Diagnosis of apical hypertrophic cardiomyopathy using magnetic resonance imaging

Apical hypertrophic cardiomyopathy is an uncommon variant of non-obstructive hypertrophic cardiomyopathy with low prevalence outside East Asia. A case is reported of a non-Asian (European) 51 year old man with characteristic ECG and morphological changes of apical hypertrophic cardiomyopathy. Although the patient underwent catheterisation three years previously because of suggested coronary ischaemic heart disease, apical hypertrophic cardiomyopathy was not diagnosed. More recently, a regional wall motion abnormality was noticed at the apex on echocardiography. To rule out an ischaemic injury a stress perfusion scintigraphy was performed; no perfusion defect was present but an apical tracer enhancement was noted. Further evaluation by magnetic resonance imaging revealed the pathognomonic "ace of spades" configuration of the left ventricle with systolic obliteration of the apical region, which led to the diagnosis of apical hypertrophic cardiomyopathy.


Keywords: apical hypertrophic cardiomyopathy; magnetic resonance imaging     

Myocardial fibrosis in a patient with apical hypertrophic cardiomyopathy detected by delayed-enhanced magnetic resonance imaging

We report a case of apical hypertrophic cardiomyopathy (HCM) presenting with non-sustained ventricular tachycardia. Whole-heart magnetic resonance imaging (MRI) showed normal coronary arteries and confirmed the diagnosis of apical HCM. First-pass gadolinium delayed-enhanced MRI showed local delayed enhancement in the endomyocardium in the hypertrophic lesion. The presence of perfusion defect and delayed enhancement may be a marker of a high-risk patient with apical HCM.     

Cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy

The use of cardiovascular magnetic resonance in patients with hypertrophic cardiomyopathy over the last decade has helped elucidate the diagnosis, prognosis, pathophysiology, and management of this disease. Studies have shown that the use of magnetic resonance imaging in a patient with a permanent pacemaker and implantable cardioverter defibrillator is safe.     

Multimodality imaging in apical hypertrophic cardiomyopathy

Apical hypertrophic cardiomyopathy(AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the firstline imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease.     

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.     

心电图对心尖肥厚型心肌病的诊断价值

心尖肥厚型心肌病（apical hypertrophic cardiomyopathy, AHCM）是1976年由日本学者首先报道的一种特殊类型的心肌病。我国自1984年刘王明心。报道首例AHCM以来的20多年来不断有这方面报道,本文对经超声心动图证实的12例心尖肥厚型心肌病患者的心电图特点进行分析,以便更好的了解和认识AHCM的心电图特点及变化。     

心脏磁共振对肥厚型心肌病应用价值的研究

肥厚型心肌病(HCM)是最常见的遗传性心肌病,在普通人群中其发病率为1/500,HCM临床表现多样,主要发病机制是心肌细胞肥厚、排列紊乱、心肌纤维化及胶原沉积以及不稳定的心电活动容易导致恶性心律失常所致。近年来心脏磁共振(CMR)被广泛应用于HCM的诊断以及与其他疾病的鉴别诊断,CMR不仅能够准确评估心室功能、心室壁厚度,并且钆延迟强化(LGE)能够无创检测心肌纤维化及瘢痕。重要的是越来越多的研究证明LGE与HCM等多种心血管病的发生相关,并可以预测其发生。本文将回顾相关文献,总结CMR对HCM的应用价值,使CMR更好的应用于临床。     

Recognition and diagnosis of apical hypertrophic cardiomyopathy

Recognition and diagnosis of nonobstructive apical hypertrophic cardiomyopathy is important to begin to understand the natural history and prognosis of such patients. Our experience with three patients indicates that a clue to the recognition of apical hypertrophic cardiomyopathy lies in the striking electrocardiographic repolarization changes consistent with subendocardial ischemia often prompting admission to the coronary care unit. The diagnosis of apical hypertrophic cardiomyopathy in two patients was confirmed by two-dimensional echocardiographic apical views, but due to a technically inadequate echocardiogram, the diagnosis in the third patient was made by left ventriculography. Two of the three patients underwent right and left cardiac catheterization and their rest and exercise hemodynamic data were consistent with restrictive cardiomyopathy.     

