An advanced form of familial hypertrophic cardiomyopathy showing massive myocardial fibrosis with intramural small arterial thickening. An autopsy case

An autopsy case of an advanced form of hypertrophic cardiomyopathy (HCM) showing marked fibrosis with intramural small arterial abnormalities is presented in this report. A 52-year-old woman, who had a positive family history of HCM, was admitted because of palpitations. The chest roentgenogram showed a mildly enlarged cardiac silhouette and the electrocardiogram revealed abnormal Q waves and R wave and T wave abnormalities. The echocardiogram revealed hypokinesis with thinning of the interventricular septum and the anterior wall of the left ventricle. Percutaneous right ventricular endomyocardial biopsies demonstrated moderate interstitial fibrosis with small arterial thickening. At necropsy, the anterior and posterior walls of the left ventricle and the interventricular septum were markedly thinned and showed a massive transmural fibrosis. Moreover, the intramural small arteries, 50-300 microns in diameter, showed marked intimal and medial hypertrophy with proliferation of elastic fibers and smooth muscle cells. From these findings, it is suggested that this was originally a case of HCM which progressed to a decompensated stage because of the abnormal intramural small arteries. The significance of small arterial lesions in HCM is discussed.     

Quantitative assessment of ultrasonic myocardial reflectivity in hypertrophic cardiomyopathy

The purpose of this study was to investigate the relation between acoustic properties of the myocardium and magnitude of left ventricular hypertrophy in patients with hypertrophic cardiomyopathy. An on-line radio frequency analysis system was used to obtain quantitative operator-independent measurements of the integrated backscatter signal of the ventricular septum and posterior free wall in 25 patients with hypertrophic cardiomyopathy and 25 normal age-matched control subjects. The integrated values of the radio frequency signal were normalized for the pericardial interface and expressed in percent. Tissue reflectivity was significantly increased in the hypertrophied ventricular septum, as well as in the nonhypertrophied posterior free wall, in patients with hypertrophic cardiomyopathy (58 +/- 15% and 37 +/- 12%, respectively) compared with values in normal subjects (33 +/- 10% and 18 +/- 5%, respectively; p less than 0.001). Furthermore, measurements of reflectivity of the septum or posterior free wall, or both, were beyond 2 SD of normal values in greater than 90% of the patients and were also abnormal in each of the five study patients who had only mild and localized left ventricular hypertrophy. No correlation was identified between myocardial tissue reflectivity and left ventricular wall thickness in the patients with hypertrophic cardiomyopathy (correlation coefficient r = 0.4; p = NS). These findings demonstrate that myocardial reflectivity is abnormal in most patients with hypertrophic cardiomyopathy and is largely independent of the magnitude of left ventricular hypertrophy. Moreover, quantitative analysis of ultrasonic reflectivity can differentiate patients with hypertrophic cardiomyopathy from normal subjects independently of clinical features and conventional echocardiographic measurements.     

Sudden death in hypertrophic cardiomyopathy with normal left ventricular mass.

An active, healthy, and symptom free 16 year old boy with a family history of hypertrophic cardiomyopathy died suddenly while walking home from school. Necropsy showed absence of left ventricular hypertrophy (that is, normal heart weight), though the characteristic histological abnormalities of hypertrophic cardiomyopathy, such as cardiac muscle cell disorganisation and abnormal intramural coronary arteries, were present. It is likely that this patient had hypertrophic cardiomyopathy and died before left ventricular hypertrophy developed.     

