Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients

In 30 of 1,002 consecutive patients who had left heart catheterization and cineangiography for evaluation of either ischemic heart disease or cardiomyopathy the electrocardiogram showed giant negative T waves (greater than 10 mm) associated with high QRS voltage (R wave greater than 26 mm in lead V₅ or the sum of the S wave in lead V₁ and the R wave in lead V₅ 35 mm or more) in the precordial leads despite absence of hypertension or significant coronary artery disease. In all 30 patients a characteristic spade-like configuration (concentric apical hypertrophy) was observed in the right anterior oblique ventriculogram at end-diastole as well as in the long axis two dimensional echocardiogram.The average apical thickness in these patients (24.8 ± 6.6 mm) was significantly greater than that in normal subjects (9.4 ± 3.1 mm) (P < 0.001) or in patients with hypertrophic obstructive cardiomyopathy (14.7 ± 5.0 mm) (P < 0.001). Values for both the mid anterior free wall thickness (13.9 ± 4.1 mm) and the mid posterior free wall thickness (14.3 ± 3.0 mm) were greater than values in normal subjects (8.9 ± 1.8 mm and 8.2 ± 2.0 mm, respectively) (P < 0.001). However, the ratio between the apical and the mid anterior free wall thickness in these 30 patients (1.86 ± 0.53) was significantly greater than the ratio in normal subjects (1.05 ± 0.24), patients with hypertrophic obstructive cardiomyopathy (0.96 ± 0.15) (P < 0.001) and patients with types of nonobstructive hypertrophic cardiomyopathy (1.26 ± 0.24) (P < 0.005) other than apical concentric hypertrophy. Obstruction of the tract did not occur because the upper half of the septem remained rather thin in systole and did not bulge into the left ventricle during systole. Pressure study with proper provocations as well as two dimensional echocardiograms revealed no peak systolic pressure gradient or obstruction within the outflow tract of the left ventricle.It is concluded that these 30 patients have nonobstructive hypertrophic cardiomyopathy with marked concentric hypertrophy in the apex (apical hypertrophic type) and with a different septal shape and contraction pattern from those seen in the obstructive type. This type of hypertrophy appears to be a fairly common type of hypertrophic cardiomyopathy in Japan.     

Systolic apical filling in a patient with progressive apical hypertrophic cardiomyopathy and giant negative T waves

A relatively asymptomatic 58-year-old nonhyper-tensive male developed changes of remarkable apical hypertrophic cardiomyopathy associated with deep negative T-wave change and marked ventricular dyssynergy without apparent precipitating cause over a nine-year period.     

Factors predicting coronary artery disease in patients with giant negative T waves

Seventy-four patients with giant negative T waves were studied to determine which electrocardiographic variables predicted the presence of coronary artery disease. The absence of left ventricular hypertrophy and the presence of symmetric T-wave inversion predicted coronary artery 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.     

运动及药物对心尖肥厚性心肌病巨大倒置T波的影响

对３例心尖肥厚性心肌病（ＡＨＣＭ）患者进行下蹲运动试验、异丙肾上腺素及普萘洛尔试验，观察各自对巨大倒置Ｔ波的影响。结果表明，３例在运动后及静滴异丙肾上腺素后巨大倒置Ｔ波均明显变浅或转直立，而在口服普萘洛尔后Ｔ波倒置明显加深。恰与冠心病患者相反。其原因可能为：（１）心肌病患者的复极由内层向外层进行，Ｔ波倒置，异丙肾上腺素增强心肌内层张力，使内层复极延迟，致复极过程由外向内扩散，Ｔ波直立，（２）ＡＨＣ     

Assessment of the cranial involvement pattern of giant cell arteritis with 3T magnetic resonance imaging

OBJECTIVE: To noninvasively determine the involvement pattern of the cranial arteries in giant cell arteritis (GCA), with high-resolution magnetic resonance imaging (MRI). METHODS: The superficial cranial arteries of 21 patients with suspected GCA were examined using a 3T high-field MRI scanner. Postcontrast T1-weighted spin-echo images were acquired with submillimeter spatial resolution, to assess mural thickness and lumen diameter of the major cranial arteries on both sides of the head. In all cases, MRI results were compared with findings of clinical examination and laboratory tests. In addition, temporal artery biopsy specimens from 10 patients were examined by histology. RESULTS: MRI sharply revealed all of the major superficial cranial arteries, allowing for an evaluation of their lumen and vessel wall. Nine of the 21 patients were diagnosed as having GCA according to the criteria of the American College of Rheumatology. In all of these patients with clinically diagnosed GCA, multiple cranial arteries showed signs of inflammation on MRI. In 1 patient, the occipital arteries were inflamed, while the temporal arteries were spared. CONCLUSION: Postcontrast high-resolution MRI visualizes the major cranial arteries on both sides of the head within a single examination. The cranial involvement pattern in GCA can be assessed precisely and noninvasively. In the majority of GCA patients, several cranial arteries were affected simultaneously, with a predominance of involvement of the frontal branch of the superficial temporal artery. Inflammation of the occipital arteries, with sparing of the temporal arteries, was also encountered.     

