Apical Left Ventricular Hypertrophy and Mid‐Ventricular Obstruction in Fabry Disease

We report the case of a rare cardiac presentation of Fabry disease. Although concentric left ventricular hypertrophy is a major cardiac finding in Fabry disease, there is no case report of dynamic obstruction at mid‐left ventricular level. We describe a 59‐year‐old‐woman suffering from a severe form of Fabry disease, mimicking an apical hypertrophic cardiomyopathy with mid‐ventricular obstruction. Differentiation of Fabry disease from hypertrophic cardiomyopathy is crucial given the therapeutic and prognostic differences. Fabry disease should always be suspected in an adult, independently of the pattern of left ventricular hypertrophy.     

Mid‐ventricular Hypertrophic Obstructive Cardiomyopathy with Apical Aneurysm Complicated with Syncope by Sustained Monomorphic Ventricular Tachycardia

Mid‐ventricular hypertrophic obstructive cardiomyopathy with secondary formation of apical aneurysm is a rare variant of hypertrophic cardiomyopathy. They have a unique behavior because unlike other variants it causes sustained monomorphic ventricular tachycardia, which makes it particularly severe.     

Apical hypertrophic cardiomyopathy presenting with sustained monomorphic ventricular tachycardia and electrocardiographic changes simulating coronary artery disease and left ventricular aneurysm

A 52-year-old male presented with sustained monomorphic ventricular tachycardia as the initial manifestation of apical hypertrophic cardiomyopathy. The electrocardiogram during normal sinus rhythm showed a pattern of an old anterior wall myocardial infarction with aneurysm formation. Cardiac catheterization documented angiographically normal coronary arteries. Apical hypertrophic cardiomyopathy was documented at cardiac catheterization and by echocardiogram and Doppler studies. Monomorphic ventricular tachycardia was reproducibly initiated and terminated during electrophysiological studies and antiarrhythmic drugs failed to control the tachycardia. At the time of implantation of a cardioverter defibrillator, left ventricular apical biopsy revealed pathologic findings characteristic of hypertrophic cardiomyopathy.     

Incremental Value of Live/Real Time Three‐Dimensional Transthoracic Echocardiography over Two‐Dimensional Echocardiography in Hypertrophic Cardiomyopathy with Mid‐Ventricular Obstruction and Apical Aneurysm

We describe a case of hypertrophic cardiomyopathy with mid‐left ventricular obstruction and apical aneurysm containing thrombi where live/real time three‐dimensional transthoracic echocardiography provided incremental value over two‐dimensional echocardiography in assessing the findings.     

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.     

The evolution of apical hypertrophic cardiomyopathy: Development of mid‐ventricular obstruction and apical aneurysm 11 years after initial diagnosis

Asian hypertrophic cardiomyopathy (AHCM) is a rare variant of hypertrophic cardiomyopathy (HCM) that is more prevalent in the Asian population. There is significant overlap between AHCM, mid‐cavitary obstruction, and apical aneurysms. Although more benign compared to HCM, the course of AHCM is not clearly defined. We present an interesting case of an African American male with known AHCM who develops symptomatic mid‐cavitary obstruction and apical aneurysm 11 years after initial diagnosis.     

Hypertrophic cardiomyopathy with mid-ventricular obstruction complicated with apical left ventricular aneurysm and ventricular tachycardia: a case report

A 69-year-old man was admitted with palpitations and syncope. His medical history included hypertension and left ventricular hypertrophy. Arterial pulsation was not palpable on admission. Electrocardiography revealed ventricular tachycardia, and cardioversion restored normal sinus rhythm. An electrophysiological study reproducibly induced polymorphic ventricular tachycardia, so a cardioverter defibrillator was implanted. Echocardiography revealed mid-ventricular obstruction and an apical aneurysm, and Doppler color flow imaging showed a diastolic paradoxic jet from the apex toward the base. Coronary angiography showed no stenosis of the extramural coronary arteries. Ventricular tachycardia on admission showed a right bundle branch block pattern and a superior axis deviation, so the arrhythmia was thought to originate from the apical aneurysm. Apical aneurysm can result from elevated intraventricular pressure or relative myocardial ischemia. This is a rare case of hypertrophic cardiomyopathy with mid-ventricular obstruction complicated with apical aneurysm and polymorphic ventricular tachycardia.     

