Successful Catheter Ablation of Hemodynamically Unstable Monomorphic Ventricular Tachycardia in a Patient with Hypertrophic Cardiomyopathy and Apical Aneurysm

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular (LV) apical aneurysm represent a previously under-recognized but important subgroup within this heterogeneous disease spectrum. Apical aneurysms and the contiguous areas of myocardial fibrosis have been associated with monomorphic ventricular tachycardia (VT) and increased risk for adverse clinical events including sudden cardiac death, prioritizing the application of primary prevention implantable defibrillators. However, VT may be repetitive, thereby raising considerations for additional treatment strategies such as radiofrequency ablation. In this report, we describe such a patient with HCM and apical aneurysm in whom the mapping and ablation procedure was effective in identifying and abolishing the VT focus.     

Continuous apex to left ventricle blood flow pattern in hypertrophic cardiomyopathy with apical aneurysm and midventricular obstruction

A 42-year-old female presented with chest pain, noted to have systolic and diastolic murmurs on physical examination and left ventricular hypertrophy on the electrocardiogram. Echocardiography with contrast enhancement revealed hypertrophic cardiomyopathy (HCM) with apical aneurysm and mid left ventricular cavity obstruction. Doppler interrogation showed continuous systolic and diastolic flow from the apex to left ventricle (LV) with a transient deceleration giving rise to a notched pattern. Myocardial ischemia of the apex was demonstrated on adenosine nuclear scintigraphy without epicardial coronary obstruction on angiography. This case demonstrates a novel Doppler pattern with continuous apex to LV flow in HCM in the setting of apical ischemia that may account for the formation of the aneurysm.     

Nonobstructive Hypertrophic Cardiomyopathy with Left Ventricular Aneurysm: The Role of Cardiac Magnetic Resonance

Hypertrophic cardiomyopathy with concomitant left ventricular aneurysm is rare and has important clinical implications, including an increased risk of sudden cardiac death. Most patients with this rare combination have obstructive hypertrophic cardiomyopathy, but we treated a 26-year-old woman who had nonobstructive hypertrophic cardiomyopathy and a family history of probable sudden cardiac death. In our patient, coronary angiograms showed distal occlusion of the left anterior descending coronary artery. Late gadolinium-enhanced cardiac magnetic resonance images showed scattered fibrosis within and beyond the left ventricular aneurysm. Precautionary therapy with an implantable cardioverter-defibrillator yielded an uneventful outcome. Cardiac magnetic resonance has emerged as a promising method for diagnosing these aneurysms and detecting associated myocardial fibrosis, thereby enabling patient risk stratification and the determination of appropriate therapeutic options. We discuss the role of cardiac magnetic resonance imaging in the management of this rare clinical entity.Key words: Cardiomyopathy, hypertrophic, familial/complications/pathology/therapy; coronary aneurysm/complications/diagnosis/therapy; death, sudden, cardiac/etiology; defibrillators, implantable; gadolinium/diagnostic use; image enhancement/instrumentation/methods; magnetic resonance imaging; risk assessmentNonobstructive hypertrophic cardiomyopathy (HCM) with left ventricular (LV) apical aneurysm is a rare clinical entity for which diagnostic, prophylactic, and therapeutic approaches are evolving. We describe the case of a patient who was diagnosed with this combination of conditions, and we discuss the role of cardiac magnetic resonance (CMR) in the therapeutic management of such patients.     

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.     

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.     

Association of ST elevation with apical aneurysm in hypertrophic cardiomyopathy

Objectives

Apical aneurysms in patients with hypertrophic cardiomyopathy (HCM) represent an underrecognized but clinically important subset of HCM patients. However it may be frequently missed by echocardiography because of poor image quality of left ventricular apex. We aimed to compare electrocardiographic STE in HCM patients with and without apical aneurysm.

Methods

We developed this clinical review using an extensive MEDLINE review of the literature and data from our laboratories; and some electrocardiographic parameters including STE were analysed in HCM patients with and without apical aneurysm.

