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化学消融术后电风暴一例

经皮经室间隔化学消融术(PTSMA)是肥厚型梗阻性心肌病患者的一种重要治疗手段,其术中和术后的主要并发症为束支阻滞和三度房室阻滞.现报道本院1例肥厚型梗阻性心肌病PTSMA后出现三度房室阻滞伴电风暴病例.     

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.     

梗阻性肥厚型心肌病治疗方法的现状与进展

梗阻性肥厚型心肌病是一种以室间隔非对称性肥厚、二尖瓣前叶收缩期前向运动征、左室流出道梗阻和不同程度的二尖瓣反流为主要特征的常染色体显性遗传性疾病。目前该病可通过药物、外科手术、经皮室间隔化学消融术等方式治疗,从而改善患者的临床症状,减少并发症及预防心源性猝死。现就梗阻性肥厚型心肌病治疗方法的现状与进展做一综述。     

Monomorphic ventricular tachycardia: a late complication of percutaneous alcohol septal ablation for hypertrophic cardiomyopathy

Percutaneous alcohol septal ablation has emerged as a promising treatment option for patients with symptomatic hypertrophic obstructive cardiomyopathy. Although the procedure involves an alcohol-induced myocardial infarction and results in a substrate potentially conducive to re-entrant tachyarrhythmias, late-occurring ventricular arrhythmias have not been described. We report a case of monomorphic ventricular tachycardia occurring several days after alcohol septal ablation. Patients with hypertrophic cardiomyopathy undergoing alcohol septal ablation should be considered for prophylactic placement of implantable cardioverter defibrillator.     

Prevalence of dignostic abnormalities in patients with genetically transmitted asymmetric septal hypertrophy

Echocardiographic studies were performed in 100 patients from a general population with cardiac disease and in 33 patients with classic hypertrophic obstructive cardiomyopathy and 116 of their first degree relatives. The findings were compared with those in 35 normal persons. The prevalence rate of asymmetric septal hypertrophy (ventricular septal to free posterior wall ratio greater than 1.3) was 8 percent in the general population with heart disease. No further clinical or echocardiographic evidence was found for a familial disease. The ventricular septal to free posterior wall ratios for this group and the normal subjects had a unimodal distribution curve. All patients with hypertrophic obstructive cardiomyopathy demonstrated asymmetric septal hypertrophy, a decreased systolic septal thickening of less than 25 percent and a characteristic left ventricular shape in the cross-sectional echocardiogram. The ventricular septal to posterior wall ratios in their 116 relatives had a bimodal distribution curve; in 35 relatives the ratio indicated asymmetric septal hypertrophy and in 81 the ratio was normal. In addition, all 35 relatives with echocardiographic evidence of asymmetric septal hypertrophy had decreased systolic septal thickening (less than 25 percent) and in 17 the echocardiographic left ventricular shape was similar to that in patients with hypertrophic obstructive cardiomyopathy. In contrast, the 81 relatives who had no asymmetric septal hypertrophy had normal systolic septal thickening and a normal left ventricular shape. The clinical examination, electrocardiogram and chest X-ray film were less sensitive than the echocardiogram in detecting diagnostic abnormalities in the 35 relatives with asymmetric septal hypertrophy.It is concluded that echocardiographically assessed asymmetric septal hypertrophy can be considered the anatomic marker for hypertrophic cardiomyopathy only when, in addition, a decreased systolic septal thickening and, to a lesser degree, an abnormal left ventricular shape are present. The asymmetric septal hypertrophy in these cases probably represents the anatomic expression of a genetic defect that has an autosomal dominant pattern of inheritance. In so-called “borderline” cases with echocardiographic signs of an abnormal ventricular septal to posterior wall ratio, a definitive clinical diagnosis of hypertrophic cardiomyopathy could be made only after echocardiographic screening of family members for the presence of asymmetric septal hypertrophy.     

B-type natriuretic peptide after percutaneous transluminal septal myocardial ablation

