阜外医院400例心脏移植的临床病理学表现的启示

心脏移植目前已成为治疗终末期心脏病较有效的手段,我们对每例受体心脏都进行了全面的病理学观察,给了我们有助于诊断和治疗的新的启示。在受检的400例心脏中有77.50%病例为不同类型的心肌疾病,15.00%为冠状动脉狭窄性疾病。心肌的病理改变基本有两大类,即损伤性病变和结构性病变,前者是心力衰竭和心肌病类型转变的基本原因,后者是发育异常性和错构性病变,有形态特征,但无明显的疾病类型特异性,是心脏易损性的结构基础。由于基本病变的组合和损及的范围不同,呈现出不同临床和病理表现,引起临床与病理诊断间的差别,诊断不一致的病例以心肌病类最多。另外冠状动脉年龄性内膜纤维增厚和错构导致的狭窄能明显影响疾病的发展和严重程度。     

单纯心壁病损性扩张心脏的病理基础分析

目的观察单纯心壁病损造成心脏扩张者心脏的病理形态特征,为临床和影像提供鉴别诊断参考依据。方法2004年6月至2006年6月底,对阜外心血管病医院进行心脏移植的56例受体心脏在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。以其中单纯心壁病损造成的心脏扩张者进行临床病理对比分析,观察其形态特点。结果56例受体心脏中38例为单纯心壁病损造成的心脏扩张者,占67．9％。这38例中50．0％为原发性扩张型心肌病,23．7％为致心律失常性右心室心肌病,15．8％为缺血性心肌病,其余的10．5％为局灶性心肌致密化不全、巨细胞性心肌炎和特异性心肌病中的酒精性心肌病和高血压性心肌病。38例中临床诊断与病理诊断不相同的有15例,不相符率为39．5％,不相符率较高的依次为巨细胞性心肌炎（100％）、缺血性心肌病（83．3％）和致心律失常性右心室心肌病（77．8％）。不相符的这几种疾病的原临床和影像学诊断都是原发性扩张型心肌病。致心律失常性右心室心肌病、缺血性心肌病、心肌致密化不全和巨细胞性心肌炎都有特征性的病理形态表现,酒精性心肌病和高血压性心肌病等的病理诊断需要参考临床资料。结论进行心脏移植病例受体心脏的病理学检查有利于提高心脏病的临床和影像诊断的正确率,有扩张表现的心脏病的诊断要排除心脏扩张的继发原因,并要注意与终末期心脏疾病作鉴别。     

心肌致密化不全诊断标准的病理学思考

心肌致密化不全是一类心壁心肌发育异常的心肌病,严格地说是一类心壁内层心肌发育不良的疾病,除心壁肌内层过度小梁化外,有的还伴有其他的先天性心脏结构异常,如合并Ebstein畸形等.     

心动过速性心力衰竭

心动过速性心力衰竭 (ｔａｃｈｙｃａｒｄｉａｉｎｄｕｃｅｄｈｅａｒｔｆａｉｌｕｒｅ ,ＴＩ ＨＦ)是一种特殊类型的心力衰竭。它是以持续或间断心动过速作为基本病因 ,导致心脏扩大、心壁变薄 ,出现类似扩张性心肌病样改变 ,表现为充血性心力衰竭 ,故又常称为心动过速性心肌病 (ｔａｃｈｙｃａｒｄｉａｉｎｄｕｃｅｄｃａｒｄｉｏｍｙｏｐａｔｈｙ) ,因其基本病因属于心律失常 ,故也有人称心律失常性心肌病 (ａｒｒｈｙｔｈ ｍｉｃｃａｒｄｉｏｍｙｏｐａｔｈｙ) [1] 。ＴＩＨＦ不仅有心室衰竭 ,亦可累及心房 ,后者称心动过速性心房心肌病…     