Comparison of diastolic septal perforator flow velocities in hypertrophic cardiomyopathy versus hypertensive left ventricular hypertrophy

In this study, we measured diastolic septal perforator flow velocities by Doppler transthoracic echocardiography (TTE) in patients with hypertrophic cardiomyopathy (HCM). Using color-guided pulsed Doppler TTE, septal perforator flow velocity recordings were attempted in 69 patients and successfully recorded in 47 (68%). First, we compared 14 patients with HCM to 12 controls and to 11 patients with hypertension with left ventricular hypertrophy. Next, in 10 additional patients with HCM, we compared the septal velocities with the epicardial left anterior descending artery (LAD) velocities recorded during the same TTE study. In the patients with HCM, the peak septal diastolic velocities were twice that of the normal controls (88 +/- 40 vs 41 +/- 13 cm/s) and also higher than in hypertensive left ventricular hypertrophy (51 +/- 18 cm/s, p < 0.0001). All 10 patients with HCM showed a step-up of peak diastolic velocity from the LAD to the septal perforator from 41 +/- 9 to 72 +/- 17 cm/s (p < 0.0001). Three patients with HCM had surgical septal myectomy. These patients had luminal narrowings of the small intramural arteries at histopathologic examination. In conclusion, pulsed Doppler measurement of septal perforator flow velocities is feasible. In HCM, the epicardial coronary arteries enlarge to accommodate increased flow, and diastolic velocity is normalized. In contrast, the increased velocities in the septal branches of patients with HCM are similar to those previously observed in tunnel-like obstructions. These findings suggest that in HCM, notwithstanding an increase in coronary flow, hemodynamically significant narrowings are present in the septal branches. Doppler TTE may become useful for evaluation of abnormal intramural coronary flow in HCM.     

Magnetic resonance imaging in familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion and cardiac enzymes

Gated magnetic resonance imaging (MRI) was performed in 6 patients with familial hypertrophic cardiomyopathy associated with abnormal thallium perfusion, and 12 patients with ordinary hypertrophic cardiomyopathy. The patients with ordinary hypertrophic cardiomyopathy and abnormal thickening of the septal wall and normal left ventricular dimensions, while the patients with familial hypertrophic cardiomyopathy had focal wall thinning (usually involving the apical-septal wall) and dilated left ventricle in addition to hypertrophied heart. The quantitative measurement for cardiac dimensions using MRI was similar to that found on echocardiography in all cases. In addition, inhomogeneous signal intensities at left ventricular wall were observed in 3 cases of familial hypertrophic cardiomyopathy, which may suggest the existence of myocardial fibrosis. Gated MRI should be performed for early detection and follow-up of hypertrophic cardiomyopathy, since some patients will progress from hypertrophic cardiomyopathy to dilated cardiomyopathy.     

Prevalence and characteristics of disporportionate ventricular septal thickening in infants with congenital heart disease.

Necropsy studies were performed in 125 infants, 2 years old or less, with a variety of congenital heart malformations. Disproportionate ventricular septal thickening (septal-to-left ventricular free wall thickness ratio greater than or equal to 1.3) was present in 31 (25%) of the 125 patients. In the majority of patients, an abnormal septal-to-free wall ratio did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy, since: 1) marked disorganization of septal myocardium (involving greater than or equal to 5% of the tissue section) was present in only five of the 31 patients with disproportionate septal thickening; and 2) echocardiographic studies in first degree relatives of four other patients with disproportionate septal thickening and normal septal architecture did not show asymmetric septal hypertrophy. The relatively high prevalence of septal-to-free wall ratios of 1.3 or greater in this population of infants with congenital heart disease appeared to be due in part to minor differerences in ventricular wall thicknesses (in patients with relatively thin absolute wall thicknesses) which may produce particularly large deviations from unity in septal-to-free wall ratio. Hence, 1) nongenetically transmitted disproportionate septal thickening is relatively common in infants with congenital heart diseases studied at necropsy: and 2) a septal-to-free wall ratio of greater than or equal to 1.3 cannot be used as the sole criterion for identifying associated hypertrophic cardiomyopathy in infants with other congenital heart diseases, particularly if marked absolute septal thickening is absent.     

Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries

Clinical and morphologic features of transmural myocardial infarction (associated with insignificant or absent atherosclerosis of the extramural coronary arteries) are described in seven patients with hypertrophic cardiomyopathy. Marked chronic congestive heart failure associated with supraventricular arrhythmias occurred in six of the seven patients, each of whom had no or mild left ventricular outflow tract obstruction under basal conditions. No patient had typical angina pectoris, and only one patient had clinically evident acute myocardial infarction. Infarction may have caused cardiac arrest in one other patient, but was “silent” in the remaining five patients.

At necropsy, six of the seven patients had extensive myocardial scarring involving the ventricular septum, left ventricular free wall and one or both left ventricular papillary muscles; in four patients portions of the right ventricular wall were also scarred. Six patients had dilated ventricular cavities, including two who were known to have nondilated ventricular cavities earlier in their clinical course.

It is concluded that transmural myocardial infarction in the absence of significant coronary atherosclerosis is a not uncommon finding (prevalence rate 15 percent) in a population of patients who had died from hypertrophic cardiomyopathy. Although transmural infarction is possibly a secondary event, it more likely contributes causally to the clinical deterioration of some patients with hypertrophic cardiomyopathy, leading to ventricular dilatation and progressive fatal cardiac failure.     

Prevalence and characteristics of disproportionate ventricular septal thickening in patients with coronary artery disease

Echocardiographic or necropsy studies were performed in 151 patients with coronary artery disease. Prevalence of disproportionate septal thickening (septal to free wall ratio greater than or equal to 1.3) was 11%. An abnormally increased septal-free wall ratio in these patients had two principal etiologies. First, it was a manifestation of genetically transmitted hypertrophic cardiomyopathy, as evidenced by disproportionate septal thickening in first degree relatives. Second, it was due to disproportionate septal thickening which did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy. This latter conclusion was suggested by negative echocardiographic studies in some families of patients with both coronary artery disease and disproportionate septal thickening. In addition, numerous disorganized cardiac muscle cells, characteristically present in patients with genetically transmitted hypertrophic cardiomyopathy, were absent from the septum of all patients with disproportionate septal thickening studied at necropsy. Although the mechanism responsible for this secondary type of disproportionate septal thickening is unknown, our results indicate that the presence of disproportionate septal thickening in a patient with coronary artery disease does not, per se, indicate the coexistence of genetically transmitted hypertrophic cardiomyopathy.     

Impaired myocardial perfusion in patients with hypertrophic cardiomyopathy: Assessment with digital subtraction coronary arteriography

Summary To study the clinical significance of abnormal myocardial perfusion in patients with hypertrophic cardiomyopathy (HCM), we performed a computerized washout analysis of digital subtraction coronary arteriograms in 28 patients with HCM and 16 control subjects. The contrast disappearance half-life (T 1/2) was calculated from a time-density curve generated in the four sectors of the myocardium perfused by the left anterior descending coronary artery and the mean T 1/2 was calculated by averaging T 1/2 values for these four sectors. Patients with HCM demonstrated longer T 1/2 in the ventricular septal region than control subjects. Thirteen (46%) of the patients with HCM presented abnormally longer mean T 1/2 values, suggesting impaired myocardial perfusion. Family histories of HCM were more frequent in patients with abnormal mean T 1/2 values (92% vs 47%;p<0.05). On the exercise stress test, patients with abnormal T 1/2 values presented significantly lower exercise tolerance with more frequent exercise-induced ST segment depression (62% vs 13%;p<0.05). However, there were no significant differences between the two groups with regard to ventricular wall thickness, left ventricular end-diastolic pressure, or the severity of systolic narrowing of the coronary arteries.These findings suggest that 13 (46%) of the patients with HCM have impaired myocardial perfusion, which may be a manifestation of intramural coronary artery disease in addition to left ventricular hypertrophy, elevated left ventricular end-diastolic pressure, or systolic narrowing of the coronary arteries. Additionally, significant association of the prolonged T 1/2 with a familial occurrence of HCM and depressed exercise tolerance with ST segment depression imply that impaired myocardial perfusion could be an important inherent pathophysiological state leading to myocardial ischemia during exercise.This study was supported in part by a Research Grant for Intractable Diseases from the Ministry of Health and Welfare of Japan.     

