肥厚性心肌病的心室造影特点

目的分析梗阻型肥厚性心肌病(HOCM)患者心室造影结果及形态学特征。方法纳入1995~2005年收治入院并接受心室造影和超声检查的74例确诊为肥厚型心肌病的患者。以左心室流出道与左心室压力差≥30 mmHg为梗阻型,30 mmHg为非梗阻型,将患者分为2组。比较其形态学和造影结果。结果 (1)入选74例患者,梗阻型14例(18.9%),其中收缩期前向运动6例(42.9%),冠心病3例(21.4%);非梗阻型60例(81.1%),其中11例合并冠心病(18.3%)。(2)超声显示室间隔增厚至(11.4±2.5)mm,左室后壁厚度(9.8±1.7)mm,左心室舒张末内径(48±5)mm,左心房内径(36±5)mm。(3)左室造影显示左室舒张末容积为(123±31)ml,左室收缩末容积为(27±11)ml,左心室射血分数(EF)为(78±6)%。(4)左心室造影形态学特征为梗阻型心室结构14例,其中正常5例;非梗阻型左心室结构60例,正常27例。结论非梗阻型肥厚性心肌病与HOCM左室舒张末内径、左室舒张末容积及左室收缩末容积、EF值,以及呈现正常形态的病例数等均无差异。     

Hypertrophic cardiomyopathy: a case of symptomatic Japanese type apical hypertrophic cardiomyopathy

A 61-year-old male patient was hospitalized due to the exertional angina pectoris. A diagnosis of apical hypertrophic cardiomyopathy was made by ECG (electrocardiography), echocardiographic, and coronary angiographic findings. This case was reported and related literature was reviewed because of its similarity to Japanese type apical hypertrophic cardiomyopathy (AHCMP) cases rarely seen outside Asia.     

Contrast-enhanced echocardiography for the diagnosis of apical hypertrophic cardiomyopathy

The echocardiographic diagnosis of apical hypertrophic cardiomyopathy can be difficult in patients with poor acoustic windows. However, contrast-enhanced echocardiography can provide better images in these patients and lead to the correct diagnosis. We present a patient with apical hypertrophic cardiomyopathy who was diagnosed using contrast-enhanced echocardiography. The use of contrast-enhanced echocardiography for the diagnosis of apical hypertrophic cardiomyopathy in patients with poor acoustic windows is discussed and the experience in the literature reviewed.     

Clinical and echocardiographic aspects of mid-ventricular hypertrophic cardiomyopathy

Three cases of patients with hypertropic cardiomyopathy, apical aneurysm, and mid-ventricular obstruction are presented. Two patients were diagnosed first by two-dimensional and Doppler echocardiography, which showed mid-ventricular obliteration, characteristic hourglass image, and paradoxic jet flow. One patient with suboptimal echocardiogram was necessary to perform contrast echocardiogram. Clinical picture was characterized by angina and dyspnea. Thallium myocardial imaging revealed perfusion abnormalities in apical region, ischemia or necrosis. Cardiac catheterism showed mid-ventricular obliteration and significant intraventricular gradient and coronary arteries angiography without lesions.     

肥厚型心肌病伴心房颤动患者临床特点分析

目的分析32例肥厚型心肌病伴心房颤动患者的临床特点。方法选择1994-2005年在解放军总医院心脏中心就诊的肥厚型心肌病患者158例，平均随访（4．2±2．8）年，按病史、心电图、动态心电图是否记录到心房颤动分为房颤组和非房颤组，观察并比较2组患者的临床特点。结果（1）肥厚型心肌病伴房颤患者共32例，占全部肥厚型心肌病患者的20．3％，其中阵发性房颤14例（43．7％），持续性房颤18例（57．3％），无症状性房颤5例（15．6％）；（2）与非房颤组患者比较，房颤组患者平均年龄偏大（58±10．4vs46±12．6）岁（P〈0．01），左房直径大（42±3．5vs34±5．3）mm（P〈0．01）；（3）房颤组12例（心力衰竭8例，猝死4例）发生心血管事件，非房颤组9例（心力衰竭6例，猝死3例）发生心血管事件。房颤组患者中，房颤引发室颤1例（3％），脑栓塞2例（6．3％），下肢动脉栓塞2例（6．3％）。结论（1）20．3％肥厚型心肌病患者伴心房颤动，其中43．7％为阵发性房颤，57．3％为持续性房颤，15．6％为无症状性房颤；（2）与肥厚型心肌病不伴房颤患者相比，伴房颤患者年龄偏大，心血管事件发生率高。     

