Hypertrophic cardiomyopathy with mid-ventricular obstruction complicated by apical aneurysm appearing as delayed contrast hyperenhancement on magnetic resonance imaging: two case reports

Case 1: A 52-year-old man presented with a chief complaint of palpitation. Diabetes mellitus was pointed out in 1992. Electrocardiography (ECG) revealed left ventricular hypertrophy in 1997. He visited our department in October 1997. Echocardiography showed increased wall thickness at the interventricular septum. The diagnosis was hypertrophic cardiomyopathy. Holter ECG revealed nonsustained ventricular tachycardia in December 1997. After this, he visited our outpatient clinic. Echocardiography indicated ventricular aneurysm in January 2002, so he was hospitalized in March 2002. Case 2: A 64-year-old woman was transferred to our hospital because of chest discomfort and tachycardial attack. She had been treated for hypertension and diabetes mellitus. She was taken to a hospital by ambulance. On admission, ECG showed wide QRS tachycardia. Cardiac magnetic resonance imaging in both patients disclosed almost complete obstruction of the mid-ventricle in the systolic phase on long- and short-axis cine images, and gadolinium delayed imaging revealed contrast hyperenhancement corresponding to an apical ventricular aneurysm on both long- and short-axis images. The final diagnosis was mid-ventricular obstructive hypertrophic cardiomyopathy with apical aneurysm characterized by delayed hyperenhancement on magnetic resonance imaging with gadolinium.     

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.     

Mid-ventricular hypertrophic obstructive cardiomyopathy presenting with acute myocardial infarction

Mid-ventricular hypertrophic obstructive cardiomyopathy is a rare type of cardiomyopathy that can be accompanied by apical aneurysm. We report the case of a patient who presented with ventricular fibrillation, ST-segment elevation on electrocardiography, and cardiac-enzyme elevation, in the presence of normal coronary arteries. Echocardiography and magnetic resonance imaging showed an hourglass appearance of the left ventricle with an aneurysm in the apex. Left-heart catheterization and continuous-wave Doppler echocardiography revealed a pressure gradient between the apical and basal chambers of the left ventricle. Impaired coronary artery circulation might play a role in the development of mid-ventricular obstruction in patients with mid-ventricular hypertrophic obstructive cardiomyopathy.     

Arrhythmogenic left ventricular apical aneurysm in hypertrophic cardiomyopathy

A 70-year old lady with prior myectomy for hypertrophic obstructive cardiomyopathy presented with sustained ventricular tachycardia. She was found to have a large left ventricular (LV) apical aneurysm. Surgical intervention was not advised, due to the risk of creating a small LV cavity after surgery and ICD was not advised based on the risk of injuring a very thin walled aneurysm. The patient's arrhythmia settled on medical therapy, but unfortunately she suffered an unwitnessed death three months later.This case represents a rare complication to a rare disease with limited management options. In such patients evidence based medicine is of little help, if any.     

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.     

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.     

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.     

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.     

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。     

Sustained ventricular tachycardia as a first manifestation of hypertrophic cardiomyopathy with mid‐ventricular obstruction and apical aneurysm in an elderly female patient

Sustained ventricular tachycardia complicating left ventricular apical aneurysms has been reported previously solely in middle‐aged patients with hypertrophic cardiomyopathy and mid‐cavity obstruction. We report a case of an elderly female patient who presented with incessant ventricular tachycardia as the first clinical manifestation of hypertrophic cardiomyopathy with mid‐ventricular obstruction and apical aneurysm.     

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.     

Beneficial effect of dual-chamber pacing for a left mid-ventricular obstruction with apical aneurysm.

Sustained ventricular tachycardia (VT) developed in a 63-year-old woman. The 2-dimensional echocardiogram revealed left mid-ventricular obstructive hypertrophy and a discrete apical chamber. A continuous wave Doppler signal across the mid-ventricular narrowing exhibited early systolic ejection flow and diastolic paradoxical jet flow from the apex to the basal chamber, implying a significant systolic and diastolic intraventricular gradient with a high apical pressure. The left ventriculogram confirmed a mid-ventricular obstruction with an apical aneurysm. Invasive assessment of intraventricular pressure showed a peak-to-peak gradient greater than 100 mmHg. Treatment with antiarrhythmic agents could not prevent the VT, but dual-chamber pacing reduced the intraventricular pressure gradient and suppressed the VT completely. Continuous wave Doppler showed that the early systolic ejection flow from the apex had disappeared, that there was isovolumetric relaxation flow toward the apex and that there was attenuation of the diastolic paradoxical jet flow toward the basal chamber. Such findings by continuous wave Doppler can be useful in pacing therapy for evaluating changes in the severity of mid-ventricular obstruction.     

Mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) complicated with coronary artery disease: a case report

Mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) is a rare type ofcardiomyopathy that can be accompanied by apical aneurysm.We presented here a case report of MVHOCM with cornary artery disease.The sixty-four years old man was sent to hospital because ofventricular tachycardia.Large inversion T wave was showed on electrocardiography in the presence of abnormal coronary arteries and normal cardiac enzyme.Echocardiography showed an hourglass appearance of the left ventricle with an aneurysm in the apex and a pressure gradient between the outflow tract of left ventricle and the middle of the left ventricle was revealed by left-heart catheterization.     