Diagnosis of cardiac amyloidosis based on the myocardial velocity profile in the hypertrophied left ventricular wall

The myocardial velocity profile (MVP), derived from color-coded tissue Doppler imaging (TDI), can identify transmural heterogeneity based on the physiology and pathology of the myocardium. This study sought to clarify whether the MVP can differentiate cardiac amyloidosis from other causes of left ventricular hypertrophy. We recorded the MVP and determined its myocardial velocity gradient (MVG) in the ventricular septum and left ventricular posterior wall using color-coded TDI in 10 patients with cardiac amyloidosis, in 25 patients with hypertensive hypertrophied left ventricular wall, in 25 patients with asymmetric septal hypertrophy of hypertrophic cardiomyopathy, and in 20 clinically normal controls. End-diastolic ventricular septal thickness was similar among the cardiac amyloidosis, hypertension, and hypertrophic cardiomyopathy groups. Percent systolic thickening of the ventricular septum and left ventricular posterior wall calculated from M-mode left ventricular echocardiograms was lower in the cardiac amyloidosis group than in the hypertension, hypertrophic cardiomyopathy, or control group. Peak MVGs during systole and early diastole were lowest in the cardiac amyloidosis group, followed, in order, by the control, hypertension, and hypertrophic cardiomyopathy groups. The systolic and early diastolic MVPs in the ventricular septum and left ventricular posterior wall showed a characteristic serrated pattern in all patients with cardiac amyloidosis, but not in any other patient groups. In conclusion, MVPs in the ventricular septum and left ventricular posterior wall show a distinctive serrated pattern that may be related to amyloid deposition in the myocardium. Myocardial tissue characterization using color-coded TDI provides diagnostic information in patients with cardiac amyloidosis.     

心壁错构性发育不良与原发性心肌病

目的 探讨心壁错构性发育不良的病理形态表现、特点、类型及其与原发性心肌病的关系.方法 以2004年6月至2007年12月底阜外心血管病医院进行心脏移植的92例心脏为观察对象,在心脏离体后立即进行了肉眼观察、测量和摄影记录,并进行了全面的病理组织学观察.结果 心壁的错构性发育见于多种类型的心脏病,且在原发性心肌病中更为多见,差别只是错构的严重程度和分布范围的不同.心壁内存在轻、中度错构不一定就是疾病,但过多的出现能明显影响心脏的结构和功能,只有某种类型的错构性发育较集中或突出时,病理和临床方面才呈现出某一类型的错构性心肌病.错构的范围和程度与原发性心肌病的心脏扩张表现和心力衰竭出现的早晚有一定的正相关趋势.92例中58例为原发性心肌病,34例为非原发性心肌病,原发性心肌病中有25例为错构性心肌病,占原发性心肌病的43.1%.心壁错构性发育异常的病理形态表现主要有:(1)心壁的过度增殖肥厚;(2)纤维/脂肪组织或脂肪/纤维组织替代;(3)心肌细胞的排列紊乱;(4)冠状动脉结构不良等.错构性心肌病一般是一种错构形式为主的多种错构形式组合,相同临床表现的错构性心肌病,其病理基础不完全相同,纤维-脂肪替代为主的多见于扩张型心肌病和致心律失常性右心室心肌病,心肌排列紊乱多伴随于肥厚型心肌病,冠状动脉结构不良常因心肌缺血而扩张.结论 心壁错构性发育不良是心脏结构变异的常见形态表现,只有错构的分布比较密集、弥漫时才表现出某一特定类型的心肌病,错构比较密集、弥漫或伴有严重心肌变性者易表现出心脏扩张和心力衰竭.     