致心律失常性右心室心肌病13例临床病理特征分析

目的:探讨致心律失常性右心室心肌病的临床病理特征。方法:收集接受心脏移植术,并确诊为致心律失常性右心室心肌病的病例13例,对受者心脏行大体观察,常规HE染色和PTAH、Masson等特殊染色后光镜观察,同时结合病史、心电图、超声心动图等临床资料进行回顾性分析。结果:(1)临床病史:患者均有心悸、胸闷、气短等症状,心脏功能Ⅱ~Ⅳ级,3例自诉有扩心病家族史,1例为二尖瓣置换术后,1例为埋藏式心律转复除颤器植入术后,1例为心脏射频消融术后。(2)超声心动图及心电图检查:心室运动及功能减低,全心扩大5例,右心室扩大为主2例,左心室扩大为主4例,2例提示为致心律失常性右心室心肌病。心电图显示:室性期前收缩、心房颤动、电轴右偏、不完全性右束支传导阻滞等。(3)心脏移植术前诊断:11例为扩张性心肌病,2例为致心律失常性右心室心肌病。(4)病理学检查:心脏质量256~660g,平均417.9g;7例有双侧心室扩张,5例以右心室扩张为主,1例以左心扩张为主。右心室壁切面呈灰白灰黄色相间,部分为灰黄色,心壁变薄,部分左心室壁略显灰白。4例有室壁瘤形成。镜下表现:主要为右心室的纤维脂肪组织或脂肪组织的浸润,心肌细胞减少,病变可自心外膜达心室肉柱,其中2例左心室镜下病理表现相同,6例伴左心室的纤维化。病理诊断:13例诊断为致心律失常性右心室心肌病,1例伴炎症,7例伴冠状动脉粥样硬化,3例伴附壁血栓。结论:致心律失常性右心室心肌病,病理表现为右心室纤维脂肪组织或脂肪组织的浸润,部分伴发心肌炎,病变可累及左心室,检出率是同期心脏移植病例的7.1%。临床表现和辅助检查缺乏特异性,诊断需结合病史、临床表现、综合包括影像学在内的多方面检查等方能较客观作出诊断,病理学活检有助于明确诊断。     

40例心脏移植术后受体心脏病理诊断与术前临床诊断的对比分析

目的通过比较40例心脏移植前、后诊断的异同来评价终末期心力衰竭患者术前诊断的准确性。方法40例患者术前全部经过询问病史,体格检查,心电图、超声心动图检查。凡有心绞痛症状,超声心动图证实存在节段性室壁运动异常或存在明确的危险因素等条件之一,且病情允许的患者在术前作冠状动脉造影。若≥1支冠状动脉主支的横断面面积减少75％以上被认为是有意义的冠状动脉病变。术前诊断为冠心病的患者全部行双核素检查,评价存活心肌。所有心脏移植术取下的心脏均被称重,测量室壁厚度,并经大体检查和组织学检查。结果心脏移植术前诊断为原发性扩张型心肌病（IDC）占45．0％,酒精性心肌病17．5％,冠心病占17．5％,肥厚型心肌病7．5％。高血压病、瓣膜病、致心律失常性右室心肌病、克山病和心肌致密化不全各1例,各占2．5％。术前诊断为冠心病者,受体心脏均有严重冠状动脉病变。术前诊断为IDC的18例和酒精性心肌病的7例患者,受体心脏病理检查共有8例（占32％）可见严重的冠状动脉病变。术前诊断为高血压心脏病和主动脉瓣换瓣术后心力衰竭的患者各1例,也可见冠状动脉严重病变。术前诊断为IDC和克山病者,6例受体心脏病理诊断为致心律失常性右室心肌病。术前诊断为IDC者1例,在移植取下心脏后被病理诊断为巨细胞心肌炎。结论早期正确诊断心力衰竭的病因,可决定患者不同的治疗方法（血管重建而不是心脏移植）,且能判断移植患者的预后。本研究的结果提示：无论临床表现如何,所有怀疑IDC的患者均应尽早作冠状动脉造影,并尽可能查明其病因。     