Apical Hypertrophic Cardiomyopathy: Preliminary Attempt at Palliation with Use of Subselective Alcohol Ablation

We report a case of severe apical hypertrophic cardiomyopathy in order to discuss the nature of this unusual condition and the possibility of using selective alcohol ablation to effectively treat symptomatic hypertrophic cardiomyopathy that presents with apical aneurysm. A 73-year-old woman with severe, progressive dyspnea and intermittent chest pain was found to have localized left ventricular apical dyskinesia distal to an obstructive mid-distal muscular ring. The ring caused total systolic obliteration of the apical left ventricular cavity. Apical cavity pressure was extremely high, up to 330 mmHg—200 mmHg above that in the main left ventricular cavity. Because of the danger of apical rupture and clot formation, we attempted the experimental use of alcohol ablation for effective palliation. We present our pilot experience, offer a novel interpretation of the nature of this obscure entity, and possibly justify a new catheter treatment. In addition, we discuss the developmental, pathophysiologic, and clinical implications of this unusual form of hypertrophic cardiomyopathy. To our knowledge, ours is the first reported use of subselective, modified-protocol alcohol septal ablation to treat an obstructive mid-apical muscular ring in a patient with apical hypertrophic cardiomyopathy.Key words: Cardiomyopathy, hypertrophic/complications/epidemiology/physiopathology/therapy; ethanol/administration & dosage/therapeutic use; heart septum/pathology; hypertrophy, left ventricular/diagnosis; myocardial ischemia/complications; treatment outcomeHypertrophic obstructive cardiomyopathy (HOCM) is a congenital heart condition characterized by ventricular hypertrophy and a progressive worsening of symptoms, as related to the worsening of the subaortic gradient.^1,2 Either traditional surgical septal myomectomy^3,4 or alcohol septal ablation (ASA)^2,4,5 can be used to treat HOCM by effectively reducing the subaortic gradient.^1,2 We describe the case of an elderly woman who had the rarer variant of hypertrophic cardiomyopathy (HCM)—that with apical aneurysm and mid-ventricular obstruction. We describe in detail the experimental use of subselective alcohol ablation to treat the obstructive mid-apical muscular ring in this patient, and we revisit the nature of apical HCM.     

Assessment of pathophysiology based on the left ventricular shape in five patients with midventricular obstructive hypertrophic cardiomyopathy

OBJECTIVES: The pathophysiology of midventricular obstructive hypertrophic cardiomyopathy (MVO) is unknown. Patients with MVO and MVO-like cardiomyopathy were classified into three groups based on the cardioimaging morphological characteristics of the left ventricle to investigate their complications and treatment. METHODS: Four patients with MVO and one patient with disease-like MVO were admitted in our hospital from 1999 to 2005. Group A consisted of one patient with indications of pressure gradient at mid-ventricle without apical aneurysm, Group B consisted of three patients with indications of pressure gradient and apical aneurysm, and Group C consisted of one patient with hour-glass appearance with apical aneurysm and decreased left ventricular systolic function without pressure gradient. RESULTS: The diagnosis was established during examination for sustained ventricular tachycardia (SVT, three patients), paroxysmal atrial fibrillation (one patient), and coronary artery disease (one patient). Cardiogenic embolization was observed in all cases which originated from atrial fibrillation (one case) and apical aneurysm (two cases). No embolic event occurred in any patient after warfarin therapy. SVT occurred in patients in Groups B and C. SVT refractory to beta-blocker and mexiletine was treated by amiodarone. Apical aneurysmectomy and cryoablation could prevent recurrent SVT with drug resistance. CONCLUSIONS: Four of the five patients with MVO had arrhythmia (atrial fibrillation, SVT) and three had cardiogenic embolization. MVO could be classified into three groups depending on the morphological characteristics and complications. Treatment of MVO should be based on these characteristics.     

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.     

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.     

Sustained cavity obliteration and apical aneurysm formation in apical hypertrophic cardiomyopathy

OBJECTIVES: In patients with apical hypertrophic cardiomyopathy (ApHCM), we estimated the severity of cavity obliteration (CO) in the apical potion of the left ventricle and correlated it with various clinical findings including apical aneurysm. BACKGROUND: Apical hypertrophic cardiomyopathy sometimes develops apical aneurysm. The apical CO is often exhibited in ApHCM along with apical hypertrophy and ischemia. It remains unclear, however, how the CO and others are related to aneurysm. METHODS: In 46 patients with ApHCM, we measured CO time on M-mode echocardiography and corrected it by the R-R interval (cCOT). We divided the 46 patients into the following groups: 17 with cCOT 200 to 350 ms (severe CO group). We then compared apical aneurysm, hypertrophy, ischemia, QT interval, and the like between the three groups. RESULTS: The severe CO group exclusively comprised 11 patients having apical aneurysm and paradoxic jet flow. Of the 11 patients, 10 exhibited irreversible defects on exercise single photon emission computed tomography with thallium-201. All with moderate CO showed reversible defects, and none with no/mild CO showed any defects. Left ventricular hypertrophy and the corrected QT interval (QTc) were largest in the severe CO group. There were high correlations between the cCOT, hypertrophy, ischemia, and QTc. Of the 11 patients with severe CO, 6 had nonsustained ventricular tachycardia and 1 had mural thrombus. CONCLUSIONS: In ApHCM, sustained CO is an important pathophysiologic condition as well as hypertrophy, ischemia, and prolonged QTc, which are considered jointly related to the development of aneurysm through interactions.     

Apical hypertrophic cardiomyopathy followed by midventricular obstruction and apical aneurysm: a case report

A 72-year-old man presented with apical hypertrophic cardiomyopathy followed by midventricular obstruction and apical aneurysm. Doppler echocardiography revealed an abnormal blood flow from the apical aneurysm to the left ventricular outflow through the obstructive lesion during diastole. Myocardial perfusion scintigraphy showed no ischemic change in the apex. This case suggests that apical aneurysm may not be caused by ischemia, although previous reports showed ischemic degeneration or fibrosis may be a cause of an apical aneurysm.