Results

There were 29 HCM patients without apical aneurysm (Group 1; 52.6 ± 17.7years, 69% male) and 28 HCM patients with apical aneurysm (Group 2; 59.6 ± 13.2years, 57% male). The STE in V4-6 derivations were statistically more frequent in patients with apical aneurysm compared to those without aneurysm (93% vs 7%, p < 0.001). There was a positive correlation between the presence of the STE in V4-6 derivations and the presence of the apical aneurysm (Spearman''s ρ = 0.895, p < 0.001).

Conclusions

Clinicians and specifically echocardiographers must pay special attention on the electrocardiography to correctly detect the frequently overlooked apical aneurysm in HCM patients, and should be careful for apical aneurysm particularly in the presence of STE in V4-6 derivations.     

肥厚型心肌病猝死危险因素分层

The natural history of hypertrophic cardiomyopathy(HCM)is extremely heterogeneous.Many patients remain asymptomatic throughout life,some develop severe symptoms of heart failure,but others die suddenly.often in the absence of previous symptoms and at a young age.Therefore,identification of those patients at high risk of sudden death represents a major clinical problem and has become an even greater challenge since the implantable cardioverter-defibrillator(ICD)has proved to be highly effective in preventing sudden death in HCM.Patients who have survived a cardiac arrest,or one or more episodes of sustained ventricular tachycardia,are considered to be at high risk and are candidates for an ICD.However,this patient subset represents a small proportion of the HCM population.The greatest difficulty concerns the identification of high risk patients who are candidates for primary prevention of sudden death with a prophylactic ICD.Decisions are based on generally accepted clinical markers which are associated with increased risk,including:family history of sudden death,extreme left ventricular(LV)wall thickness(≥30 mm),nonsustained ventricular tachycardia on Holter monitoring,unexplained(non-neurocardiogenic)syncope particularly in young patients,and hypotensive blood pressure response to exercise.Patients with end-stage HCM or a LV apical aneurysm represent important arrhythmogenic subsets also associated with substantially increased risk.Multiple or single strong risk markers are associated with increased sudden death risk and iustify consideration for a prophylactic ICD.     

多普勒组织成像评价肥厚型心肌病左室舒张功能

目的应用多普勒组织成像脉冲技术测量二尖瓣环舒张速度,以评价肥厚型心肌病左室舒张功能.方法对90例肥厚型心肌病患者及50例正常人进行常规超声心电图及多普勒组织成像检查,测量各房室内径,室壁厚度,射血分数及二尖瓣环各点舒张早期峰值速度(Ea)、舒张晚期峰值速度(Aa).两组指标比较采用成组t检验.结果肥厚型心肌病患者室间隔厚度(25.5±6.6)mm,左室后壁厚度(9.9±2.3)mm,左室内径(42.9±5.9)mm,左房内径(39.9±4.7)mm,LVEF(71.9±4.3)%,二尖瓣血流E/A为1.42±0.7.肥厚型心肌病患者Ea较正常人减低.Aa无明显差异.结论肥厚型心肌病左室长轴主动松弛功能较正常人减低.     

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.     

The Hypertrophic Cardiomyopathy Phenotype Viewed Through the Prism of Multimodality Imaging: Clinical and Etiologic Implications

Noninvasive contemporary imaging with echocardiography and cardiovascular magnetic resonance (CMR) provide comprehensive characterization of the hypertrophic cardiomyopathy (HCM) heart including precise definition of left ventricle (LV) wall thickness and reliable identification of morphologic abnormalities of the mitral valve, LV chamber, and myocardial tissue characterization with late gadolinium enhancement (LGE) (fibrosis). Imaging also contributes to identification of patients at risk for sudden death including novel high-risk features such as LV apical aneurysm and extensive LGE. Exercise (stress) echocardiography should be considered to demonstrate physiologic provocation of LV outflow gradients and to distinguish from patients with nonobstructive HCM. Multimodality imaging identifies patients who are optimal candidates for invasive septal reduction therapy and directs preoperative planning for extended myectomy and to optimize alcohol septal ablation. Contemporary imaging interwoven with current management strategies have resulted in a low HCM-related mortality rate.     

