Serial electrocardiographic and angiographic changes of patients with hypertrophic cardiomyopathy

The characteristics of ECG findings as they relate to myocardial changes during long-term course of hypertrophic cardiomyopathy (HCM) were studied. Serial changes in ECG were compared with changes left ventriculogram and bi-ventriculogram in 15 patients with HCM without intraventricular conduction disturbance. Serial changes in ECG findings, with special reference to the negative T wave, were categorized in three groups; 1) 5 patients with increase or appearance of the negative T wave (A-1 group). 2) 4 patients with decrease or disappearance of the negative T wave (A-2 group). 3) 6 patients without significant changes in the T wave (B-group). A-1 group presented a marked increase in SV1 + RV5, and of the thickness of anterior and apical wall, and a decrease of the peak dV/dt/EDV (end diastolic volume-normalized peak filling rate), serially. End diastolic volume (EDV) and ejection fraction (EF) did not change significantly. The configuration of left ventriculogram changed from a round form to a spade-like form. A-2 group presented a marked decrease in SV1 + RV5, the thickness of anterior and apical wall, peak dV/dt/EDV and peak dV/dt/V (left ventricular volume of the time of peak filling-normalized peak filling rote). EF decreased serially, 2 cases of A-2 group presented the clinical picture of dilated cardiomyopathy in the end stage. In B group, SV1 + RV5, the wall thickness and left ventricular function did not change, serially. In conclusion, serial observations of ECG are useful for assessing alterations in wall thickness, LV-form and LV-function.     

Electrocardiographic features differentiating dilated cardiomyopathy from hypertrophic cardiomyopathy

To determine the usefulness of electrocardiographic (ECG) features in differentiating between hypertrophic cardiomyopathy with features mimicking dilated cardiomyopathy (D-HCM) and true dilated cardiomyopathy (DCM), we compared ECGs of 52 consecutive patients (11 with D-HCM, 41 with DCM). Left atrial dimension, left ventricular internal dimension, and septal and posterior wall thickness were employed as echocardiographic indexes, while QRS duration, amplitude of RV₅ or V₆ + SV₁, number of abnormal Q waves, P-terminal force in V₁, and frontal plane QRS axis were used as ECG parameters. The patients with D-HCM demonstrated a larger number of abnormal Q waves (P < .0001), greater prolongation of QRS duration (P < .0001), and lower amplitude of RV₅ or V₆ + SV₁ (P < .0001). In all cases of D-HCM, atrial overload was observed and abnormal QRS axis in 9 (82%) of the 11 patients. These features were noted in 21 (51%) and 17 (41%), respectively, of the 41 DCM patients (P < .005 and P < .05, respectively). Despite significant differences in the echocardiographic parameters between D-HCM and DCM, excluding left ventricular end-diastolic dimension, ECG abnormalities were more significant between the two groups. The results indicate that ECG features are extremely useful in differentiation between DCM and D-HCM.     

