Effect of electroconvulsive therapy on cardiac rhythm, conduction and repolarization.

Because sympathetic stimulation has been implicated in the genesis of arrhythmias, we studied the effects on arrhythmias of electroconvulsive therapy (ECT). Fifteen psychiatric patients (male: 8, female: 7, age: 19-51, mean: 29.8) without known heart disease underwent 24-hout Holter recordings before, during, and after ECT (25 episodes). All patients were taking psychotropic drugs and received atropine (0.4-1.2 mg, mean: 1.1 mg IV), methohexital, and succinylcholine prior to ECT. Following ECT, mean maximum heart rate increased (106 +/- 3.2 to 142 +/- 6.0 beats/min, p less than .001), PR interval decreased (149 +/- 3.3 to 131 +/- 3.7 msec, p less than .001) and QTc interval increased (132 +/- 6.5 to 454 +/- 9.7 msec, p less than .001) compared to values obtained after atropine administration. Mean PVC or PAC frequently immediately after ECT or per 24 hours did not change significantly (PVC per 24 hours 6.8 +/- 3.2 to 10.4 +/- 6.4, NS; PAC per 24 hours 0.4 +/- 0.3 to 0.3 +/- 0.2, NS) and no complex arrhythmias were noted. Rate and PR changes suggest adrenergic effects of ECT and QTc increase may be due to imbalanced sympathetic discharge. Autonomic stimulation produced by ECT did not induce arrhythmias in these patients without heart disease. The possible antiarrhythmic role of psychotropic agents or premedication is unknown.     

Dual chamber pacemaker therapy for mid-cavity obstructive hypertrophic cardiomyopathy

Intracavitary LV obstruction is an important determinant of clinical outcome in hypertrophic cardiomyopathy (HCM). In a minority of patients the obstruction is at the level of the papillary muscles. Mid-cavity obstructive HCM may be associated with a distal LV aneurysm and a worse prognosis. It is often not amenable to standard cardiac surgeryfor LV outflow obstruction. The long-term effects (mean follow-up 4.8+/-2.9 years) of dual chamber (DDD) pacemaker therapy in 14 patients with mid-cavity obstructive HCM (mean age 34+/-16 years, range 15-65 years) were studied. Patients were evaluated by cardiac catheterization at baseline and 6 months to 1 year after receiving DDD pacemakers off all drug therapy. Symptoms were improved in all patients and NYHA functional class reduced from 2.8+/-0.1 to 1.9+/-0.4 (P < 0.0005). Intracavitary LV pressure gradients was reduced significantly (43+/-36 vs 84+/-31 mmHg at baseline, P < 0.0005). There was a significant associated reduction in apical LV systolic pressure (152+/-37 vs 188+/-34 mmHg, P < 0.001). In addition, there was a trend towards increased exercise tolerance (445+/-123 vs 396+/-165). Cardiac output and LV filling pressures were unchanged. In conclusion, chronic DDD pacing results in significant symptomatic and hemodynamic improvement in this uncommon but important subset of patients with obstructive HCM in whom the role of cardiac surgery is less well defined compared with the more typical outflow tract location of LV obstruction.     

Myocardial thallium defects in apical hypertrophic cardiomyopathy are associated with a benign prognosis

Background: We investigated whether myocardial thallium-201 defects in patients with apical hypertrophic cardiomyopathy are associated with an unfavorable clinical outcome, and additionally compared the presence of defects to echocardiography and angiography findings. Methods: Dipyridamole thallium-201 single photon emission tomography was performed in 26 apical hypertrophic cardiomyopathy patients, aged 41–78 (22 men, 6 women). Patients with or without perfusion defects were compared for echocardiographic measurements of wall thickness, chamber dimensions, and fractional shortening. Twelve patients underwent coronary angiography. The occurrence of cardiac events was evaluated during clinical follow-up for a mean period of 4.3 ± 1.9 years (range: 1.0–7.0 year). Results: Thirteen patients (50%) showed perfusion defects, which were predominantly reversible (fixed in 1) and mostly apical (9 of 13). Patients with and without defects did not differ in symptoms, EKG findings, echocardiographic morphology or fractional shortening (41 ± 4 vs. 41 ± 7%). Coronary angiography was normal in all patients in whom it was performed (nine with and three without defects). During follow-up, there were no deaths or myocardial infarction. Of the defect positive group, one case developed paroxysmal supraventricular tachycardia, and another had a cerebrovascular accident. Of the defect negative group, one case developed sick sinus syndrome, while another had a hospital admission for anginal pain. Conclusions: While reversible thallium defects in the absence of coronary artery disease occur frequently in patients with apical hypertrophic cardiomyopathy, the prognosis remains benign despite the presence of ischemia and there is no evidence for an association with adverse patient outcome.     