Failure of gated SPECT and echocardiography to detect large apical aneurysm secondary to hyperdynamic left ventricular wall at the aneurysm neck

We present a patient with a history of coronary artery disease and exertional angina after an acute anterior myocardial infarction. Angiography and ventriculography revealed multivessel coronary artery disease and a large apical aneurysm. Echocardiography and gated SPECT studies were performed for further evaluation of ischemia and assessment of left ventricular function. Gated SPECT and echocardiography failed to detect a large apical aneurysm due to a hyperdynamic left ventricular wall at the neck of the aneurysm. This case demonstrates the importance of using multiple imaging modalities in the evaluation of ventricular function in the setting of coronary artery disease.     

Tissue-level inflammation and ventricular remodeling in hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a common cardiac condition caused primarily by sarcomeric protein mutations with several distinct phenotypes, ranging from asymmetric septal hypertrophy, either with or without left ventricular outflow tract obstruction, to moderate left ventricular dilation with or without apical aneurysm formation and marked, end-stage dilation with refractory heart failure. Sudden cardiac death can occur at any stage. The phenotypic variability observed in HCM is the end-result of many factors, including pre-load, after-load, wall stress and myocardial ischemia stemming from microvascular dysfunction and thrombosis; however, tissue level inflammation to include leukocyte-derived extracellular traps consisting of chromatin and histones, apoptosis, proliferation of matrix proteins and impaired or dysfunctional regulatory pathways contribute as well. Our current understanding of the pathobiology, developmental stages, transition from hypertrophy to dilation and natural history of HCM with emphasis on the role of tissue-level inflammation in myocardial fibrosis and ventricular remodeling is summarized.

     

Hypertrophic cardiomyopathy with left ventricular dilatation

There is increasing interest in the notion that some patients with hypertrophic cardiomyopathy (HCM) progress to morphological and functional manifestations similar to those of dilated cardiomyopathy (DCM). From 165 consecutive patients with HCM, 20 patients with left ventricular dilatation (left ventricular end-diastolic diameter greater than or equal to 50 mm) were selected and designated as dilated HCM. The diagnosis of HCM was established in these patients either by detection of the classical form of HCM in family members, with 2-dimensional echocardiographic evidence of asymmetric septal hypertrophy (ASH; septal thickness greater than or equal to 15 mm and a ratio of septal to posterior wall thickness greater than or equal to 1.3); or by demonstrating myocardial fiber disarray in autopsy or biopsy samples. The clinical manifestations of these patients with dilated HCM were then compared with those of other forms of HCM without left ventricular dilatation; 1) 40 patients with hypertrophic obstructive cardiomyopathy (HOCM) who had resting intraventricular pressure gradients of 20 mmHg or more, 2) 80 patients with non-obstructive HCM, each of whom had ASH of the entire ventricular septum (typical ASH), and 3) 25 non-obstructive patients whose hypertrophy was localized to the apical region of the ventricular septum (apical ASH). Patients having apical hypertrophy with a spade-like configuration on the left ventriculogram were excluded from the study. Compared with HOCM and typical ASH groups, the patients with dilated HCM had family histories of significantly more frequent HCM and less frequent hypertension. The patients with dilated HCM also had significantly less fractional shortening (FS), decreased interventricular septal thickness, greater left ventricular end-diastolic pressure (LVEDP), and left ventricular dilatation. During the follow-up period (average: 3.5 years), seven patients (35%) with dilated HCM died; five from congestive heart failure (CHF), one suddenly, and one three days following mitral valve replacement. The other five patients had CHF at the time of their follow-up examination. The patients with apical ASH had clinical features similar to those of dilated HCM; a higher familial frequency, less marked septal hypertrophy, and higher LVEDP. They tended to develop left ventricular dilatation, associated with reduced fractional shortening, although left ventricular diameter at end-diastole did not exceed 50 mm. These findings suggested that dilated HCM is not a rare condition. It is observed in 12% of consecutive patients with HCM.(ABSTRACT TRUNCATED AT 400 WORDS)     