199例肥厚型心肌病患者的长期随访研究

目的探讨肥厚型心肌病（HCM）患者的长期预后及其死亡的相关危险因素。方法回顾性分析1999年6月至2006年3月期间收入院的234例HCM患者的病史资料,并对其中获得随访的199例患者的随访结果采用SPSS13．0软件包进行统计学处理。结果234例患者中,199例（85％）获得随访,35例（15％）患者失访。平均随访时间为（31．7±22．6）个月,由确诊到随访结束时间中位数为35个月。随访中21例患者死亡,其中19例患者被确认为HCM相关性死亡,包括心脏骤停11例（57．9％）、心力衰竭死亡7例（36．8％）、脑卒中死亡1例,另有2例患者分别因意外事故和急性胰腺炎死亡。患者确诊后1、2、3、4、5年生存率分别为96．7％、94．7％、94．7％、93．6％、89．0％。单因素分析显示,男性、心功能Ⅲ及以上、心房颤动、持续或短阵室速、左心房增大、左心室流出道梗阻、HCM家族史7个变量与HCM预后相关。而多因素分析显示,仅持续或短阵室速（RR=2．234,P〈0．001）、心功能Ⅲ及以上（RR=1．964,P=0．003）是HCM的独立预后危险因素。心脑事件死亡患者中,超声心动图表现以MaronⅢ型为多见（73．7％）,仅1例患者表现为心尖肥厚型心肌病（5．2％）。对心脏骤停者和心力衰竭死亡者进行分析显示,7例（63．6％）心脏骤停者发生在60岁以下的患者中（P=0．12,但仅1例患者小于35岁）,5例（71．4％）心力衰竭死亡者发生在60岁以上的患者中（P=0．22）。仅2例心脏骤停者和1例心力衰竭死亡者存在静息状态下左心室流出道压差。7例（63．6％）心脏骤停者和2例（28．6％）心力衰竭死亡者合并阵发性室速。心脏骤停者与心力衰竭死亡者的室壁厚度分别为（20．4±4．7）mm和（22．7±6．3）mm。结论HCM患者的中长期预后相对较好,以心肌肥厚仅累及心尖部者预后最佳。持续或短阵室速、心功能Ⅲ级以上是发生HCM相关心脑血管不良事件的独立危险因素。心脏骤停可发生在各年龄段,对其的预防在年轻和中老年患者中同样重要。     

Electrocardiographic findings in patients with hypertrophic cardiomyopathy. Relation to presenting features and prognosis

The relation of ECG findings to presenting features and prognosis was evaluated in 125 consecutive patients with hypertrophic cardiomyopathy (HC). Seventy-nine men and 46 women (mean age, 34 +/- 7 years) were studied since 1970. Most ECG features were similar in patients with and without a left ventricular outflow tract gradient. Those with obstruction had a higher prevalence of left ventricular hypertrophy according to ECG voltage criteria (54% vs. 28%, p less than 0.01), whereas higher grade ventricular arrhythmias were more common in patients without an outflow gradient (20% vs. 7%, p less than 0.05). The prevalence of ECG abnormalities was also similar in younger (less than or equal to 14 years) and older patients (greater than 14 years), and only repolarization abnormalities were more frequently detected in the older age group (56% vs. 32%, p less than 0.025). Stratification of patients according to the clinical state revealed that those who had moderate to severe functional limitation had a higher prevalence of atrial fibrillation than asymptomatic or mildly symptomatic patients (24% vs. 1%, p less than 0.001). There were no significant differences in most hemodynamic variables among patients dichotomized according to any specific ECG abnormality. Only patients with atrial fibrillation had significantly higher right ventricular end-diastolic pressure (10 +/- 7 vs. 6 +/- 4 mmHg, p less than 0.01), lower systolic index (22 +/- 8 vs. 37 +/- 15 ml/m2; beat, p less than 0.02) and lower ejection fraction (53 +/- 8 vs. 64 +/- 10%, p less than 0.001) than those in sinus rhythm.(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term follow-up of patients with obstructive hypertrophic cardiomyopathy treated with dual-chamber pacing

In this study, patients with obstructive hypertrophic cardiomyopathy (HC) were treated with dual-chamber pacemaker therapy. Long-term follow-up analysis showed that dual-chamber pacemaker therapy in selected patients resulted in a significant reduction in symptoms and in the left ventricular outflow tract gradient, which was maintained up to 10 years after implantation. Dual-chamber pacing is of potential long-term benefit in selected groups of patients with obstructive HC.     

肥厚型梗阻性心肌病患者行左室流出道疏通术后的心电图特点

目的 探讨肥厚型梗阻性心肌病患者行左室流出道疏通术后心电图的相关变化及特点.方法 入选2018年1月1日至12月31日期间在上海交通大学医学院附属瑞金医院明确诊断为肥厚型梗阻性心肌病并接受左室流出道疏通术的患者共38例,收集临床资料及手术前后心电图,进行对比分析,总结其相关变化及特点.结果 ①术前术后患者心室率、QRS...     