15例终末期心脏病的心肌组织病理学观察

目的:探讨终末期心脏病的心肌组织病理改变及特征。方法:对接受心脏移植的9例扩张型心肌病(DCM)患者、6例其他类型终末期心脏病患者[肥厚型心肌病(HCM)2例,缺血性心肌病、心脏横纹肌肉瘤、先天性心脏病房间隔缺损、法洛四联症各1例]及1例正常对照的心肌标本进行组织病理学和电镜检查,并与临床心功能分级对照分析。结果:DCM患者光镜下均可见广泛纤维组织增生,以血管周围最为明显,心功能Ⅲ~Ⅳ级的心肌纤维化程度为,较心功能Ⅱ级者(+)为重;另可见心肌细胞形态异常,但少见间质炎症细胞浸润、心肌细胞排列紊乱及小血管变化;电镜下均可见肌原纤维稀疏,线粒体肿胀、形态不规则,其中呈线粒体空泡样变、巨大线粒体、肌质网明显扩张各1例。1例HCM患者光镜下呈现与DCM者类似的改变,但肌原纤维有断裂现象,细胞排列紊乱,胞核畸形,血管扩张,较多炎症细胞浸润;1例HCM患者及2例终末期先天性心脏病患者的心肌组织病理学改变与DCM患者相似。6例其他类型终末期心脏病患者电镜下的心肌超微结构改变均与DCM患者相似,但未见有巨大线粒体。结论:①心肌纤维化是终末期心脏病心肌组织病理学的主要特征,纤维化程度越重,心功能越差;②心肌细胞排列紊乱及炎症细...     

致心律失常性右室心肌病的研究进展

致心律失常性右室心肌病（ARVC）,又称致心律失常性右室发育不良／心肌病（ARVD／C）为遗传性原发性心肌疾病,呈常染色体显性遗传,为运动猝死中常见的病因,占年轻猝死病的20％,大多数病例死亡时的年龄〈40岁,有些发生于儿童。ARVC的病理特征为右心室内的心肌萎缩和纤维脂肪组织替代。以左束支阻滞图形的单形性室性心动过速为特征的、具有多种临床表型的心肌病。以右心室受累为主,晚期可累及左室。尽管心力衰竭是疾病晚期的重要并发症,但ARVD／C主要表现为室性心律失常和心脏性猝死（SCD）。     

充血型缺血性心肌病诊治体会

充血型缺血性心肌病(CICM)是由于冠状动脉粥样硬化长期心肌缺血引起的心肌弥漫性纤维化所致.主要表现为心脏扩大和充血性心力衰竭.CICM临床表现与原发性心肌病极为相似,诊断困难,治疗效果不满意,愈后不佳.故正确认识CICM有助于本病的及时诊断和治疗.2006年5月-2011年5月共收治12例CICM患者,现将其诊治体会报告如下.     

心肌病诊治进展 扩张型心肌病

扩张型原发性心肌病(DCM)是原因不明心肌病中最常见的类型,其突出的表现是左心室及左心房扩大,亦有两侧心室扩张者。因心脏收缩功能受损,可导致充血性心力衰竭,故又称为充血型原因不明心肌病。DCM的临床表现无特异性,主要表现为进行性心力衰竭、心律失常、房室腔内血栓形成及栓塞并发症、猝死。目前对其尚无理想的治疗方法,本文着重讨论DCM的病因和诊断。1　病因1.1　心肌慢性炎症或后遗症　许多研究证明,DCM可能系病毒感染所致。炎症急性期表现多不明显,大部分患者可完全恢复。少部分患者心脏的病理变化继续发展,心脏逐渐增大,症状和…     

Intramural ("small vessel") coronary artery disease in hypertrophic cardiomyopathy