Left ventricular apical aneurysm associated with normal coronary arteries following cardiac surgery: Echocardiographic features and differential diagnosis

Left ventricular aneurysms mainly occur in patients with transmural myocardial infarction caused by left anterior descending coronary artery occlusion. Left ventricular apical aneurysm is rarely found in patients with normal coronary arteriograms, and even rarer in these patients following cardiac operations. We analyzed 37 patients with postoperative left ventricular apical aneurysm, including 1 case from our hospital and 36 cases from the literature; 23 cases (62%) had left ventricular apical true aneurysms and 14 cases (38%) had left ventricular apical pseudoaneurysms, all confirmed at surgery and/or angiography. All cases, with the exception of one, had previously undergone cardiac surgery under cardiopulmonary bypass with apical venting. Although left ventriculography is generally regarded as the gold standard for diagnosis of ventricular aneurysm, echocardiography is an accurate and sensitive method in the evaluation of left ventricular apical aneurysm. Differential diagnosis of left ventricular apical aneurysm includes takotsubo cardiomyopathy, post transapical approach for transcatheter aortic valve implantation, and left ventricular diverticulum.     

Cardiac arrest and ventricular tachycardia in Japanese-type apical hypertrophic cardiomyopathy

Apical hypertrophic cardiomyopathy (HCM) is a specific variant of HCM. This disease has been first described in Japan where the prevalence is much higher than in the western world. The prognosis of apical HCM with regard to sudden cardiac death is believed to be better than that of common HCM. We present, however, two male caucasian patients with apical HCM and malignant arrhythmias. Both patients had marked apical hypertrophy on echocardiography, 'giant' negative T-waves on the ECG and spade-like configuration of the left ventricle on angiography. The first patient had been successfully recussitated from cardiac arrest at the age of 52 years. The second patient had a syncope at the age of 42 years and had non-sustained ventricular tachycardia. In both cases, a cardioverter-defibrillator was implanted and treatment with verapamil was initiated. These observations suggest that the risk of sudden cardiac death might be increased not only in common HCM, but also in Japanese-type apical HCM.     

肥厚型心肌病的特殊表现形式

目的分析不典型肥厚型心肌病的特殊表现（临床及影像），提高临床诊断能力。方法系统分析9例病人的病史、临床表现及其影像学特征，互为补充印证以得出正确的结论。结果5例以胸痛伴缺血性ST－T改变者中3例被诊断为左室非对称肥厚型，2例确诊为心失肥厚型；2例以心源性哮喘为特征者均被诊断为左室梗阻型；2例以进行性浮肿伴浆膜腔积液为特征者1例确诊为双心室肥厚型，另1例则为肥厚型心肌病伴进行性心脏扩大。结论重视肥厚型心肌病的特殊表现，综合系统分析是得出正确诊断的关键。     

Morphological onset and early diagnosis in apical hypertrophic cardiomyopathy: a long term analysis with nuclear magnetic resonance imaging

Objectives. A long-term follow-up study with nuclear magnetic resonance (NMR) imaging was undertaken to detect the morphological onset and to establish the early diagnosis in apical hypertrophic cardiomyopathy (HCM).Background. A spadelike configuration on left ventriculogram (LVG) is regarded as a diagnostic criterion for the classical apical HCM. There also exists a segmented hypertrophy at the apical level without indicating the spadelike features (a nonspade configuration). To detect the hypertrophied myocardium of the nonspade configuration, circumferential scrutiny of the apex is required. Although both configurations can be underlying causes of giant negative T waves, etiological relationship between the two is not clarified.Methods. The criteria for the spadelike configuration defined on left ventricular short-axis NMR images were as follows: (apical maximal thickness ≥15 mm), (apical anterior thickness over basal anterior thickness ≥1.3) and (apical posterior thickness over basal posterior thickness ≥1.3). Thirteen patients who had predominant hypertrophy (≥15 mm) at the apical level without the spadelike configuration underwent NMR imaging twice before and after 54 ± 10 months’ follow-up.Results. Apical hypertrophy that had been confined to the lateral wall in four, the anterior-lateral wall in two, and the septal-anterior wall in one developed to become circumferential hypertrophy that fulfilled the criteria for the spadelike configuration after the follow-up period.Conclusions. The spadelike configuration can begin with the nonspade configuration and therefore, both can constitute a single disease entity of apical HCM. The early diagnosis of apical HCM can be achieved by identifying the hypertrophy frequently confined to the lateral wall at the apical level.     