梗阻性肥厚型心肌病室间隔心肌消融术后心电图变化

目的 :探讨梗阻性肥厚型心肌病经皮腔内室间隔消融术后心电图变化及临床意义。方法 :5例梗阻性肥厚型心肌病患者完成经皮腔内室间隔消融术 ,观察并比较术后不同时间记录的心电图和动态心电图。结果 :5例患者术中和术后均发生右束支传导阻滞 ;术中 3例出现一过性Ⅲ度房室传导阻滞 ,其中 1例发生心室颤动 ,1例出现频发室性期前收缩 ,1例出现Ⅰ度房室传导阻滞 ;术后未见新发Q波 ,QRS时限、QT和QTc间期明显延长 ,Sv1+Rv5显著降低 ,动态心电图随访未见室性心律失常增加。结论 :经皮腔内室间隔消融术致心律失常的发生率较高 ,但常为一过性。 12导联心电图和动态心电图监测是最为实用的诊断手段     

左心室中部肥厚型梗阻性心肌病临床及造影特征分析

目的 分析左心室中部肥厚型梗阻性心肌病患者的临床及心血管造影特征.方法 5例(男:女=3:2)临床诊断为左心室中部肥厚型梗阻性心肌病的患者,年龄16～73(44±22)岁.所有患者均接受左心导管及心血管造影检查,并记录左心室心尖部-左心室基底部-左心室流出道-升主动脉连续压力.结果 5例患者中胸闷气短者4例,其中2例合并晕厥.所有患者心前区均可闻及收缩期杂音,且均存在左心室壁肥厚,室间隔厚度为19～31(23.8±5.4)mm,左心室舒张末期横径为35～55(43.4±7.4)mm,左心室射血分数为53%～70%、平均为(64.2±6.9)%.5例患者均存在左心室高电压伴异常Q波,其中1例患者合并阵发性室性心动过速,另有1例患者合并完全性左束支传导阻滞.左心导管检查证实收缩期左心室中部均存在梗阻,其中1例患者同时合并左心室流出道梗阻.左心室中部收缩期压力阶差为45～102(68.6±24.1)mm Hg(1 mm Hg=0.133 kPa).心血管造影检查提示前降支中段肌桥1例,冠状动脉粥样硬化性心脏病1例.另外,2例患者合并左心室心尖部室壁瘤.结论 左心室中部肥厚型梗阻性心肌病的临床特征不同于其他类型的肥厚型心肌病,完善的左心导管检查及造影有利于诊断并指导临床治疗.     

Electrocardiographic features of sarcomere mutation carriers with and without clinically overt hypertrophic cardiomyopathy

In hypertrophic cardiomyopathy (HC), electrocardiographic (ECG) changes have been postulated to be an early marker of disease, detectable in sarcomere mutation carriers when left ventricular (LV) wall thickness is still normal. However, the ECG features of mutation carriers have not been fully characterized. Therefore, we systematically analyzed ECGs in a genotyped HC population to characterize ECG findings in mutation carriers (G+) with and without echocardiographic LV hypertrophy (LVH), and to evaluate the accuracy of ECG findings to differentiate at-risk mutation carriers from genetically unaffected relatives during family screening. The ECG and echocardiographic findings were analyzed from 213 genotyped subjects (76 G+/LVH-, 57 G+/LVH+ overt HC, 80 genetically unaffected controls). Cardiac magnetic resonance imaging was available on a subset. Q waves and repolarization abnormalities (QST) were highly specific (98% specificity) markers for LVH- mutation carriers, present in 25% of G+/LVH- subjects, and 3% of controls (p <0.001). QST ECG abnormalities remained independently predictive of carrying a sarcomere mutation after adjusting for age and impaired relaxation, another distinguishing feature of G+/LVH- subjects (odds ratio 8.4, p = 0.007). Myocardial scar or perfusion abnormalities were not detected on cardiac magnetic resonance imaging in G+/LVH- subjects, irrespective of the ECG features. In overt HC, 75% had Q waves and/or repolarization changes, but <25% demonstrated common isolated voltage criteria for LVH. In conclusion, Q waves and repolarization abnormalities are the most discriminating ECG features of sarcomere mutation carriers with and without LVH. However, owing to the limited sensitivity of ECG and echocardiographic screening, genetic testing is required to definitively identify at-risk family members.     

