左室心尖气球样综合征的诊断与治疗现状

左室心尖气球样综合征主要表现为间歇性左心室功能障碍伴左室心尖部心肌气球样变,但无显著的冠状动脉疾病。现结合近年来发表的相关文献,对其病理生理学特征、临床表现、实验室检查以及治疗现状进行综述。     

Clinical Characteristics,Demographics and Prognosis of Transient Left Ventricular Apical Ballooning Syndrome

Background: Transient left ventricular apical ballooning syndrome also called Tako-Tsubo cardiomyopathy is defined as a syndrome consisting of stress induced apical ballooning of the left ventricle and normal coronary arteries. The majority of the published reports are case reports or case series with a small number of patients. The goal of this study was to perform statistical analysis of reported cases and case series in the literature in order to study demographic, clinical characteristics and prognosis of this syndrome. Method: A PubMed search of the terms transient left ventricular apical ballooning, Tako-Tsubo, takotsubo, and apical cardiomyopathy was conducted for this study. All reports that contained information about individual patients were included in the statistical analysis. Results: The majority of the patients were women (93.5%). Asians were the largest group (57.2%) followed by Caucasians (40%). The presentations mimics an acute myocardial infarction. The most frequent presenting symptom was chest pain (65.9%) followed by dyspnea (16.2%). ST elevation was the most common ECG abnormality (87.5%). Contrary to most reports, this is not a benign disease with complications occurring in 18.9% and death in 3.2%. The most common complication was shock followed by left ventricular thrombus formation and congestive heart failure. Conclusion: Apical ballooning syndrome occurs most commonly in women and is associated with high complication rate. There are significant differences in presenting symptoms between race and gender which warrant further investigation.     

心尖球形综合征的诊断及鉴别诊断

心尖球形综合征的发病机制、诊断、处理及预后均不同与急性冠状动脉综合征,但二者的临床特征类似,很容易误诊误治,因此正确诊断及鉴别诊断具有重要的临床意义。谨就二者的临床特征、心电图、心肌酶学、冠状动脉造影、影象学、心功能等改变的异同,诊断及鉴别诊断进行了综述,并对心尖球形综合征的发病机制进行了探讨。     

Transient Apical Thickening Following Apical Ballooning Syndrome: A Case Series

The authors describe transient apical thickening mimicking apical hypertrophic cardiomyopathy following apical ballooning syndrome. These findings are observed on multimodality cardiac imaging and appear to constitute a novel entity. Possible pathophysiologic mechanisms are explored.     

Apical Thrombus Associated with Takotsubo Cardiomyopathy in a Young Woman

We report a case of Takotsubo cardiomyopathy complicated by left ventricular apical thrombus in a young woman; the thrombus and wall motion abnormalities disappeared after 3 weeks of anticoagulant therapy. These findings suggest that appropriate anticoagulation therapy should be performed in patients with Takotsubo cardiomyopathy until wall motion abnormalities improve .     

短暂性左心室球形性综合征的临床特征及预后研究

目的:研究短暂性左心室球形性综合征(TLVABS)的临床特征及预后.方法:复习2005-09到2008-08行冠状动脉造影检查所有患者的临床及影像学资料,确诊15例TLVABS,并进行临床随访.结果:15例患者中男性2例(13.3%),女性13例(86.7%),平均年龄(63.60±10.43)岁,73.3%(11/15)发病前有应激事件,主要临床表现为胸痛(93.3%)及呼吸困难(20.0%).60.0%(9/15)患者起初的心电图表现为ST段抬高,15例患者肌钙蛋白均轻度升高,86.7%(13/15)表现为伴心尖受累的典型TLVABS,13.3%(2/15)为心尖活动正常的非典型TLVABS.1例(6.7%)有左心室流出道梗阻.住院期间无严重并发症及死亡.14例(93.3%)接受随访,1例(6.7%)失访.平均随访16.95±9.38(5.5～31.5)月,1例(7.1%)复发,1例(6.7%)死于车祸,无心源性死亡及慢性心力衰竭.结论:TLVABS的临床表现酷似急性心肌梗死,有明显应激因素,但预后良好.     

Apical ballooning syndrome in a postoperative patient with normal microvascular perfusion by myocardial contrast echocardiography

Apical ballooning syndrome is classically described as transient left ventricular (LV) dysfunction, marked LV akinesia, and normal or near-normal coronary arteries. The etiology is unclear and there is limited information based on case reports and small case series. We describe a 35-year-old woman who underwent surgical hepatectomy and developed apical ballooning syndrome in the postoperative period. The novel use of myocardial contrast echocardiography (MCE) in this setting demonstrated intact microvascular perfusion and lack of coronary flow-limiting abnormalities despite apical akinesis. In select patients with similar clinical presentations, performing MCE is safe and may be pursued as an alternative to invasive coronary angiography.     