Systolic compression of epicardial coronary and intramural arteries in children with hypertrophic cardiomyopathy

It has been suggested that systolic compression of epicardial coronary arteries is an important cause of myocardial ischemia and sudden death in children with hypertrophic cardiomyopathy. We examined the associations between sudden death, systolic coronary compression of intra- and epicardial arteries, myocardial perfusion abnormalities, and severity of hypertrophy in children with hypertrophic cardiomyopathy. We reviewed the angiograms from 57 children with hypertrophic cardiomyopathy for the presence of coronary and septal artery compression; coronary compression was present in 23 (40%). The left anterior descending artery was most often affected, and multiple sites were found in 4 children. Myocardial perfusion abnormalities were more frequently present in children with coronary compression than in those without (94% vs 47%, P = 0.002). Coronary compression was also associated with more severe septal hypertrophy and greater left ventricular outflow gradient. Septal branch compression was present in 65% of the children and was significantly associated with coronary compression, severity of septal hypertrophy, and outflow obstruction. Multivariate analysis showed that septal thickness and septal branch compression, but not coronary compression, were independent predictors of perfusion abnormalities. Coronary compression was not associated with symptom severity, ventricular tachycardia, or a worse prognosis. We conclude that compression of coronary arteries and their septal branches is common in children with hypertrophic cardiomyopathy and is related to the magnitude of left ventricular hypertrophy. Our findings suggest that coronary compression does not make an important contribution to myocardial ischemia in hypertrophic cardiomyopathy; however, left ventricular hypertrophy and compression of intramural arteries may contribute significantly.     

Fatal myocardial embolus after myectomy

Coronary embolism is an infrequent phenomenon. A 56-year-old man with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation who underwent left ventricular septal myectomy and mitral valve annular repair is presented. The patient had a cardiac arrest 36 h after surgery. Cardiac standstill, tamponade and a left ventricular rupture were noted when the chest was opened during attempted resuscitation. Autopsy revealed an occlusive embolus of myocardium in the proximal left anterior descending coronary artery. It showed pathological features of hypertrophic cardiomyopathy. There was an extensive acute transmural anteroseptal left ventricular myocardial infarction with rupture of the anterior free wall. Embolism of myocardium - to the coronary arteries, the systemic circulation or the pulmonary circulation - is a rare event, with only nine other cases reported in the literature in the past 30 years. This is the first reported case of myocardial embolus to a coronary artery in a patient with hypertrophic obstructive cardiomyopathy following septal myectomy.     

Prevalence of dignostic abnormalities in patients with genetically transmitted asymmetric septal hypertrophy

Echocardiographic studies were performed in 100 patients from a general population with cardiac disease and in 33 patients with classic hypertrophic obstructive cardiomyopathy and 116 of their first degree relatives. The findings were compared with those in 35 normal persons. The prevalence rate of asymmetric septal hypertrophy (ventricular septal to free posterior wall ratio greater than 1.3) was 8 percent in the general population with heart disease. No further clinical or echocardiographic evidence was found for a familial disease. The ventricular septal to free posterior wall ratios for this group and the normal subjects had a unimodal distribution curve. All patients with hypertrophic obstructive cardiomyopathy demonstrated asymmetric septal hypertrophy, a decreased systolic septal thickening of less than 25 percent and a characteristic left ventricular shape in the cross-sectional echocardiogram. The ventricular septal to posterior wall ratios in their 116 relatives had a bimodal distribution curve; in 35 relatives the ratio indicated asymmetric septal hypertrophy and in 81 the ratio was normal. In addition, all 35 relatives with echocardiographic evidence of asymmetric septal hypertrophy had decreased systolic septal thickening (less than 25 percent) and in 17 the echocardiographic left ventricular shape was similar to that in patients with hypertrophic obstructive cardiomyopathy. In contrast, the 81 relatives who had no asymmetric septal hypertrophy had normal systolic septal thickening and a normal left ventricular shape. The clinical examination, electrocardiogram and chest X-ray film were less sensitive than the echocardiogram in detecting diagnostic abnormalities in the 35 relatives with asymmetric septal hypertrophy.It is concluded that echocardiographically assessed asymmetric septal hypertrophy can be considered the anatomic marker for hypertrophic cardiomyopathy only when, in addition, a decreased systolic septal thickening and, to a lesser degree, an abnormal left ventricular shape are present. The asymmetric septal hypertrophy in these cases probably represents the anatomic expression of a genetic defect that has an autosomal dominant pattern of inheritance. In so-called “borderline” cases with echocardiographic signs of an abnormal ventricular septal to posterior wall ratio, a definitive clinical diagnosis of hypertrophic cardiomyopathy could be made only after echocardiographic screening of family members for the presence of asymmetric septal hypertrophy.     