Many patients with hypertrophic cardiomyopathy have signs and symptoms of myocardial ischemia and dysfunction. Although hypertrophy and increased left ventricular pressure can account for such abnormalities, altered small intramural coronary arteries have also been described in such patients. To determine the prevalence and extent as well as the clinical relevance of abnormal intramural coronary arteries, a histologic analysis of left ventricular myocardium obtained at necropsy was performed in 48 patients with hypertrophic cardiomyopathy (but without atherosclerosis of the extramural coronary arteries) and in 68 control patients with either a normal heart or acquired heart disease. In hypertrophic cardiomyopathy, abnormal intramural coronary arteries were characterized by thickening of the vessel wall and a decrease in luminal size. The wall thickening was due to proliferation of medial or intimal components, or both, particularly smooth muscle cells and collagen. Of the 48 patients with hypertrophic cardiomyopathy, 40 (83%) had abnormalities of intramural coronary arteries located in the ventricular septum (33 patients), anterior left ventricular free wall (20 patients) or posterior free wall (9 patients); an average of 3.0 +/- 0.7 abnormal arteries were identified per tissue section. Altered intramural coronary arteries were also significantly more common in tissue sections having considerable myocardial fibrosis (31 [74%] of 42) than in those with no or mild fibrosis (31 [30%] of 102; p less than 0.001). Abnormal intramural coronary arteries were also identified in three of eight infants who died of hypertrophic cardiomyopathy before 1 year of age. In contrast, only rare altered intramural coronary arteries were identified in 6 (9%) of the 68 control patients (0.1 +/- 0.05 abnormal arteries per section; p less than 0.001) and those arteries showed only mild thickening of the wall and minimal luminal narrowing. Moreover, of those patients with abnormal intramural coronary arteries, such vessels were about 20 times more frequent in patients with hypertrophic cardiomyopathy (0.9 +/- 0.2/cm2 myocardium) than in control patients (0.04 +/- 0.02/cm2 myocardium). Hence, abnormal intramural coronary arteries with markedly thickened walls and narrowed lumens are present in increased numbers in most patients with hypertrophic cardiomyopathy studied at necropsy and may represent a congenital component of the underlying cardiomyopathic process.(ABSTRACT TRUNCATED AT 400 WORDS)     

Left ventricular involvement in right ventricular dysplasia/cardiomyopathy

OBJECTIVE: To characterize pathological features of left ventricular (LV) involvement in right ventricular dysplasia/cardiomyopathy (RVD/C). DESIGN: Retrospective morphological case study. SETTING: Two referral-based university medical centres. MATERIALS: Seventeen hearts were studied: 15 from sudden cardiac deaths outside hospital and two explanted hearts, one removed for intractable arrhythmias and the other for right-sided heart failure. The subjects (three female) were aged 16 to 60 years. MAIN RESULTS: All had typical right ventricular features of RVD/C and morphological evidence of LV wall involvement, seven with microscopic changes only. Of 10 hearts with gross and microscopic lesions, nine had large or laminar segments involved. The LV free wall was affected in all cases and the ventricular septum (VS) in 15. Sixteen hearts were hypertrophied. In involved areas, the LV or VS walls were of 'normal' thickness or slightly thinned. Five histological patterns of involvement were recognized, of which four were found in the LV. More severe LV involvement was seen in the hearts of older patients. Complete transmural fatty replacement of the myocardium was not observed, nor were the LVs aneurysmal. Minimal or mild focal aggregates of inflammatory cells were seen in nine hearts and moderate inflammatory changes in two. Inflammation was usually associated with myocyte atrophy and only rarely with myonecrosis. CONCLUSIONS: This study suggests that patients with RVD/C who live long enough will likely have LV free wall involvement with frequent VS involvement. Pathologists may miss LV involvement on gross examination. It should be sought diligently in patients dying of the condition or receiving transplants for heart failure. Appropriate histological sections from both free wall and septum must be examined.     

Right ventricular dysplasia: morphological findings in 13 cases.