Noninvasive findings in patients with hypertrophic and dilative cardiomyopathy: a combined echocardiographic and radionuclide angiographic study

Fifteen patients with hypertrophic (HCM) and 15 with dilative cardiomyopathy (DCM) were examined with radionuclide angiography and M-mode echocardiography to evaluate the combination of two noninvasive methods for measuring left ventricular performance. The patients with HCM had delayed myocardial relaxation and rapid filling time with preserved peak rate of early ventricular filling. DCM patients, on the contrary, had low values of left ventricular systolic performance and low peak filling rate. Myocardial relaxation and rapid filling time, however, were short, indicating compensatory mechanisms in the failing ventricle improving rapid filling.     

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肥厚型心肌病(HCM)是一种相对常见的遗传性疾病,部分出现流出道梗阻,需要药物、介入治疗或外科治疗。心外科治疗是梗阻性HCM治疗的金标准,有近50年的历史。目前"改良扩大MORROW术式"治疗效果明显,绝大多数患者5年内症状改善,左室舒张和收缩功能恢复,晕厥次数减少。经皮经腔室间隔心肌化学消融术(PTSMA)1995年英国医生Sigwart首次在Lancet上报道了应用96%酒精阻塞间隔支成功治疗肥厚型心肌病。机制是使用化学的方法消除肥厚的室间隔,使左室流出道增宽,心脏后负荷减轻,心排出量增加。PTSMA需注意掌握好适应证、做好激发试验、术中注意靶血管的选择和心肌声学造影(MCE)以及无水酒精的使用。     

Clinical and echocardiographic follow-up of patients with hypertrophic obstructive cardiomyopathy treated by percutaneous septal ablation

INTRODUCTION AND OBJECTIVES: Alcohol septal ablation is a therapeutic option for patients with hypertrophic obstructive cardiomyopathy who remain symptomatic despite medical treatment. Our aim was to monitor clinical and echocardiographic progression in patients with hypertrophic obstructive cardiomyopathy treated by septal ablation at our center. METHODS: Thirty-five septal ablations were performed in 34 patients (79% male) who had symptomatic hypertrophic obstructive cardiomyopathy despite optimum medical treatment. The procedure was successful in 32 (i.e., the reduction in left ventricular outflow tract pressure gradient, or LVOTPG, was >50%). During clinical and echocardiographic follow-up, New York Heart Association (NYHA) functional class and LVOTPG were monitored. RESULTS: The patients' mean age was 63 (12) years. The mean follow-up period was 9 (3) months. Immediately after septal ablation, LVOTPG decreased significantly, from 74.2 (25.3) mm Hg to 26 (25) mm Hg (P<.001), and remained low throughout follow-up. Moreover, echocardiography showed that the interventricular septum thickness also decreased during follow-up, from 19 (3) mm to 15 (2) mm (P<.0001). A significant improvement in NYHA functional class (from 93% in class III-IV to 84% in class I-II) was also observed. Two deaths occurred within 48 hours after the procedure. The most frequent complication was complete heart block (20%; n=6). CONCLUSIONS: Alcohol septal ablation is effective in patients with hypertrophic obstructive cardiomyopathy who remain symptomatic despite medical treatment. However, the procedure is associated with a significant rate of complications and should, therefore, be reserved for selected patients, in particular for elderly patients and those with comorbid conditions.     