Obstructive hypertrophic myocardiopathy and coronary atherosclerosis

The purpose of this study was to determine the coexistence of idiopathic hypertrophic cardiomyopathy (IHC) and atheromatous coronary artery disease. We studied forty six patients with IHC documented by complete clinical study and also by left heart catheterization. The diagnosis was considered established, when the patient had a significant left intraventricular pressure gradient (LIPG) and by angiographic and or echocardiographic demonstration of systolic anterior movement of the mitral valve and asymmetric septal hypertrophy. In 15 patients a selective coronary angiography was performed because ischemic myocardial disease was suspected. There were eight patients with significant atherosclerotic obstruction (greater than 75% narrowing). There was one vessel disease in 13%, two vessels in 37% and three vessels in 50% of the patients. The LIPG was 43 +/- 20 mmHg, the left ventricular and diastolic pressure (LVEDP) was 18 +/- 11 mmHg in patients with atheromatous disease and there was no significant difference with the patients with normal coronary arteries with LIPG 52 +/- 31 mmHg and LVEDP 21 +/- 9 mmHg. Our data demonstrate that ischemic heart disease in patients with idiopathic hypertrophic cardiomyopathy is related not only to increase of the cardiac mass and/or the ventricular wall stress, but in some of them atherosclerosis plays a role in its pathogenesis. Significant differences were found between the aged in both groups. In the patients who had coronary atherosclerosis the mean age was 54 years; on the other hand in the patients with normal coronary arteries the mean age was 44 years. This last fact could explain the high frequency of atherosclerosis in the older patients.     

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.     

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.     

Hypertrophic cardiomyopathy without asymmetric hypertrophy.

A 49-year-old women with congestive heart failure and heart block died of cerebral embolism. Clinical and echocardiographic findings suggested a diagnosis of atypical dilated cardiomyopathy with predominantly right ventricular involvement. At necropsy, all the cardiac chambers were slightly dilated and the interventricular septum and the left ventricular wall were of normal thickness and symmetry. Histological examination, however, disclosed extensive disarray of abnormal myocardial tissue, especially in the interventricular septum. Her father had similar clinical and echocardiographic findings, while one of her brothers had typical hypertrophic cardiomyopathy at necropsy. It is likely that the patient actually had inherited hypertrophic cardiomyopathy. The case illustrates the difficulty in diagnosing hypertrophic cardiomyopathy when based solely on the left ventricular gross anatomy.     

老年心尖肥厚型心肌病冠脉造影特征

目的了解老年心尖肥厚型心肌病(APH)患者冠脉造影及心室造影特征。方法对我科1996～2003年7例老年APH患者冠脉造影资料进行分析。结果所有患者左冠脉普遍增粗,肌桥形成,心室造影见左室“黑桃样”改变,5例合并冠脉狭窄。结论老年APH患者以冠脉普遍增粗、肌桥形成及左室“黑桃样”改变为特征性冠脉造影改变,且合并冠心病概率高。     

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.