OBJECTIVE: To characterize the pathological features of right ventricular dysplasia (RVD). DESIGN: Retrospective morphological case study. SETTING: Three referral-based university medical centres. PATIENTS: Thirteen subjects (one female) aged 16 to 55 years including 10 necropsy hearts from sudden deaths out of hospital, one explant heart and two partial right ventricular resections from patients with intractable ventricular tachycardia. MAIN RESULTS: Most hearts showed hypertrophy and localized or generalized dilatation of the right ventricle. Transillumination revealed myocardial thinning of variable configuration usually conforming to regions of dilatation. Common sites of involvement were apex, infundibular region and posterobasal wall. Histologically, focal or extensive segments of right ventricular myocardium were absent or replaced. Three patterns were found: right ventricle markedly thinned, epicardium and endocardium contiguous, virtually no intervening tissue; wall normal thickness or thinned, myocardium almost totally replaced by fat; and wall normal or thin, myocardium largely replaced by fat with scattered residual myocardial cells and fibrous tissue (the predominant pattern). Endocardial fibrosis was present in eight cases and focal mononuclear cell infiltrates in 10. Electron microscopy in two cases showed nonspecific findings. CONCLUSIONS: RVD has gross and microscopic features which permit its recognition. While a majority of cases are likely congenital (genetic or acquired in utero), the possibility of postnatally acquired conditions (inflammatory, toxic, ischemic) inducing RVD must be explored. The incidence and importance of RVD as a cause of sudden death can only be assessed by continued systematic and detailed studies of patients with recurrent ventricular tachycardia and of hearts, especially from sudden death victims. Although uncommon, RVD should be considered in the differential diagnosis of arrhythmia and sudden death by both clinicians and pathologists.     

Use of a coronary sinus lead and biventricular ICD to correct a sensing abnormality in a patient with arrhythmogenic right ventricular dysplasia/cardiomyopathy

Implantable cardioverter defibrillators (ICDs) are frequently offered to patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Yet ICDs in these patients may be complicated by poor sensed amplitudes resulting from fatty and fibrous tissue replacement of right ventricular myocardium. We present the case of a patient with ARVD/C who had inappropriate detection of ventricular tachycardia with a single-chamber ICD due to poor sensed right ventricular amplitudes. We discuss how the use of a bipolar coronary sinus lead and a biventricular ICD generator with a novel header configuration solved the problem.     

Constituents of the human ventricular myocardium: connective tissue hyperplasia accompanying muscular hypertrophy

Left-sided congestive heart failure may be secondary to decreased left ventricular myocardial compliance in some patients. To investigate the anatomic basis for altered wall stiffness, morphometric determinations of muscle cell nuclear density and percent of myocardium consisting of muscle cells were made for right and left ventricular free wall and septum in 127 hearts with normal coronary arteries. The hearts were normal (33 patients), had left ventricular hypertrophy (28 patients), right ventricular hypertrophy (25 patients), or chronic dilatation (41 patients). With cardiac enlargement, the average percent of myocardium consisting of muscle did not change from the approximately 75% value characteristic of normal hearts. In contrast, muscle cell nuclear density decreased proportionate to cardiac enlargement, demonstrating that muscle cell hypertrophy, not hyperplasia, is the basis for weight increase. Some hearts with marked longstanding dilatation also had perivascular and interstitital "striae" of connective tissue differing from replacement fibrosis. An increase in epicardial coronary artery caliber commensurate with increased heart weight suggests that ischemia is not the basis of connective tissue increase. The results show that cardiac muscle cell hypertrophy is accompanied by commensurate increase in interstitial connective tissues. This pattern of myocardial growth with cardiac enlargement may produce increased myocardial stiffness simply as a result of increased wall thickness, and may lead to left-sided congestive heart failure.     

Spontaneously occurring hypertrophic cardiomyopathy in the rat. I. Pathologic features

The gross anatomic and microscopic appearance of the hearts of young and adult WKY/NCrj rats was examined in comparison with that of normotensive Wistar and SHR/NCrj rats. In a substantial number of the WKY rats, the heart weight and thickness of ventricular septum were much greater than those of the Wistar and SHR rats. The ventricular septum to left ventricular free wall thickness ratio was greater than 1.3 in about one sixth of the WKY rats. In most of the hypertrophied WKY hearts, the transverse area of the left ventricular cavity was smaller in relation to the wall area than in the Wistar and SHR rat hearts, although in a few it was greater. Abnormal fiber arrangement, myocyte hypertrophy, and myocardial fibrosis were far more prominent in the hypertrophied myocardium of the WKY rats compared with the Wistar or SHR rats. Intramural arteries with marked wall thickening existed frequently in the hypertrophied and dilated hearts. Electron microscopic examination revealed marked disarrangement of bundles of myofilaments and widened Z-bands in the hypertrophied myocardium. Blood pressure was not elevated in the rats with cardiac hypertrophy. These findings show that a disease of the myocardium with the pathologic features similar to those of hypertrophic cardiomyopathy in man occurs spontaneously in rats.     

