慢性肺心病患者血浆脑钠素前体水平及其意义

目的 :选择慢性阻塞性肺疾患和慢性肺心病病人 ,探讨脑钠素前体 (proBNP1 -76)血浆水平与慢性肺心病之间的关系及其意义。方法 :放射免疫分析检测血浆 proBNP1 -76含量 (ng/L)。 结果 :慢性阻塞性肺疾患组的 proBNP1 -76浓度较正常对照组升高 (p <0 0 5 ) ,右下肺动脉横径、右室流出道内径较正常对照组显著增大 (p <0 0 0 1 ) ,右室前壁厚度与proBNP1 -76浓度存在正相关 (r =0 5 4 1 ,p <0 0 1 ) ;慢性肺心病组的proB NP1 -76浓度、右下肺动脉横径、右室流出道内径、右室前壁厚度较正常对照组和慢性阻塞性肺疾患组均显著升高 (p <0 0 0 1 ) ,右室流出道内径与 proBNP1 -76浓度水平存在正相关 (r =0 4 77,p <0 0 5 )。 结论 :测定血浆proBNP1 -76水平对于监测慢性肺心病的发生可以起到一个早期标志物的作用 ,对于临床早期诊断和预防慢性肺心病提供一个有意义的参考指标     

Histometric research on cor pulmonale in chronic nonspecific lung diseases

Micrometric parameters of lungs and the right ventricle myocardium under the pulmonary heart compensation and decompensation were studied. Correlative and information analysis of parameters at various stages of the pulmonary heart formation in chronic non-specific pulmonary diseases in 14 patients dying at the age of 48 to 80 years is performed. Morphometric analysis allowed to distinguish micrometric signs of the pulmonary heart compensation and decompensation. Information analysis of the pulmonary heart parameters established the thickness of cardiomyocytes and transversal diameter of their nuclei to be the most informative indexes. Correlation analysis revealed the enhancement of the parameters links, in proportion with the development of the pulmonary heart decompensation. The changes observed reflect a structural-functional myocardium restructuring and indicate an energy deficiency of the right ventricle cardiomyocytes.     

Prognostic value of cardiodynamic indexes with respect to the development of heart failure during massive pulmonary embolism

The cardiodynamics of the right ventricle was studied during the first 30 min of massive pulmonary embolism complicated and uncomplicated by heart failure. Both variants were accompanied by diastolic dysfunction of the right ventricle. Some changes in the cardiodynamics observed during complicated massive pulmonary embolism indicated a lower increase in the contractile activity, decreased myocardial contractility, and more pronounced dilation of the right ventricle. The absolute indexes of mechanical activity including indexes of contractility are not reliable criteria for early diagnostics of heart failure in the acute stage of massive pulmonary embolism, whereas changes in these indexes hold much prognostic value. Translated fromByulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 128, No. 12, pp. 634–637, December, 1999     

Chronic right ventricular pressure overload results in a hyperplastic rather than a hypertrophic myocardial response

Myocardial hyperplasia is generally considered to occur only during fetal development. However, recent evidence suggests that this type of response may also be triggered by cardiac overload after birth. In congenital heart disease, loading conditions are frequently abnormal, thereby affecting ventricular function. We hypothesized that chronic right ventricular pressure overload imposed on neonatal hearts initiates a hyperplastic response in the right ventricular myocardium. To test this, young lambs (aged 2-3 weeks) underwent adjustable pulmonary artery banding to obtain peak right ventricular pressures equal to left ventricular pressures for 8 weeks. Transmural cardiac tissue samples from the right and left ventricles of five banded and five age-matched control animals were studied. We found that chronic right ventricular pressure overload resulted in a twofold increase in right-to-left ventricle wall thickness ratio. Morphometric right ventricular myocardial tissue analysis revealed no changes in tissue composition between the two groups; nor were right ventricular myocyte dimensions, relative number of binucleated myocytes, or myocardial DNA concentration significantly different from control values. In chronic pressure overloaded right ventricular myocardium, significantly (P < 0.01) more myocyte nuclei were positive for the proliferation marker proliferating cellular nuclear antigen than in control right ventricular myocardium. Chronic right ventricular pressure overload applied in neonatal sheep hearts results in a significant increase in right ventricular free wall thickness which is primarily the result of a hyperplastic myocardial response.     

The left ventricle in anomalous pulmonary venous return. Morphometric analysis of 36 fatal cases in infancy.