肥厚型梗阻性心肌病合并左室心尖部室壁瘤同期外科手术治疗的远期结果

目的研究肥厚型梗阻性心肌病合并左室心尖部室壁瘤的外科治疗效果。方法回顾性分析2012年10月至2017年7月接受外科手术治疗的8例肥厚型梗阻性心肌病合并左室心尖部室壁瘤的病例资料。男6例,女2例;年龄14~73岁,平均(38.6±19.9)岁。所有患者均接受室间隔心肌切除术和室壁瘤切除术治疗。采集患者院内的病历资料,并通过门诊及电话随访记录患者情况。结果术前全部患者均通过冠状动脉造影排除了固定的冠状动脉狭窄病变,6例患者合并左室中部梗阻,3例有室性心动过速病史。全组病例无手术死亡,4例患者治疗过程中有不同程度的并发症。术后心脏超声提示LVOTPG、IVS、LAD、MI程度与术前比较均有显著性改善(P<0.05),但术后超声提示仍有4例患者心尖部可见室壁瘤样结构。所有患者随访(47.4±24.0)个月,随访率100%,术后NYHA心功能分级明显好于术前(P=0.001),远期死亡1例。结论肥厚型梗阻性心肌病合并左室心尖部室壁瘤的患者病情危重,同期行室间隔心肌切除术和室壁瘤切除术临床结果可以接受,但一些问题仍有待进一步研究。     

Transient left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve: A stunning cause

Left ventricular outflow tract obstruction (LVOTO) and systolic anterior motion (SAM) of the mitral valve may have various etiologies, of which hypertrophic cardiomyopathy is the most common. More rarely, an acute coronary syndrome, myocardial stunning, and takotsubo cardiomyopathy may give rise to LVOTO and SAM. Here, we present a 70‐year‐old female patient with a non‐ST‐elevation acute coronary syndrome treated with percutaneous coronary intervention. Echocardiography the day after, because of dyspnea and hypotension, revealed apical akinesia, LVOTO, and SAM, which proved completely reversible after treatment with a β‐blocker and a 2‐month follow‐up period. It was concluded that postischemic apical stunning had caused LVOTO and SAM.     

Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm

Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. In both patients the arrhythmia was associated with the uncommon finding of an apical aneurysm with angiographically normal coronary arteries.     

Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm.

Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. In both patients the arrhythmia was associated with the uncommon finding of an apical aneurysm with angiographically normal coronary arteries.     

Radiofrequency Catheter Ablation of Sustained Monomorphic Ventricular Tachycardia in Hypertrophic Cardiomyopathy

Monomorphic VT in HCM. Introduction : Incessant monomorphic ventricular tachycardia (VT) with a right bundle branch block morphology and a northwest axis is a rare arrhythmic complication in a patient with hypertrophic cardiomyopathy and apical left ventricular aneurysm.
Methods and Results : The origin of this VT was localized using the following criteria: the presence of entrainment without fusion, equal internals from the stimulus to the beginning of the QRS complex and from the electrogram to the QRS complex during VT, and the first postpacing interval identical to the tachycardia cycle length. Radiofrequency energy applied to the septoapical part of the apical left ventricular aneurysm terminated the tachycardia within 2 seconds.
Conclusion : Using criteria to guide radiofrequency (RF) ablation of VT in patients with coronary artery disease, an incessant monomorphic VT in a patient with hypertrophic cardiomyopathy was successfully ablated.     

Longitudinal left ventricular strain in hypertrophic cardiomyopathy: correlation with nonsustained ventricular tachycardia