Right ventricular dysplasia: a clinical and pathological study of two families with left ventricular involvement.

BACKGROUND--Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle. AIM--To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy. METHODS AND RESULTS--Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients. CONCLUSIONS--Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy.     

Angiographic findings in arrhythmogenic dysplasia of the right ventricle

Arrhythmogenic right ventricular dysplasia is characterized by fibrous and adipose replacement of the right ventricular myocardium and recurrent ventricular arrhythmias of left bundle branch block morphologic pattern. Sometimes the diagnosis is difficult because not all the clinical and instrumental findings are present and the separation between arrhythmogenic right ventricular dysplasia and other right ventricular cardiopathies is uncertain. In such cases the angiographic appearance of the right ventricle has been considered the "gold standard". To assess the diagnostic value of right ventricular morphology in identifying arrhythmogenic right ventricular dysplasia, we compared the angiographic findings of 8 patients with arrhythmogenic right ventricular dysplasia, 10 with biventricular dilated cardiomyopathy and 10 with Ebstein's anomaly. The following aspects were considered: deep fissuring of the anterior or inferior wall, outflow tract enlargement, contrast persistence in the right ventricle during the levophase, regional wall motion abnormalities including aneurysmal formations and tricuspid regurgitation. Aneurysmal formations of the right ventricle were found only in arrhythmogenic right ventricular dysplasia whereas the other angiographic findings were common to all the above mentioned diseases. Right ventricular angiography is an important adjunct to the clinical and instrumental diagnosis of arrhythmogenic right ventricular dysplasia, but most of its angiographic features are common to other diseases which cause right ventricular dilatation.     

左心室心肌脂肪替代的临床特征及意义

目的 回顾性分析左心室心肌发生脂肪替代患者的临床特征及其意义.方法 心血管CT筛查示左心室心肌脂肪替代患者45例(男28例,女17例)纳入本研究,平均年龄(51.9±14.7)岁.以左心室心肌CT值≤-30 Hu为左心室心肌脂肪替代标准.分析左心室心肌脂肪替代患者左心室受累节段、受累程度等.结果 45例患者中冠心病患者25例(男20例,女5例),平均年龄(61.2±10.4)岁.非冠心病患者20例(男8例,女12例),平均年龄(57.8±13.3)岁.冠心病患者单支病变占56%,双支病变占20%,三支病变占24%,合并室壁瘤形成者3例,合并左心室附壁血栓者1例,平均左心室横径(54.5±9.4)mm,平均左心室射血分数(51.8±13)%.非冠心病组患者扩张型心肌病患者3例,房间隔缺损1例,风湿性心脏病1例,其余15例患者心内结构未见异常,平均左心室横径(51.1±9.1)mm,平均左心室射血分数(59.4±13.9)%.冠心病患者脂肪替代均位于左心室梗死区,表现为心内膜下条带状或线状低密度区,其中伴左心室室壁变薄(＜5 mm)患者21例,18例患者脂肪替代累及左心室心尖部,其次为室间隔远段(15例)和左心室前壁远段(11例),室间隔中段和前壁中段患者各7例,左心室基底段受累患者仅1例.非冠心病患者脂肪替代主要累及室间隔,表现为条带状脂肪替代17例,小片状替代3例,其中室间隔中段受累11例,室间隔远段10例,心尖部9例,脂肪替代累及左心室壁间患者14例,累及心内膜下患者10例,其中4例患者左心室壁间与心内膜下均受累.结论 冠心病与非冠心病患者左心室心肌脂肪替代在受累部位、形态等方面不同,了解这些差异对判断左心室心肌脂肪替代的病因有重要意义,非冠心病患者左心室脂肪替代的临床意义有待于进一步研究.