Advanced sequelae of apical hypertrophic cardiomyopathy: report of two cases with wall motion abnormalities

In view of the paucity of reports describing symptoms of increased degree, and deterioration of left ventricular systolic function in patients with apical hypertrophic cardiomyopathy (apical HCM), two cases with congestive heart failure and progressive thinning of previously hypertrophied apical portions of the left ventricle are reported. These were among 13 patients observed from eight to 10 years. Case 1: A 56-year-old man was diagnosed as having apical HCM at the age of 49 years. Severe left ventricular hypertrophy and prominent ST-T changes were observed on ECG during his first admission. His left ventricular end-diastolic pressure (LVEDP) was 24 mmHg and a left ventriculo-gram revealed a decrease in the left ventricular cavity in the apex and marked hypertrophy of the apical wall. Moderate interstitial fibrosis without hypertrophy or disarray of myocytes was observed in a left ventricular endomyocardial biopsy specimen. In two episodes of cardiac arrest he was successfully resuscitated at the age of 50 years. At the age of 55 years, two-dimensional echocardiography revealed thinning and abnormal motion in the apical wall, and a defect in 201T1 accumulation was observed in the same region by perfusion scintigraphy. This patient was readmitted with a diagnosis of cerebral embolism at the age of 56 years. Cardiac catheterization revealed normal LVEDP (8 mmHg), and a left ventriculogram revealed an aneurysm in the left ventricular apex with normal major epicardial coronary arteries. He has been under treatment with antiarrhythmic medications, calcium antagonists and anticoagulants, and has become relatively asymptomatic. Case 2: A 69-year-old-man was diagnosed as having apical HCM after a complete evaluation, including cardiac catheterization, at the age of 59 years. His LVEDP was elevated (17 mmHg), and a left ventricular angiogram revealed marked hypertrophy localized to the apex. Ejection fraction was 64%. A left ventricular endomyocardial biopsy revealed interstitial fibrosis without hypertrophy of myocytes. Thereafter, he has been followed as a New York Heart Association functional class III to IV with occasional elevation of cardiac enzymes but without chest pain or acute changes in his ECGs. However, atrial fibrillation with complete right bundle branch block developed at the age of 60 years. Apical wall thinning and dyskinesis were diagnosed by 2D echocardiography and a defect in the 201T1 accumulation was observed at about 65 years of age. He was readmitted in severe cardiac failure at the age of 69 years, and he was diagnosed as having cardiac asthma with pulmonary capillary wedge pressure of 35 mmHg.(ABSTRACT TRUNCATED AT 400 WORDS)     

Severe Midventricular Hypertrophic Obstructive Cardiomyopathy and Apical Aneurysm

A 40-year-old man was found to have hypertrophic cardiomyopathy (HCM) with severe mid ventricular obstruction. The obstruction produced two distinct left ventricular chambers with an estimated 60 mmHg continuous wave (CW) Doppler intraventricular gradient. Pulsed wave (PW) Doppler showed high velocity systodiastolic flow from apex to base and flow from base to apex confined mostly to the second half of diastole. Cardiac magnetic resonance (CMR) showed midventricular obstruction, due to septal, parietal, and to an hypertrophic, double posteromedial papillary muscle; an apical aneurysm was detected. Aneurysm is underdiagnosed by echocardiography in HCM and an accurate anatomic definition is needed if surgery is planned; thus, a CMR should always be obtained in these patients.     

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.