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 通过与典型的肥厚型心肌病(HCM)比较,探讨中国汉族人心尖肥厚型心肌病(AHCM)的临床特征及其诊治方法.方法 回顾性收集160例HCM住院患者的临床资料,分成3组进行比较.AHCM组:AHCM 41例.典型HCM患者(室间隔以及左心室壁肥厚),根据是否存在流出道梗阻分成2组,即非梗阻性肥厚型心肌病(NOHCM)组,52例,左心室流出道压差＜30 mm Hg(1 mm Hg=0.133 kPa);梗阻性肥厚型心肌病(OHCM)组,67例,左心室流出道压差≥30mm Hg.比较3组患者的临床症状、诊治方法以及血浆生物标记物水平.结果 (1)AHCM组患者的发病年龄较OHCM组晚[(49.9±13.6)岁比(41.4±14.6)岁,P＜0.01],无猝死家族史,较少出现劳力性呼吸困难,血浆血N末端B型利钠肽原(NT-pro BNP)水平较OHCM组低(P＜0.01).血浆心肌酶中肌酸激酶同工酶(CK-MB)、乳酸脱氢酶(LDH)、肌钙蛋白I(TnI)和肌红蛋白(MYO)的水平在3组间差异均无统计学意义.(2)31例AHCM患者因冠心病收治入院,经检查后,18例(18/41,43.9%)排除了冠心病.(3)AHCM组、NOHCM组和OHCM组心电图上深尖倒置T波(GNT)出现的频率分别为43.9%、13.5%和4.4%(P＜0.01),且AHCM组多伴有左心室高电压.(4)心脏核磁共振诊断AHCM明显优于心脏超声,与诊断典型HCM相比更有优势.结论 AHCM与典型OHCM的临床特点比较差异有统计学意义,而与典型的NOHCM比较差异无统计学意义.心脏核磁共振检查阳性及心电图胸导联上典型的GNT可为确诊AHCM提供依据.

Abstract：

Objective To evaluate the clinical features in Chinese patients with apical hypertrophic cardiomyopathy (AHCM) and typical hypertrophic cardiomyopathy (HCM). Methods This retrospective analysis included 160 patients hospitalized in Fuwai hospital. Patients were divided into three groups: apical hypertrophic cardiomyopathy ( AHCM, n = 41 ) group, non-obstructive typical hypertrophic cardiomyopathy group[NOHCM, LVOT ＜30 mm Hg(1 mm Hg =0. 133 kPa) at rest, n =52] and obstructive typical hypertrophic cardiomyopathy (OHCM, LVOT ≥ 30 mm Hg at rest, n = 67). Clinical features, diagnosis,therapy, and plasma levels of biomarkers of these three groups were analyzed. Results ( 1 ) The age at disease onset was older in AHCM group than in OHCM group [(49. 9 + 13. 6) years vs. (41.4± 14. 6)years, P ＜ 0. 01]. Exertional dyspnea appered more often in HCM patients than in AHCM patients, NT-proBNP level was significantly lower in AHCM patients than in OHCM patients (P =0. 001 ). Plasma CK-MB, LDH, TnI and MYO levels were similar among the three groups. (2) Thirty-three AHCM patients were first hospitalized for suspected coronary heart disease (CHD) and CHD was excluded in 18 cases (43.9%).(3) The frequency of giant negative T waves (depth≥10 mm) on ECG was 43.9%, 13.5% and 4.4%(P ＜ 0. 01 ) in AHCM, NOHCM and OHCM respectively. Half of AHCM patients showed left ventricular high voltage on ECG. (4) Cardiac magnetic resonance imaging is superior to echocardiography on correctly diagnosing AHCM. Conclusion AHCM patients differ from typical OHCM patients in clinical characteristics. There were significant differences on echocardiography and electrocardiography features among three groups. Cardiac magnetic resonance imaging and giant negative T waves on ECG are helpful for the diagnosis of AHCM.     

Electrocardiographic changes associated with training and discontinuation of training in an athlete with hypertrophic cardiomyopathy

The 12-lead electrocardiogram of a 17-year-old African professional soccer player had signs of left ventricular (LV) hypertrophy and deeply inverted T waves in the inferior and precordial leads. Two-dimensional echocardiography showed mild LV hypertrophy with normal cavity size consistent with nonobstructive hypertrophic cardiomyopathy. After 5 months of complete discontinuation of training, the electrocardiogram normalized; however, the echocardiogram was unchanged. Subsequently, and contrary to our advice, the athlete resumed training and professional soccer. One year later the electrocardiogram again showed a similar and markedly abnormal pattern without cardiac dimensional changes.