心尖肥厚型心肌病患者心电图的诊断意义

目的探讨心尖肥厚型心肌病患者的心电图特征性改变及临床诊断意义。方法分析38例心尖肥厚型心肌病患者的心电图及超声心动图资料。结果心尖肥厚型心肌病合并有心电图异常和超声心动图异常改变者38例(100%),其中伴胸前导联巨大倒置T波≥0.2mV～0.3mV,以V3～V4最明显29例(76%);左胸V4～V6导联R波电压增高,且RV4>RV5>RV6者26例(68.4%);ST段压低,以V3～V4最明显者29例(76%);超声心动图示心尖部肥厚达15mm或以上伴心尖部心腔狭小者38例。结论心电图对心尖肥厚型心肌病具有早期诊断价值和预测意义。     

Coronary artery-left ventricular fistula with apical hypertrophic cardiomyopathy

We describe two patients with coronary artery-left ventricularfistulae in association with apical hypertrophic cardiomyopathywho presented with chest pain.     

起搏治疗肥厚梗阻型心肌病的长期疗效观察

摘要 目的 评价起搏治疗对肥厚梗阻型心肌病(hypertrophic obstructive cardiomyopathy，HOCM)左室流出道梗阻及临床症状的长期疗效。方法 25例HOMC患者接受起搏治疗，临床随访12个月，观察临床症状体征；采用超声心动图观察起搏前后室间隔厚度(IVS)、左室后壁(LVPW)、左室舒张末内径（LVDd）、左室收缩末内径（LVDs）、左房内径（LAD）、跨左室流出道压力阶差（LVOT）、及二尖瓣前向运动程度（SAM）、二尖瓣反流的变化。结果 25例患者中植入双腔生理性起搏器21例，占84%，单腔SSIR起搏4例，占16%。起搏治疗12个月后，临床症状明显改善，15例晕厥发作术后症状完全消失，劳力性呼吸困难、胸痛改善分别占82.6%和75.0%， LVOT显著下降，从术前89.7±23.8 mmHg 降至术后36.8±12.6 mmHg ，P<0.01，IVS、LAD、MVA减少，SAM运动明显改善，其他指标无显著变化。结论起搏治疗对HOCM长期临床效果满意。     

56例肥厚型心肌病患者的临床分析

目的探讨肥厚型心肌病患者出现晕厥等严重临床症状的预测因素。方法回顾性分析56例肥厚型心肌病患者临床特征,按照有无晕厥的发生进行对照性研究。结果晕厥组中12例QT间期较非晕厥组延长更明显(452±20msvs429±25ms,p<0.01);且晕厥组中病例都出现心尖区3/6级以上收缩期杂音,而非晕厥组中心尖区杂音只有9%(4/44);另外,左室流出道梗阻在晕厥组中亦明显增多(p<0.05)。结论本研究发现对肥厚型心肌病患者发生晕厥等严重临床症状的危险性预测因素有:①QT间期延长;②心尖区3/6级以上收缩期杂音;③左室流出道梗阻。     

Early outcomes of alcohol septal ablation for hypertrophic obstructive cardiomyopathy

Background: This study was designed to evaluate the outcomes of alcohol septal ablation (ASA) under multicenter and multinational conditions. Methods: Data for 459 patients (age 57 ± 13 years) from nine European centers were prospectively collected and retrospectively analyzed. Results: ASA led to a significant reduction in outflow gradient (PG) and dyspnea [median of PG from 88 (58–123) mm Hg to 21 (11–41) mm Hg; median of NYHA class from 3 (2–3) to 1 (1–2); P < 0.01]. The incidence of 3‐month major adverse events (death, electrical cardioversion for tachyarrhythmias, resuscitation) and mortality was 2.8% and 0.7%, respectively. Permanent pacemakers for post‐ASA complete heart block were implanted in 43 patients (9%). Multivariate analysis identified higher amount of alcohol (however, in generally low‐dose procedures), higher baseline left ventricular ejection fraction and higher age as independent predictors of PG decrease ≥50%. Conclusions: The results of the first European multicenter and multinational study demonstrate that real‐world early outcomes of ASA patients are better than was reported in observations from the first decade after ASA introduction. © 2013 Wiley Periodicals, Inc.     