Left Ventricular Apical Thin Point Viewed with Two-Dimensional Echocardiography

The aim of this study was to evaluate the usefulness of two-dimensional echocardiography in observing the left ventricular apical thin point (LVATP) and to view the change in thickness and width of the LVATP during the cardiac cycle. Transthoracic echocardiography was performed in 32 healthy adult volunteers to observe the LVATP in an apical three-chamber view. The width and thickness of the LVATP were measured at the end-diastole as well as at the end-systole. With two-dimensional echocardiography, the LVATP could be clearly shown. The width of the LVATP at the end-diastole and end-systole was 3.3 mm ± 1.4 mm versus 0.9 mm ± 0.4 mm, P < 0.001; the thickness of the LVATP at the end-diastole and end-systole was 1.7 mm ± 0.6 mm versus 1.8 mm ± 0.8 mm, P > 0.05. The LVATP can be viewed with two-dimensional echocardiography; the LVATP changes significantly in width during the cardiac cycle, whereas the thickness of the LVATP changes insignificantly.     

心尖部心肌收缩障碍综合征

心尖部心肌收缩障碍综合征是临床上表现为一过性类似急性心肌梗死样胸痛及心电图改变,冠状动脉造影未见器质性狭窄,左心室造影心尖部收缩功能异常的一类病症。病因尚不清楚,大多在数天至数周恢复正常。     

心尖球形综合征一例

1病例资料患者男性,62岁,因活动性胸闷、气促伴双下肢水肿1周于2010年4月26日入院。入院前一周因受凉后出现活动性胸闷、气促、双下肢水肿,伴咳嗽、咳少量白痰,夜间不能平卧。4月19日曾至外院就诊查心电图：窦速,左室肥厚,     

Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review.

AIMS: To clarify the major features of the apical ballooning syndrome, we performed a systematic review of the existing literature. METHODS AND RESULTS: Review of all relevant case series using the MEDLINE and EMBASE databases resulted in the identification of 14 studies. These studies suggest that the apical ballooning syndrome accounts for approximately 2.0% of ST-segment elevation infarcts, with most cases described in post-menopausal women. The most common clinical presentations are chest pain and dyspnoea, reported in 67.8 and 17.8% of the patients, respectively. Cardiogenic shock (4.2% of the patients) and ventricular fibrillation (1.5%) were not infrequent. ST-segment elevation was reported in 81.6% of the patients, T wave abnormalities in 64.3%, and Q waves in 31.8%. Cardiac biomarkers were usually mildly elevated, as reported in 86.2% of the patients. Typically, patients had left ventricular (LV) dysfunction on admission, with mean ejection fraction ranging from 20 to 49%. However, over a period of days to weeks, all patients experienced dramatic improvement in LV function. The onset of symptoms was often preceded by emotional (26.8%) or physical stress (37.8%). Norepinephrine concentration was elevated in 74.3% of the patients. Prognosis was generally excellent, with full recovery in most patients. In-hospital mortality was 1.1%. Only 3.5% of the patients experienced a recurrence. CONCLUSION: Clinicians should consider this syndrome in the differential diagnosis of patients presenting with chest pain, especially in post-menopausal women with a recent history of emotional or physical stress.     

Apical sparing in tako-tsubo cardiomyopathy

Background: Tako‐tsubo cardiomyopathy (TTC) is an acute reversible cause of segmental myocardial dysfunction that is poorly understood. We have noted a variant of this condition where a tiny segment at the apex retains some contractile function. This paper delineates the frequency of this variant relative to the classical form and the clinical differences between patients suffering from the two forms. Methods: All cases of TTC (n = 35) were identified from our infarct angiography database and separated on the basis of apical sparing (n = 14) or no apical sparing (n = 21). Results: Compared with the classical form, those with apical sparing were significantly younger (63 ± 12 vs 72 ± 13 years) and were more likely premenopausal (5/14 vs 0/21) and had higher ejection fractions (35 ± 6% vs 32 ± 4%). There was a trend towards higher recurrence (4/21 vs 0/14). There were no differences in time or season of presentation, precipitant stressor, premorbid drug therapy, haemodynamics at catheterization or acute outcomes. Conclusion: The apical sparing variant of TTC is common, accounting for 40% of cases. While the patients are younger and more likely premenopausal, there are no other distinguishing features between the classical and the variant form.     

肥厚型心肌病的特殊亚型——合并左心室心尖部室壁瘤

肥厚型心肌病合并有左心室心尖部室壁瘤发生率虽然较低,但是一类未得到充分认识的临床亚型,其合并较高的心血管事件发生率和猝死率,及时识别这类患者并启动抗凝、抗心力衰竭及心律转复除颤器植入可改善临床预后.     

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.     

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.