BACKGROUND: Plasma level of B-type natriuretic peptide is a sensitive marker of left ventricular dysfunction and the level is markedly elevated in patients with hypertrophic obstructive cardiomyopathy. Percutaneous transluminal septal myocardial ablation, a catheter-based treatment of hypertrophic obstructive cardiomyopathy, has been widely used as a new therapeutic option for the disease. This study was designed to evaluate clinical implications of natriuretic peptides after the new treatment. METHODS: Seven consecutive patients with hypertrophic obstructive cardiomyopathy unresponsive to usual medical treatments (age: 57.9+/-22.0 years) were enrolled in the study. Serial changes in atrial and B-type natriuretic peptide in plasma were examined after percutaneous transluminal septal myocardial ablation. RESULTS: Atrial and B-type natriuretic peptides levels (pg/ml, mean+/-S.D.) at baseline were higher in hypertrophic obstructive cardiomyopathy than in control (80.0+/-43.0 vs. 12.8+/-5.2, P<0.0001; 858.0+/-458.4 vs. 12.4+/-7.0, P<0.0001; respectively). Left ventricular outflow-tract pressure gradient (mmHg) immediately decreased from 115.3+/-23.3 to 30.6+/-12.4 (P<0.0001) after the treatment and concomitantly B-type natriuretic peptide level decreased (858.0+/-458.4 to 264.1+/-137.7, P=0.0084). The level re-increased and peaked at the 2nd day (634.4+/-429.6) and gradually decreased again until 4 weeks. Reduction rate of left ventricular outflow-tract pressure gradient between before and 4 weeks after percutaneous transluminal septal myocardial ablation positively correlated with that of B-type natriuretic peptide (r(2)=0.817, P=0.0053). Changes in atrial natriuretic peptide were not significant in contrast to those of B-type natriuretic peptide. CONCLUSIONS: Plasma B-type natriuretic peptide level could be useful to predict the effects of percutaneous transluminal septal myocardial ablation in patients with hypertrophic obstructive cardiomyopathy.     

Retrograde Percutaneous Closure of a Ventricular Septal Defect after Myectomy for Hypertrophic Obstructive Cardiomyopathy

In patients with hypertrophic obstructive cardiomyopathy, hemodynamically significant ventricular septal defect after septal myectomy is a rare sequela that warrants closure. Percutaneous closure provides a safer alternative to repeated sternotomy, which is associated with significant morbidity and mortality rates. We report a possibly unique case of successful retrograde percutaneous closure, with an AMPLATZER Muscular VSD Occluder, of an iatrogenic ventricular septal defect consequent to surgical therapy for hypertrophic obstructive cardiomyopathy.Key words: Cardiac catheterization/methods, cardiomyopathy, hypertrophic/therapy, heart septal defects, ventricular/therapy, iatrogenic disease, myectomy, postoperative complications, prosthesis implantation/methods, septal occluder device, ventricular septal defectPatients with hypertrophic obstructive cardiomyopathy (HOCM) are candidates for surgical myectomy or ethanol ablation if, despite medical therapy, they remain symptomatic with New York Heart Association functional class III disease and severe left ventricular outflow tract (LVOT) gradients.¹ A rare sequela of myectomy via the Morrow procedure is iatrogenic ventricular septal defect (VSD). Although surgical closure remains the mainstay of treatment for most VSDs, treatment of clinically significant postoperative residual VSDs remains a challenge. Percutaneous closure provides a safer alternative to reoperation.^2,3 We report a possibly unique case of successful retrograde percutaneous closure of an iatrogenic VSD, consequent to surgical therapy for HOCM, by means of an AMPLATZER® Muscular VSD Occluder (St. Jude Medical, Inc.; St. Paul, Minn).     

M mode echocardiography in hypertrophic cardiomyopathy: Diagnostic criteria and prediction of obstruction

To assess the reliability of the classic echocardiographic features in the heart with hypertrophic cardiomyopathy as criteria that differentiate it from normal heart and as predictors of outflow tract obstruction versus nonobstruction, 70 patients with clinical and angiographic evidence of hypertrophic cardiomyopathy were studied with M mode echocardiography. The diagnostic sensitivity and specificity of the classic features were assessed: ventricular septal thickness, 83 and 94 percent (sensitivity and specificity, respectively); ventricular septal amplitude of movement, 71 and 89 percent; ventricular septal thickness to left ventricular posterior wall ratio, 79 and 94 percent; left ventricular end-systolic dimension, 54 and 86 percent; septal-mitral valve distance at the onset of systole, 29 and 100 percent; systolic anterior motion of the mitral valve, 61 and 100 percent; and mid systolic closure of the aortic valve, 61 and 100 percent.No single M mode echocardiographic feature was consistently abnormal in hypertrophic cardiomyopathy. In nonobstructive hypertrophic cardiomyopathy, ventricular septal thickness greater than or equal to 13 mm (sensitivity 68 percent and specificity 94 percent) and ventricular septal thickness to posterior wall ratio greater than or equal to 1.5 (sensitivity 82 percent and specificity 94 percent) were the individual features with the greatest diagnostic value from the norm. Patients with obstruction at rest and labile obstruction (gradient only on provocation) had echocardiographically identical features. Ventricular septal thickness greater than or equal to 13 mm plus systolic anterior motion of the mitral valve or mid systolic closure of the aortic valve were the features that in combination best differentiated obstructive (resting and labile) from nonobstructive hypertrophic cardiomyopathy (sensitivity 82 percent and specificity 68 percent) and the heart with obstructive hypertrophic cardiomyopathy from the normal heart (sensitivity 82 percent and specificity 100 percent).     