Long-term evaluation of patients with apical hypertrophic cardiomyopathy: Correlation between quantitative echocardiographic assessment of apical hypertrophy and clinical-electrocardiographic findings

Apical hypertrophic cardiomyopathy (AHCM) is characterized byprimary hypertrophy localized exclusively in the apex of theleft ventricle. Previous studies have indicated that AHCM resultsin a unique combination of cross-sectional echocardiographic(CSE) and ECG findings (‘giant’ Twave inversionand high R wave voltage in the precordial leads). The aims ofthis study were: (1) to assess the degree of AHCM in a quantitativefashion (2) to evaluate the possible relationship between apicalhypertrophy, quantitatively determined, and ECG findings inpatients with AHCM (3) to verify the changes in echocardiographicand ECG parameters over time (4) to define the relationshipbetween the severity of AHCM and the clinical course of suchpatients. Eleven selected patients with AHCM were studied for an average6 year follow-up period; there were seven men and four women(age from 18 to 62 years, mean 49). Apical hypertrophy was assessedquantitatively by determining the muscle cross-sectional areain the apical region, which was considered an index of myocardialmass. From the end-diastolic apical four chamber view, endocardialand epicardial contours were digitized in order to obtain thetotal muscle cross-sectional area of the left ventricle. Thewalls of the left ventricle were then divided into three regions(basal, intermediate, apical). The final value of each cross-sectionalmuscle area was obtained from the mean measurements of fourindependent and blinded observers. In AHCM the apical musclecross-sectional area (AMA) ranged from 10.3 to 17.9 cm², mean13.2 ±2.6 cm². The comparison between CSE and ECG findingsshowed that patients with giant negative T wave inversions (Twave >10 mm) and high R wave voltages (R wave >25 mm)had a more severe degree of apical hypertrophy. However, therewas incomplete agreement between CSE and ECG findings. During follow-up, negative T wave amplitude increased from 8.5±3.4 to 11.9 ±3.6 mm (mean 4.2 ±2.7) in10 patients (P>0.01) and there was a mild increase of precordialR wave (from 28.0 ±5.9 to 29.3 ± 5.2 mm, mean1.5 ± 1.6) (P–ns). The AMA change over time, from13.2 ± 26 to 13.8 ± 2.3 was not significant. Allpatients were alive at the most recent evaluation, and witlioutsignificant symptomatic deterioration. This study demonstrates a wide spectrum in the degree of severityof apical hypertrophy among patients with AHCM. Furthermore,ECG findings are not uniform and are not significantly relatedto the severity of the hypertrophy itself Therefore, AHCM shouldbe considered as a part of the morphological spectrum of hypertrophiccardiomyopathy rather than a separate entity with univocal CSEand ECG findings. Follow-up data indicate that despite ECG results worsening overtime, a significant progression in apical left ventricular wallthickness does not occur. Changes in negative T wave amplitudeare not related to symptoms and are not predictive of the functionalseverity of AHCM. Finally, the clinical outcome of patientswith AHCM seems not be dependent on the entity of apical hypertrophy.     

Magnetic resonance imaging for assessment of apical hypertrophy in hypertrophic cardiomyopathy

The purpose of the study was to evaluate the value of magnetic resonance imaging as compared with two-dimensional echocardiography for a reliable assessment of the degree and distribution of apical hypertrophy in hypertrophic cardiomyopathy (HCM). The study includes 10 HCM patients (8 males and 2 females, mean age: 42±7 years). Two-dimensional echocardiography was not definitive in assessing the abnormal thickening of the apical myocardium in two patients. Two other patients had inadequate echocardiographic visualization of the lower left ventricle due to technical reasons. At magnetic resonance imaging, 3 patients showed localized hypertrophy at the left ventricular apex only. Three other patients had evidence of hypertrophy at the apex as well as at the left ventricular free wall. In four patients, the hypertrophy was detected at either the apex or the lower interventricular septum. It is concluded that magnetic resonance imaging might provide an accurate assessment of myocardial hypertrophy in HCM patients. This technique appears to be of major value in those with wall thickening localized to (or predominant in) the apical portion of the ventricle.     

Clinical outcomes of radiofrequency catheter ablation of ventricular tachycardia in patients with hypertrophic cardiomyopathy

Background

Monomorphic ventricular tachycardia (VT) is rare in patients with hypertrophic cardiomyopathy (HCM), management of which is challenging. Limited data exists on the utility of catheter ablation for the treatment of VT in this population.

Objectives

We aimed to assess clinical outcomes of catheter ablation for VT in HCM patients.

Methods

A systematic search, without language restriction, using PubMed, EMBASE, SCOPUS, Google Scholar, and ClinicalTrials.gov was performed. The meta-analysis was performed using a meta-package for R version 4.0/RStudio version 1.2 and Freeman Tukey double arcsine method to establish the variance of raw proportions. Outcomes measured included (1) acute procedure success (defined as noninducible for clinical VT), (2) freedom from VT at follow-up, (3) mortality.