Evaluation of transient apical ballooning with cardiac magnetic resonance imaging and 31-phosphorous magnetic resonance spectroscopy

Apical ballooning is an increasingly reported transient cardiomyopathy with yet unknown origin. In this study 2 cases of apical ballooning are described in whom we used a combined approach of cardiac magnetic resonance imaging (CMR) and 31-Phosphorous magnetic resonance spectroscopy (31P MRS). Electrocardiogram showed ST abnormalities and cardiac serum markers were mildly elevated, but CAG demonstrated smooth coronary arteries. Cine-CMR revealed severe apical akinesia and significantly decreased ejection fraction. Furthermore we detected reduced myocardial phosphocreatine to beta-ATP (PCr/b-ATP) ratios during the first week of acute disease. After 1 week we observed an improvement of PCr/b-ATP ratios by 68% and 34%, which was associated with an increase in left ventricular function. Our data suggest that 31P MRS might be a valuable tool in the evaluation of apical ballooning, but larger cohorts are needed to improve the understanding of metabolic changes during transient apical ballooning.     

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.     

Bone marrow patterns in patients with aplastic anaemia and myelodysplastic syndrome: observations with magnetic resonance imaging

Abstract: Bone marrow magnetic resonance imaging (MRI) was obtained in 48 patients with myelodysplastic syndrome (MDS) (35 cases) or aplastic anaemia (AA) (13 cases). The lower thoracic and lumbar spine were evaluated on sagittal plane using a 1.5 Tesla superconducting MR unit with a surface coil. Pulse sequence of STIRs (TR 2000 msec, TI 160 msec, TE 20 msec) were applied. Four distinct patterns of signal intensity (SI) on the STIR images were classified as follows: pattern 1, homogeneously low SI; 2, marginally high SI; 3, heterogeneously high SI; 4, homogeneously high SI. In all 13 patients with AA, STIR images initially revealed pattern 1. In 25 of 35 cases with MDS patients, the STIR images were initially classified as pattern 3. The STIR images of 6 AA and 5 MDS patients with a clinical response to treatment showed pattern 2 similar to that of normal marrow distribution. The STIR images of MDS patients showed an abnormal distribution of SI. Significant signal changes in the STIR images can be observed in successive examinations of the patients, thus facilitating follow-up of the disease and treatment. MRI of the bone marrow provides a noninvasive means of grossly examining a large fraction and is a useful technique in patients with aplastic anaemia or myelodysplastic syndrome.     

Cerebral abnormalities in patients with cirrhosis detected by proton magnetic resonance spectroscopy and magnetic resonance imaging

Hepatic encephalopathy is a common problem in cirrhosis. The pathogenesis of this complication of advanced liver disease still remains unclear. Magnetic resonance spectroscopy was used to assess prospectively cerebral metabolism in 51 patients with histologically proven cirrhosis (Child-Pugh classes A, B, and C, 18, 18, and 15, respectively) and 36 healthy volunteers. According to the results of psychometric tests, overt hepatic encephalopathy, subclinical encephalopathy, and no encephalopathy were found in 14, 21, and 16 patients, respectively. Myoinositol/creatine ratios in gray (.36 +/- .17) and white (.35 +/- .22) matter voxel were reduced significantly (P < .0001) in cirrhotic patients compared with healthy volunteers (gray matter, .51 +/- .11; white matter, .64 +/- .16). In addition, patients showed a significant reduction (P = .024) in white matter choline/creatine ratio (.77 +/- .27) compared with controls (.92 +/- .25), and glutamine/glutamate level was elevated in cirrhotic patients compared with controls (gray matter, P < .0001; white matter, P = .036). Changes in cerebral myoinositol and glutamine/glutamate levels correlated significantly with the severity of hepatic encephalopathy (P < .0001). However, these metabolic alterations were also detected in patients without hepatic encephalopathy (normal psychometric test results). N-acetyl aspartate/creatine ratios did not differ between patients and controls. Magnetic resonance imaging detected bright basal ganglia in 37 patients, which correlated significantly with portal-systemic shunting and elevation of glutamine/glutamate, but not with the degree of hepatic encephalopathy. In conclusion, magnetic resonance imaging and spectroscopy showed that alterations of cerebral metabolism are common in patients with cirrhosis, even without evidence of clinical or subclinical hepatic encephalopathy.(Hepatology 1997 Jan;25(1):48-54)     