Long term evolution of magnetic resonance imaging characteristics in a case of atypical left lateral wall hypertrophic cardiomyopathy

We are reporting a long-time magnetic resonance imaging (MRI) follow-up in a rare case of cardiac left lateral wall hypertrophy. Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disorder and a significant cause of sudden cardiac death. Cardiac magnetic resonance (CMR) imaging can be a valuable tool for assessment of detailed information on size, localization, and tissue characteristics of hypertrophied myocardium. However, there is still little knowledge of long-term evolution of HCM as visualized by magnetic resonance imaging. Recently, our group reported a case of left lateral wall HCM as a rare variant of the more common forms, such as septal HCM, or apical HCM. As we now retrieved an old cardiac MRI acquired in this patient more than 20 years ago, we are able to provide the thrilling experience of an ultra-long MRI follow-up presentation in this rare case of left lateral wall hypertrophy. Furthermore, this case outlines the tremendous improvements in imaging quality within the last two decades of CMR imaging.     

Nuclear magnetic resonance in hypertrophic cardiomyopathy

The large differences in the spin lattice relaxation times (T1) of blood and myocardium (measured by nuclear magnetic resonance) allow the heart to be visualised without the use of contrast media. The findings using nuclear magnetic resonance in 11 unselected patients with hypertrophic cardiomyopathy were compared with those in equal numbers of normal subjects and patients with electrocardiographic features of left ventricular hypertrophy. In patients with hypertrophic cardiomyopathy characteristic septal hypertrophy was noted together with variable and sometimes pronounced hypertrophy of the left ventricular free wall, which is consistent with the heterogeneous nature of this disease. The mean (SD) ratio of septal to free wall thickness was 1.5(0.8) for patients with hypertrophic cardiomyopathy, 0.8(0.2) for those with left ventricular hypertrophy, and 0.9(0.2) for normal subjects. Although septal measurements by nuclear magnetic resonance were greater than those obtained by echocardiography there was a significant correlation between the two. Septal and free wall area were significantly smaller in normal subjects. There were no differences in septal or free wall T1 values between the three groups. Non-gated nuclear magnetic resonance can detect septal and free wall hypertrophy. With the addition of multiple slice acquisition, rapid estimation of myocardial mass will be possible allowing the potentially important assessment of progression or regression of myocardial hypertrophy.     

Magnetic resonance imaging in hypertrophic cardiomyopathy

Gated magnetic resonance imaging (MRI) was performed using a 0.35-Tesla cryogenic system in 14 patients with hypertrophic cardiomyopathy (HC) in order to define the site and extent of abnormal wall thickness. These studies were compared with 2-dimensional (2-D) echocardiograms. Gated magnetic resonance imaging studies in 12 normal volunteers were used for comparison. In normal subjects and in patients with HC, the sharp demarcation of the myocardial wall permitted measurement of wall thickness. The thickness of the septal and posterolateral walls in normal subjects was 10.2 +/- 0.4 mm (+/- standard deviation) and 10.8 +/- 0.5 mm, respectively, whereas septal thickness in all but 1 patient with HC was 15.0 mm or greater. In patients with HC, septal and posterolateral wall thickness were 2.2 +/- 0.8 cm and 1.3 +/- 0.17 cm, respectively, by MRI. The 2-D echocardiographic measurements for septal and posterolateral walls were 2.4 +/- 0.6 cm and 1.4 +/- 0.7 cm, respectively. The severity and distribution of abnormal wall thickness were comparable on 2-D echo and MRI. Gated MRI is an effective and completely noninvasive technique for demonstrating the presence, site and extent of abnormal wall thickness in HC. The large field of view, ability to image directly in multiple planes, and discrete blood-endocardial interfaces are advantages for cardiovascular imaging.