Aims: Stratifying risk of sudden death is a major issue in the management of hypertrophic cardiomyopathy (HCM). Existing risk factors have low positive predictive value and new parameters are needed. Determination of myocardial deformation (strain) by 2D Speckle tracking is a new methodology for determining LV regional function and could correlate with myocite disarray and fibrosis. The aim of this study was to assess the relationship between strain analysis and nonsustained ventricular tachycardia (NSVT) in patients with HCM. Methods: Thirty‐two consecutive patients with HCM (mean age 55, 17–78) were studied. All underwent standard echocardiographic and two‐dimensional strain examination. Twenty‐four‐hour Holter monitoring was performed and echocardiographic parameters were correlated with NSVT. Results: Nine patients (28%) had one or more episodes of NSVT. Patients with NSVT had a higher value of maximal LV thickness (23.6 mm vs. 19.4 mm, P = 0.027). There were no significant associations between NSVT on Holter monitoring and LV outflow gradient left atrial diameter, E/Em or left ventricle ejection fraction. Patients with HCM and NSVT had significant reductions in mid septal, apical‐septal, apical‐lateral strain, and in mean longitudinal strain. Midseptal strain >–10.5% had a sensitivity of 89% and a specificity of 74% (area under the curve, 0.787; P < 0.0013) for predicting NSVT independently of age or maximum wall thickness. Conclusion: Lower end‐systolic peak longitudinal strain obtained by 2D speckle tracking was a predictor of NSVT in HCM patients. This parameter could become a useful tool in stratifying SCD risk in this population. (Echocardiography 2011;28:709‐714)     

Right ventricular obstruction in various types of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is most probably a genetically transmitted disease with different clinical and hemodynamic features. In hypertrophic obstructive cardiomyopathy (HOCM) the obstruction is predominantly in the left ventricular outflow tract (IHSS). In a minority of cases the obstruction is strictly located in midventricle (midventricular obstruction, MO). Hypertrophic nonobstructive cardiomyopathy (HNCM) includes asymmetric septal hypertrophy (ASH) and apical hypertrophy (AH). Right ventricular hypertrophic obstruction (RVHO) is an uncommon type of HCM and is almost always combined with other types of left ventricular HCM. We describe in the present report 1 case of RVHO with IHSS, 2 cases with MO and, to our knowledge, the first case with AH.     

Value of Electro‐Vectorcardiogram in Hypertrophic Cardiomyopathy

The electrocardiogram is an important tool for the initial diagnostic suspicion of hypertrophic cardiomyopathy in any of its forms, both in symptomatic and in asymptomatic patients because it is altered in more than 90 percent of the cases. Electrocardiographic anomalies are more common in patients carriers of manifest hypertrophic cardiomyopathy and the electrocardiogram alterations are earlier and more sensitive than the increase in left ventricular wall thickness detected by the echocardiogram. Nevertheless, despite being the leading cause of sudden death among young competitive athletes there is no consensus over the need to include the method in the pre‐participation screening. In apical hypertrophic cardiomyopathy the electrocardiographic hallmarks are the giant negative T waves in anterior precordial leads. In the vectorcardiogram, the QRS loop is located predominantly in the left anterior quadrant and T loop in the opposite right posterior quadrant, which justifies the deeply negative T waves recorded. The method allows estimating the left ventricular mass because it relates to the maximal spatial vector voltage of the left ventricle in the QRS loop. The recording on electrocardiogram or Holter monitoring of nonsustained monomorphic ventricular tachycardia in patients with syncope, recurrent syncope in young patient, hypotension induced by strain, bradyarrhythmia, or concealed conduction are markers of poor prognosis. The presence of rare sustained ventricular tachycardia is observed in mid‐septal obstructive HCM with apical aneurysm. The presence of complete right bundle branch block pattern is frequent after the percutaneous treatment and complete left bundle branch block is the rule after myectomy.     

Echocardiographic assessment of right ventricular obstruction in hypertrophic cardiomyopathy.

Echocardiography was used to evaluate the incidence, flow dynamics and morphological characteristics of right ventricular obstruction in 91 patients with hypertrophic cardiomyopathy. Color flow mapping was used to define the sites of obstruction in the left and right ventricles. Ventricular obstruction was considered to be present if the flow velocity was less than 2.0 m/s as measured by continuous wave Doppler. The thickness of both the right ventricular free wall and anterior ventricular septum was measured to assess the magnitude and extent of hypertrophy. Right ventricular obstruction was present in 14 patients of whom 6 (43%) had left ventricular obstruction also. The right ventricular obstructions were found in the outflow tract (9 patients), mid-base septal bulge (2 patients) and apical trabecular region (3 patients). Doppler waveform was confined to systole in all patients with obstruction in the outflow tract and in one of the patients with mid-base septal bulge. Moreover, the flow wave persisted into early diastole in 4 patients, including 2 with apical trabecular obstruction. The thickness of both the right ventricular free wall and anterior ventricular septum suggested that these hypertrophied regions were the sites of right ventricular obstruction. Thus, echocardiography was useful in evaluating right ventricular obstruction in hypertrophic cardiomyopathy.     

Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report

A 71-year-old woman had hypertrophic cardiomyopathy associated with midventricular obstruction and an apical aneurysm in the left ventricle. She had had abnormal electrocardiograms for more than 30 years and for the past year had been suffering from occasional attacks of dizziness and low systemic blood pressure. Holter 24-h electrocardiographic monitoring revealed ventricular paroxysmal contractions (676/day) with nonsustained ventricular tachycardia. Doppler echocardiography revealed paradoxical jet flow from the apical aneurysm to the left ventricular outflow during early diastole. Magnetic resonance imaging depicted midventricular hypertrophy and a dyskinetic thin apical wall, which were confirmed by angiography. Coronary angiograms showed no narrowing of the major extramural coronary arteries, but there was compression of aberrant coronary arteries apparently feeding the hypertrophic portion of the left ventricular wall. Stress thallium-201 myocardial imaging showed a persistent severe defect in the left ventricular apex. A hemodynamic study revealed low cardiac output and an intraventricular pressure gradient (approximately 90 mmHg) between the left ventricular apical high-pressure chamber and the subaortic low-pressure chamber. The present case represents a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and an apical aneurysm in an elderly woman. Myocardial ischemia may have played an important role in the genesis of the apical aneurysm.     

Hypertrophic cardiomyopathy with apical aneurysm: a case of catheter and surgical therapy of sustained monomorphic ventricular tachycardia.

The case is presented of a patient with hypertrophic cardiomyopathy, midventricular obstruction, apical aneurysm, and very frequent episodes of sustained monomorphic ventricular tachycardia (VT) unresponsive to common antiarrhythmic drugs. Left ventricular catheter mapping during sinus rhythm suggested the presence of an extensively scarred apical region; early fractionated ECGs were recorded at the neck of the aneurysm during monomorphic VT, suggesting a possible role of this region as VT substrate. Radiofrequency delivery at these sites stopped the VT and it was no longer inducible; however, it spontaneously recurred the following day. An apical aneurysmectomy, guided by the results of catheter mapping, was performed and was successful in preventing arrhythmic recurrences during 12 months' follow up.     

经胸超声心动图评价高血压心脏病与左心室流出道梗阻的应用价值

目的:应用经胸超声心动图(transthoracic echocardiography,TTE),评价高血压心脏病与肥厚型心肌病(HCM)左心室流出道梗阻的二维及血流动力学特征,为临床鉴别诊断提供影像学依据。方法:左心室流出道梗阻患者31例,其中高血压性左心室流出道梗阻12例,肥厚型梗阻性心肌病19例,二维图像下测量两组患者的室间隔厚度及左心室后壁厚度,在静息状态和激发试验后测量两组患者的左心室流出道流速及最大压差,分别进行组间及组内比较。结果:室间隔厚度肥厚型心肌病组(19.6±1.8)mm明显高于高血压组(12.4±0.6)mm,差异有统计学意义(P<0.05)。左心室后壁厚度:肥厚型心肌病组(11.5±0.5)mm,高血压组(11.3±0.6)mm,两组间比较差异无统计学意义(P>0.05)。高血压组组内比较激发试验后左心室流出道流速(398.6±36.7)cm/s及压差[(68.4±12.9)mmHg,1 mmHg=0.133kPa],均高于静息状态下流速178.2±23.4)cm/s,压差(13.5±6.2)mmHg,差异有统计学意义(P<0.05)。结论:经胸超声心动图能准确评价左心室流出道梗阻的解剖结构与血流动力学特征,可准确鉴别梗阻类型及梗阻程度。     

Mid‐ventricular Obstructive Hypertrophic Cardiomyopathy during Pregnancy Complicated by Preeclampsia and Acute Myocardial Infarction: A Case Report

Hypertrophic cardiomyopathy (HCM) with mid‐ventricular obstruction (MVO) is a rare condition occurring in 1% of HCM patients. It is characterized by asymmetric left ventricular hypertrophy with MVO and elevated intraventricular pressure gradients. Myocardial infarction has been associated with mid‐ventricular obstructive HCM. Briefly, this case presents an unusual clinical scenario where a young pregnant woman complicated by preeclampsia presents with myocardial injury and hemodynamic compromise related to undiagnosed HCM with MVO illustrating hemodynamic challenges created by pregnancy and surgery.