Device therapy in hypertrophic cardiomyopathy

With increasing awareness of the condition and particular attention being paid to family screening, the number of patients being diagnosed with hypertrophic cardiomyopathy is increasing. Although the majority of patients remain at low risk for sudden cardiac death, all patients need to undergo rigorous and ongoing risk factor stratification in order to best identify those at high risk. Although implantable cardioverter–defibrillators have proven to be effective in the prevention of sudden cardiac death, careful consideration of device implantation in high-risk patients is necessary in view of the potential for device complications and their impact on quality of life.     

肥厚性心肌病的冠状动脉造影分析

目的：了解肥厚性心肌病患者冠状动脉及其特征。方法：25例肥厚性心肌病患者行冠状动脉造影,观察其冠状动脉分布及特点,测量其各分支中段内径,并与同期行冠状动脉造影的正常人相比。结果：肥厚性心肌病患者冠状动脉供血呈左优势者占20％,各亚型间无明显判别：5例发现合并冠心病,其中1例行PTCA及冠脉内支架术,另1例植入永久性DDD起搏器治疗,1例左前降支中段发现冠脉肌桥;无冠状协脉病变的19例患者左主干,回旋支及右冠脉中段内径较正常者明显粗大（P＜005）,结论：肥厚性心肌病患者冠脉供血呈左优势者比较较高,其冠状动脉内径较正常者粗大,疑有合并冠心病者须行冠状动脉造影,对指导治疗有一定意义。     

肥厚梗阻性心肌病围手术期的护理

目的探讨肥厚梗阻性心肌病围手术期的护理经验。方法回顾性分析8例肥厚梗阻性心肌病围手术期护理方法。结果7例患者治愈出院,1例患者出现严重心率失常,合并肾功能衰竭死亡。结论做好术前护理,并根据患者的心理特点积极做好心理疏导。密切观察术后合并症并及时采取措施,对保证肥厚梗阻性心肌病的手术成功具有积极意义。     

Successful use of propranolol in migraine associated with electroconvulsive therapy

To date, there have been no reports on the use of propranolol in electroconvulsive therapy (ECT)-induced migraine; we describe a 32-year-old woman who was successfully treated with propranolol for this condition. Over a course of ECT, the patient developed increasingly severe migraine which was refractory to treatment with acetaminophen, codeine, and naproxen. Sumatriptan did not relieve the headache and aggravated the nausea. Successful migraine relief was achieved with a combination of propranolol and naproxen, administered before and after ECT. Propranolol reduced blood pressure and decreased the heart rate, measured before and immediately after ECT. Propranolol, possibly in combination with naproxen, may be useful in both acute and prophylactic treatment of post-ECT migraine.     

Surgical management of obstructive hypertrophic cardiomyopathy: the gold standard

While medication is the first line of therapy in obstructive hypertrophic cardiomyopathy, patients who have symptoms refractory to medical treatment or asymptomatic patients with high resting gradients (≥30 mmHg) may require septal myectomy. Surgical septal myectomy can be performed safely, with excellent survival, relief from symptoms and low morbidity. Alcohol septal ablation is an alternative to surgical treatment, but late outcomes are uncertain. Although both methods of septal reduction relieve left ventricular outflow tract gradients and improve functional status, the need for permanent pacing appears higher with alcohol ablation compared with surgical myectomy. As our understanding of obstructive hypertrophic cardiomyopathy continues to grow, the indications for intervention will evolve. In our practice, septal myectomy remains the gold standard for treatment of obstructive hypertrophic cardiomyopathy.     

目标基因捕获测序技术检测1例肥厚型心肌病致病基因突变

目的通过靶向捕获高通量测序技术初步筛查肥厚型心肌病(HCM)致病基因突变。方法收集1例HCM患者临床信息和外周血,并提取基因组DNA,制备DNA全基因组文库。挑选导致HCM的8个候选基因,用GenCap基因序列捕获技术靶向富集该患者外周血DNA候选基因并行高通量测序。通过生物信息学分析筛选致病突变。用Sanger测序来验证相应致病基因突变位点。结果目标基因靶向捕获测序结果经与公共数据库和内部健康人测序数据库对比,发现致病基因突变位点MYBPC3 D770N,该致病基因突变与Sanger测序结果一致。结论用目标基因靶向捕获测序技术可实现对HCM致病基因突变的初步筛查,对HCM的临床基因诊断具有潜在的应用价值。     