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。     

Impact of percutaneous transluminal septal myocardial ablation on refractory paroxysmal atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy

Atrial fibrillation is commonly observed in patients with hypertrophic obstructive cardiomyopathy. Episodes of paroxysmal atrial fibrillation are often torturous and limit the quality of life by causing congestive heart failure, transient hypotension, or bradycardia. Control of paroxysmal atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy is considered to be important for symptomatic improvement and prevention of the development to chronic atrial fibrillation. The authors report on 3 patients with hypertrophic obstructive cardiomyopathy who suffered from paroxysmal atrial fibrillation despite receiving medical treatment using antiarrhythmic agents. However, after undergoing percutaneous transluminal septal myocardial ablation, the incidence of episodes became significantly less frequent. Percutaneous transluminal septal myocardial ablation is normally performed for attenuating left ventricular obstruction by reducing the systolic anterior motion of the mitral leaflet. However, in these patients, this procedure was also effective in preventing supraventricular arrhythmia, probably by improving left ventricular diastolic dysfunction, smooth blood inflow into the left ventricular, and decreasing the pressure stress against the left atrial wall.     

Drink more, and eat less: advice in obstructive hypertrophic cardiomyopathy

This report describes a series of symptomatic patients with obstructive hypertrophic cardiomyopathy with significant postprandial hemodynamic changes. This finding was identified by history, clinical examination, and echocardiography in 6 consecutive symptomatic patients referred for the evaluation of ventricular septal reduction therapy. Counseling these patients with dietary changes to include small frequent meals and to increase noncaffeinated fluid intake resulted in reductions in symptoms. In conclusion, severe symptoms in obstructive hypertrophic cardiomyopathy unresponsive to pharmacologic treatment frequently result in referral for definitive septal reduction therapy through surgery or, less frequently, alcohol septal ablation therapy. However, recognition of postprandial exacerbation in symptomatic patients may allow for nonpharmacologic dietary interventions that may obviate the need for more invasive therapies and their associated complications.     

Nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy: one-year follow-up

OBJECTIVE: The objective of this study is to evaluate the one-year outcome of the first 50 patients who underwent nonsurgical septal reduction for symptomatic hypertrophic obstructive cardiomyopathy at our institution. BACKGROUND: Left ventricular outflow tract obstruction is an important determinant of clinical symptoms in patients with hypertrophic obstructive cardiomyopathy. Nonsurgical septal reduction is a new therapy that has been shown to result in left ventricular outflow tract gradient reduction and resolution of symptoms immediately after the procedure and on midterm follow-up. METHODS: Fifty patients with hypertrophic obstructive cardiomyopathy who underwent nonsurgical septal reduction at our institution and completed 1-year follow-up are described. Complete history, physical examination, two-dimensional echocardiography with Doppler and exercise treadmill testing have been analyzed. RESULTS: The mean age of the study group was 53 +/- 17 years. All patients had refractory symptoms before enrollment. Ninety-four percent had class III or IV New York Heart Association class symptoms at baseline compared to none at 1 year (p < 0.001). The exercise duration increased by 136 s at 1 year (p < 0.021). Only 20% of patients were either receiving beta-blockers or calcium-channel blockers on follow-up. The resting left ventricular outflow tract gradient decreased from 74 +/- 23 mm Hg to 6 +/- 18 mm Hg (p < 0.01) and from 84 +/- 28 mm Hg to 30 +/- 33 mm Hg (p < 0.01) in patients with dobutamine-provoked gradient at one year. These changes are associated with decreased septal thickness and preserved systolic function. CONCLUSION: Nonsurgical septal reduction therapy is an effective therapy for symptomatic patients with hypertrophic obstructive cardiomyopathy with persistence of the favorable outcome up to one year after the procedure.     

Coronary fistula of the right ventricular outflow tract as a complication of combined transaortic-trans-right ventricular myectomy in hypertrophic obstructive cardiomyopathy

We report on a patient who showed a fistula between the first septal branch of the left anterior descending coronary artery and the right ventricular outflow tract, as a complication of combined trans-aortic right ventricular myectomy for treatment of hypertrophic obstructive cardiomyopathy (HOCM).