Abstract：

Objective To evaluate the clinical characteristics of left ventricular fat replacement. Methods We identified 45 patients [28M/17F, mean age (51.9 ± 14. 7 )years] with left ventricular myocardial fat replacement ( CT value ≤ - 30 Hu) by cardiovascular CT. Results Among 45 patients, 25 patients[20M/5F, mean age (61.2 ± 10. 4) years] were diagnosed as coronary artery disease (CAD). There was 56% single-vessel disease, 20% double-vessel disease and 24% triple-vessel disease,true left ventricular aneurysm was detected in 3 patients and left ventricular thrombi in 1 patient, the dimension of left ventricle was (54. 5 ±9. 4) mm and the LVEF was (51.8 ± 13 ) % in CAD group. In this group, fat replacement occurred in the region of myocardial infarction and presented as curvilinear band in subendocardial region. The left ventricular wall thickness was lower than 5 mm in 21 cases. The location of fat replacement in CAD group is as follows: apical region in 18 patients, distal septal in 15 patients, distal anterior in 11 patients, mid-septal in 7 patients, mid-anterior in 7 patients and basal in 1 patients. The age of remaining 20 patients (8M/12F) without CAD were (57. 8 ± 13.3) years. In the group of non-CAD,dilated cardiomyopathy was diagnosed in 3 patients, atrial septal defect in 1 patient, rheumatic heart disease in 1 patient, there was no structural heart disease in the remaining 15 patients. The dimension of left ventricle was (51.1 ± 9. 1 ) mm and the LVEF was (59. 4 ± 13.9 )%. In non-CAD group, fat replacement mainly occurred in septal region, presented as curvilinear band in 17 patients and patch in 3 patients. The location of fat replacement in this group is as follows: mid-septal region in 11 patients, distal-septal in 10 patients and apical in 9 patients. The intramural fat replacement was detected in 14 patients: subendocardial fat replacement in 10 patients and both intramural and subendocardial fat replacement in 4 patients. Conclusions Left ventricular fat replacement could be documented in CAD patients, non-CAD cardiomypathy patients and in patients without structural heart disease. Left ventricular fat replacement often positioned in apical region in CAD patients as a consequence of infarct healing while mostly positioned in septal region in non-CAD patients, the definite clinical implication of left ventricular fat replacement in nonCAD patients remains to be clarified.     

Value of the isoprenaline test in arrhythmogenic heart diseases

The sympathetic nervous system seems to play a major role in the genesis of ventricular arrhythmias. The authors studied this adrenergic factor prospectively by exercise stress testing and intravenous isoprenaline in 107 patients referred for evaluation of arrhythmias or symptoms thought to be due to arrhythmias: 30 patients had morphologically normal hearts (15 ventricular extrasystoles, 15 bursts of ventricular tachycardia); 55 patients had dilated cardiomyopathy and 22 had probable or proven arrhythmogenic right ventricular dysplasia. Exercise testing was carried out with 30 watt increments every 3 minutes. Ventricular tachycardia was induced in 6 patients with apparently normal hearts (17%), 13 patients with dilated cardiomyopathy (31%) and 7 patients with arrhythmogenic right ventricular dysplasia (40%). Isoprenaline was infused for 3 minutes at a dose of 8-12 g/min: ventricular tachycardia was induced in 7 patients with apparently normal hearts (24%) and 23 patients with dilated cardiomyopathy. In some patients presenting with syncope, an arrhythmogenic response to isoprenaline was the only abnormality detected by the study protocol. An arrhythmia was induced by isoprenaline in 17 of the 18 patients with confirmed right ventricular dysplasia (94%), 12 of whom had sustained mono or polymorphic ventricular tachycardia. Two of these patients did not have significant right ventricular wall motion abnormalities. Four asymptomatic subjects related to patients with right ventricular dysplasia underwent the isoprenaline test; bursts of ventricular tachycardia were recorded in 3 of them. Polymorphic ventricular tachycardia was specifically associated with cardiac disease. The maximum heart rate attained by exercise testing (148 +/- 19/min) was higher than that attained with isoprenaline (148 +/- 22/min).(ABSTRACT TRUNCATED AT 250 WORDS)