Ventricular Arrhythmias in Dilated Cardiomyopathy

Although prognosis of dilated cardiomyopathy (DCM) has improved due to advances in diagnosis and therapy, still too many sudden cardiac deaths occur in DCM. Spontaneous ventricular ectopy is a very common finding in patients with DCM, but the prognostic significance of Holter monitoring remains controversial. Other noninvasive methods, e.g., late potentials and QT dispersion, have not yet contributed to the evaluation of prognosis for arrhythmogenic events in DCM. Programmed ventricular stimulation has been repeatedly used to stratify long-term prognosis, yet satisfactory data are still missing as many deaths occur in patients without inducible arrhythmias. Several prognostic studies are still in progess, and preliminary data for the use of ICDs already appear to be promising. In patients with poor left ventricular function and ICDs in situ, prognosis is determined by progression of heart failure. Heart transplantation may be the ultimate therapeutic instrument for end-stage heart failure patients. For patients with advanced DCM and increased risk for malignant arrhythmias who are unsuitable for orthotopic heart transplantation, the combined therapy with an ICD and dynamic cardiomyoplasty may be an alternative treatment.     

The death of electroconvulsive therapy

In Italy, where it began more than 62 years ago, ECT has almost been abolished. In some countries, however, ECT is still used inappropriately, particularly in elderly patients. There is no medical, moral, or legal justification for ECT, and the new requirements of modern psychiatric practice can all be achieved with-out it. Like prefrontal lobotomy and all previous shock treatments, ECT is nonviable. The death of ECT will help promote mental health and put the treatment where it belongs—in the archaeology of science.     

超声左心室压力-应变环评价肥厚型心肌病

目的 观察超声左心室压力-应变环（PSL）评价肥厚型心肌病（HCM）患者左心室收缩功能的价值,评价心肌做功（MW）参数与常规舒张收缩功能、左心室应变参数间的相关性。方法 纳入43例HCM患者（HCM组）与49名健康志愿者（对照组）,比较组间常规超声参数、应变参数和MW参数。绘制受试者工作特征（ROC）曲线,计算曲线下面积（AUC）,评价各参数诊断HCM的效能。采用Pearson或Spearman相关分析评估常规超声参数、应变参数与MW参数间的相关性,以多元线性回归分析与MW独立相关的参数。结果 HCM组室间隔舒张末期厚度（IVSD）、左心室后壁舒张末期厚度（LVPWD）、左心房前后径（LAD）、容积指数（LAVI）、A峰流速、E/A、E/e''、左心室心内膜下心肌圆周应变（CS-Endo）、左心室整体扭转应变（Twist）、峰值应变离散度（PSD）及整体无效功（GWW）均明显高于对照组（P均<0.05）,舒张早期二尖瓣环侧壁（Lateral e''）、室间隔运动速度（Septal e''）、e''、左心室舒张末期容积（LVEDV）、收缩末期容积（LVESV）、左心室中层心肌（CS-Mid）、心外膜下心肌圆周应变（CS-Epi）、左心室整体纵向及分层应变、整体做功指数（GWI）、整体做功效率（GWE）均明显低于对照组（P均<0.05）。MW指标、左心室整体纵向应变（LVGLS）及PSD诊断HCM的效能均较高（AUC均>0.80）。对照组MW参数与传统收缩舒张功能参数、LVGLS及PSD多呈中度相关,且具有独立相关性（P均<0.05）;HCM组不同方向整体和分层应变、PSD及A峰流速均多与MW参数呈低度及以上相关,且具有独立相关性（P均<0.05）。结论 超声PSL技术可无创评估HCM患者左心室收缩功能;MW参数与常规舒张收缩功能、左心室应变参数具有一定相关性。     

心电图和心脏超声心动图对心尖肥厚型心肌病诊断价值的相关性分析

目的：回顾性分析心尖肥厚型心肌病（AHCM）患者心电图和超声心动图特点,了解两者的关联性,探讨两者在AHCM中的诊断价值。方法：对62例AHCM患者（AHCM组）与27名无器质性心脏病的健康体检者（健康对照组）的超声心动图和心电图资料进行回顾性分析,并对AHCM组患者的心电图和超声心动图进行相关性分析。结果：AHCM组患者心电图主要表现为胸前导联巨大倒置的T波伴ST段下移,左室高电压。超声心动图有不同程度的心尖部肥厚,心尖部心腔明显变小。心电图和超声心动图指标具有相关性：R波最大波幅与心尖心肌厚度、左室后壁厚度呈正相关,ST段压低深度与心尖部室壁厚度无相关性,与心尖心肌厚度呈负相关,T波倒置深度与心尖部厚度呈负相关。结论：心电图及超声心动图对诊断AHCM具有重要价值,心电图和超声心动图中的许多异常指标具有相关性,两者结合可提高本病诊断率。     