磁共振成像在心内膜心肌活检阴性心肌炎患者中的诊断价值:回顾性病例系列研究

目的分析临床诊断心肌炎但心内膜心肌活检(EMB)阴性患者的心脏磁共振(CMR)表现,探讨CMR在此类患者中的诊断价值。方法该研究为回顾性分析。纳入符合2013年欧洲心脏病学会(ESC)心肌炎临床诊断标准,但EMB阴性的患者14例。所有患者均行CMR检查,通过黑血序列、电影序列、T2W-STIR序列及钆对比剂延迟增强(LGE)序列,评价心脏的形态学、功能学、组织学改变。结果该研究共纳入14例符合临床诊断标准的心肌炎患者,男10例,女4例,年龄为(25.6±13.2)岁,症状发作至行CMR检查时间间隔为21(13,60)d,症状发作距离EMB检查时间为19(9,40)d。CMR阳性患者13例,表现为心肌水肿、纤维化、射血分数减低、心包积液或心腔增大等。14例患者中共9例患者存在CMR形态和/或功能异常,其中左心房增大1例,左心室增大1例,右心室增大3例,左心室收缩末期容积指数超过正常范围4例,左心室射血分数<50%3例,右心室射血分数<40%5例,心包积液深度>3 mm 3例。14例患者中,有11例存在组织学改变,其中6例患者的T2比值≥2;LGE阳性10例(10/14),最常见的强化模式为左心室侧壁心外膜下和/或室间隔肌壁间(9例),另外左心室壁广泛心内膜下强化2例,无右心室壁强化。结论CMR能够在体评估心脏形态、功能及组织学改变,对于EMB阴性的心肌炎患者,CMR具有良好的补充诊断价值。     

A case of asymmetrical apical hypertrophy which is a form of hypertrophic nonobstructive cardiomyopathy with giant negative T-waves

Clinical, hemodynamic, electrocardiographic (ECG), echocardiographic, left ventricular (LV), and coronary angiographic (CA) findings are reported in a case with apical hypertrophy (AH), a form of hypertrophic nonobstructive cardiomyopathy (HNCM). The most striking symptom was chest pain and the most conspicuous electrocardiographic finding consisted of giant negative T waves, reaching an amplitude of 4.0 mV. Echocardiography revealed an apical thickness of the septum and posterior wall of 40 mm; this was significantly greater than septal and posterior free wall thickening in the LV outflow area. The anterior motion (SAM) of the anterior mitral leaflet, was present, and, in hemodymic investigation, the isoproterenol test was negative. The left ventricular end-diastolic pressure (LVEDP) and the EF were elevated. In the LV angiogram from the right anterior oblique position (RAO), the LV free wall thickness at the apex was significantly thicker than at the outflow tract level. The patient had dilated coronary arteries. We conclude that these findings are typical for AH (HNCM) and it seems that hypertrophic obstructive cardiomyopathy (IHSS, MO), and hypertrophic non-obstructive cardiomyopathy (ASH, AH) are different manifestations of a wide spectrum of hypertrophic cardiomyopathy.     

3.0 T magnetic resonance imaging scanning on different body regions in patients with pacemakers

Purpose

Magnetic resonance imaging (MRI) at 3.0 T is becoming more common, but there is a lack of sufficient evidence on the safety of a 3.0 T scan in patients with pacemakers. This study aimed to investigate the safety and practical concerns of 3.0 T scans for patients with MR-conditional pacemakers.

Methods

Twenty consecutive patients were enrolled. A standardized protocol was developed by cardiologists, pacemaker engineers, and radiologists. Pacemaker interrogation was performed immediately before and after the scan. Scan-related adverse events were documented, and imaging quality was graded as level 1 to 4 by radiologists.

Results

Twenty-three MRI scans of different body regions (brain?=?13, lumbar spine?=?4, cervical spine?=?2, and heart?=?4) were performed, and the average time of a scan was 25?±?11 min. No significant changes in sensing amplitude (atrial 3.1?±?1.1 mV vs. 2.9?±?1.2 mV, P?=?0.71; ventricular 9.3?±?3.5 mV vs. 10.2?±?3.4 mV, P?=?0.46), lead impedances (atrial 647?±?146 Ω vs. 627?±?151 Ω, P?=?0.7; ventricular: 780?±?247 Ω vs.711?±?226 Ω, P?=?0.36), or pacing threshold (atrial 0.6?±?0.2 V/0.4 ms vs. 0.6?±?0.2 V/0.4 ms, P?=?0.71; ventricular 0.7?±?0.3 V/0.4 ms vs. 0.7?±?0.2 V/0.4 ms, P?=?0.85) were observed pre- and postscan. No adverse events were detected. Image quality review showed grade 1 quality in 16 patients and grade 2 quality in 4 patients with artifacts of pulse generators and leads in cardiac MRI scan and no impact on diagnostic value.

Conclusion

Our initial data indicated that 3.0 T scanning might be feasible under a standardized protocol with good diagnostic imaging quality irrespective of body region in patients with MR-conditional pacemakers.