Results

This systematic review of six studies (three from the United States and three from Japan) incorporated a total of 68 drug-refractory HCM patients who underwent VT radiofrequency catheter ablation (mean age 57.6 ± 13.3 years, mean LVEF 45.8 ± 15.4%, 85% men, maximum septal wall thickness 17.4 ± 4.6 mm, and 32.3% with an apical aneurysm). Acute procedural success was achieved in 84.5% patients (95% confidence interval [CI]: 70.6%–95.2%) with 27.9% patients had recurrent VT requiring multiple ablations (median 1, IQR 1–3). During the follow-up period (18.3 ± 11.7 months), the pooled incidence of freedom from recurrent VT after index procedure was 70.2% (95% CI: 51.9%–86.2%), while after the last ablation was 82.8% (95% CI: 57%–99.2%). There were two deaths during follow-up, one from heart failure and one from SCD 0.8% (95% CI: 0%–5.8%).

Conclusion

The results of our pooled analysis demonstrated that catheter ablation for VT in HCM patients was associated with high acute procedural success, and reduced VT recurrence—findings comparable to previously published reports in other disease substrates.     

Live three-dimensional transthoracic contrast echocardiographic assessment of apical hypertrophic cardiomyopathy

We describe an adult patient in whom live three-dimensional transthoracic echocardiography combined with intravenous use of an echo contrast agent was useful in making a definitive diagnosis of apical hypertrophic cardiomyopathy and in characterizing the nature and full extent of the hypertrophy.     

Apical hypertrophic cardiomyopathy

We describe a patient with asymptomatic apical hypertrophic cardiomyopathy(AHCM)who later developed cardiac arrhythmias,and briefly discuss the diagnostic modalities,differential diagnosis and treatment option for this condition.AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle.AHCM can be an incidental finding,or patients may present with chest pain,palpitations,dyspnea,syncope,atrial fibrillation,myocardial infarction,embolic events,ventricular fibrillation and congestive heart failure.AHCM is frequently sporadic,but autosomal dominant inheritance has been reported in few families.The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular(LV)hypertrophy.A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of ACHM and shows hypertrophy of the LV apex.AHCM may mimic other conditions such as LV apical cardiac tumors,LV apical thrombus,isolated ventricular non-compaction,endomyocardial fibrosis and coronary artery disease.Other modalities,including left ventriculography,multislice spiral computed tomography,and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions.Medications used to treat symptomatic patients with AHCM include verapamil,beta-blockers and antiarrhythmic agents such as amiodarone and procainamide.An implantable cardioverter defibrillator is recommended for high risk patients.     

合并高血压的肥厚型梗阻性心肌病患者的临床特点分析

目的：探讨合并高血压的肥厚型梗阻性心肌病（hypertrophic obstructive cardiomyopathy,HOCM）患者的临床表现、超声心动图及预后特点. 方法：入选HOCM患者80例,根据是否合并高血压将患者分为2组：合并高血压的HOCM恶者为H-HOCM组（n=43）,无高血压的HOCM患者为N-HOCM组（n=37）.随访并对比分析两组患者的临床资料. 结果：与N-HOCM组患者相比,H-HOCM组患者年龄较大,合并糖尿病者较多,左室舒张末期内径和收缩末期内径增大,室间隔厚度降低（P＜0.05）.药物治疗方面,两组患者β受体阻滞剂应用率均较高（＞80％）,H-HOCM组患者血管紧张素受体阻断剂（ARB）和血管紧张素转换酶抑制剂（ACEI）应用率明显高于N-HOCM组（P＜0.05）.随访期间两组患者临床相关事件发生率无统计学差异. 结论：合并高血压的HOCM患者呈高龄趋势,多合并糖尿病,左室呈离心性肥厚,其预后特征有待深入观察.     

肥厚型梗阻性心肌病合并二尖瓣自身病变致二尖瓣关闭不全的外科治疗

目的 评价外科治疗肥厚型梗阻性心肌病合并二尖瓣自身病变致二尖瓣关闭不全（MR）的临床效果.方法 2000年1月至2009年12月我科收治13例肥厚型梗阻性心肌病合并二尖瓣自身病变致二尖瓣关闭不全患者.所有患者均行室间隔心肌切除术（改良marrow术）,7例行二尖瓣置换术（MVR）,6例行二尖瓣成形术（MVP）.3例患者再次转机,1例因左室流出道狭窄解除不满意,1例有残余MR行MVP,1例残余MR行MVR.结果 全组体外循环时间（131.9±61.8）min,主动脉阻断时间（92.7±39.8）min.超声心动图（UCG）显示术后左房大小、左室流出道压差均较术前有显著下降.MVP组有2例因再次MR行MVR.结论 此类患者理想的手术方法是充分切除肥厚的室间隔肌肉,自身有病变的二尖瓣可考虑行MVR或MVP,如果条件许可,应首选MVP.