Coexistent atrioventricular and nodoventricular pathways in a patient with hypertrophic cardiomyopathy

A 17-year-old girl with concentric hypertrophic cardiomyopathy presented with a wide complex tachycardia and underwent electrophysiological study. She was found to have an antidromic tachycardia utilizing a decremental atrioventricular fiber as the anterograde limb with retrograde conduction occurring through the septum. Ablation of a right free-wall pathway rendered tachycardia noninducible, yet ventricular preexcitation remained. After ablation there was evidence of a second nodoventricular connection. We believe this to be the first report of coexistent "Mahaim" fibers; one a decremental atrioventricular connection and the second nodoventricular.     

肥厚型心肌病QT离散度的临床意义及卡维地洛对其作用

目的探讨肥厚型心肌病QT离散度的临床意义及卡维地洛对其的作用。方法入选48例患者,根据超声结果将患者分梗阻性20例（梗阻组）争非梗阻28例（非梗阻组）,正常对照组20例。为每个患者记录体表常规12导联同步心电图,分别计算QT、QT离散度（QTd）、校正的QT离散度（QTcd）。在治疗过程中给予卡维地洛,在给药前、1个月和3个月后复查以上指标。结果梗阻性肥厚型心肌病和非梗阻性肥厚型心肌病QT、QTd、QTcd均超过正常值（P〈0.01）;卡维地洛能明显缩短两组的QTd,且梗阻组QTd的变化程度较非梗阻组更显著,用药前（84.2±27.1）ms vs（46.5±20.7）ms,用药3个月后（49.8±19.2）ms vs（41.3±16.3）ms（P〈0.05或〈0.01）。结论两组肥厚型心肌病QT、QTd、QTcd均超过正常值,卡维地洛能明显缩短两组的QTd,且梗阻组QTd的变化程度较非梗阻组更显著,有利于改善患者预后。     

Inappropriate therapy from a defibrillator complicating transcoronary ablation of septal hypertrophy in a patient with hypertrophic obstructive cardiomyopathy

This case report describes a patient with obstructive hypertrophic cardiomyopathy who received therapy inappropriately from his implanted defibrillator, subsequent to transcoronary alcohol ablation for septal hypertrophy (TASH). Widening of the intracardiac electrogram postablation resulted in "double counting" of the intrinsic ventricular electrogram by the device and inappropriate tachycardia detection.     

Genetic basis of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) was the first cardiovascular disorder in which a genetic basis was identified. The disease is characterized by a marked thickening of the left ventricle and is the most common structural cause of sudden cardiac death in those aged under 35 years. HCM is primarily a disease of the sarcomere with over 250 mutations identified currently within 13 sarcomere-related genes. At present, genetic screening is available for the genes shown to cause HCM most frequently, with a mutation pick-up rate of up to 60%. Current research is focused on the identification of additional causative genes and elucidation into signaling mechanisms involved in HCM pathogenesis, as well as investigation of modifying factors that can alter the clinical phenotype in HCM. The unifying goal of these studies is to improve our understanding of disease pathogenesis in HCM, thereby facilitating the process of new diagnostic and therapeutic approaches in patients, ultimately leading to disease prevention and possible curative treatment.     

Focal AF-ablation after pulmonary vein isolation in a patient with hypertrophic cardiomyopathy using cryothermal energy

A 42-year-old man, with a history of hypertrophic cardiomyopathy (HCM), an electrocardiogram pattern of ventricular preexcitation typical for mutations in the PRKAG2 gene, and highly symptomatic paroxysmal drug-resistant atrial fibrillation (AF), underwent successful circumferential isolation of his pulmonary veins using a 28-mm double lumen cryoballoon. Because AF was still inducible with programmed stimulation, fractionated signals were targeted in the left atrium with a conventional cryocatheter. Ablation of an endocardial focus with fractionated potentials at the base of the left appendage terminated the episode and rendered AF noninducible. No recurrence of AF was observed during a 10-month follow-up period.