The mass of the left ventricle is normal in infants with total anomalous pulmonary venous return, but the ventricular cavity is compromised by leftward displacement of the interventricular septum due to the combined pressure-volume overload of the right side of the heart. Severe septal displacement is associated with distorted myocardial architecture in the region of the septal attachments of the left ventricular free wall. Abnormalities of septal motion are frequently detected in echocardiograms. Left ventricular output is low, as judged by arrested development of the aortic valve circumference, and is probably compromised both by the left to right shunt and by septal displacement. Successful correction of the anomaly may depend on intervention before the changes in septal position, structure, and function have become irreversible.     

Changes in the lesser circulation vessels in chronic nonspecific lung diseases

Clinicomorphological study of the pulmonary circulation vessels was performed in 20 patients with bronchiectatic disease without right ventricle hypertrophy and in 14 patients with chronic bronchitis complicated with pulmonary heart. Pathology data of persons dying from diseases other than vitium cordis and lung diseases were used as control. Morphological signs of pulmonary hypertension are found in all cases of chronic nonspecific lung diseases. The most pronounced changes develop in the vascular segment starting from arterioles and up to the arteries of muscle type with the diameter of 400 microns. The dependence of the pulmonary arteries changes on the type of lung disease is established. Pathological changes in the walls of the pulmonary circulation vessels, in parallel with pronounced compensatory alterations, are found in cases complicated with pulmonary heart.     

Right ventricular infarction associated with anteroseptal myocardial infarction: a clinicopathologic study of nine cases.

The association of right ventricular (RV) infarction with inferoseptal myocardial infarction is well established. However, a question remains about the occurrence of RV infarction in association with anteroseptal myocardial infarction. To determine the frequency and clinical correlates of this entity, we studied autopsied hearts from patients with isolated anteroseptal left ventricular (LV) infarcts. Among 3,249 autopsy specimens, 88 cases were identified. From each, sections were taken from the RV anterior, lateral, and inferior regions at basal, middle, and apical levels. All 1,584 slides were reviewed by blinded assessment. RV and LV infarcts were compared to confirm similarity in age. Patient records and cardiac investigations were reviewed for evidence of RV involvement. Of the 88 hearts with anteroseptal LV infarcts, 9 (10%) had coexistent RV infarction (6, old; 3, new). For these 9, the RV infarction involved 11% to 33% of the RV area, and the left anterior descending coronary artery was the infarct-related artery in each. All 3 patients who had an echocardiographic examination within 4 weeks of anteroseptal LV infarction had RV dysfunction. One patient, studied 15 years after infarction, had a normal right ventricle by echocardiography. In 3 patients with acute myocardial infarction, right heart catheterization during the acute phase revealed increased right-sided diastolic pressures out of proportion to left-sided diastolic pressures (right atrial pressure to pulmonary capillary wedge pressure, 60% to 95%). In conclusion, 10% of patients with an isolated anteroseptal LV infarct had evidence of RV free wall infarction. The RV infarction was associated with identifiable hemodynamic and echocardiographic features.     

Isolated right ventricular infarction with massive dilation: acquired Uhl's disease?

A patient had extensive isolated right ventricular infarction, which as the result of proximal occlusion of a small right coronary artery in a dominant left circumflex coronary arterial system, with minimal left-sided disease. A healed right ventricular infarction and Uhl's disease may have similar clinical and morphologic features. The sequence of events in our patient raises the question of whether coronary artery occlusion and myocardial infarction may be associated with a parchmentlike right ventricle. Damage to the right ventricular myocardium with subsequent superimposed chamber pressures and resulting dilation may lead to morphologic features similar to the parchment heart of Uhl's anomaly.     

Arterial blood gases,muscle fiber diameter and intercapillary distance in cardiac hypertrophy of rats with an old myocardial infarction

In rats with a myocardial infarction due to ligation of the left coronary artery a marked right ventricular hypertrophy developed after 41/2 weeks. At this time no difference against control animals was observed in arterial , , pH, ideal alveolar , and alveolar-arterial O₂ pressure difference, as measured in unanesthetized animals at normoxia. In histological sections of the heart stained by PAS reaction capillaries and muscle fibers were counted, and the mean intercapillary distance and muscle fiber diameter were estimated. In the right ventricle of the rats with myocardial infarction both increased when compared with control animals or with sham-operated rats. Fibercapillary ratio was the same in all three groups. Similar results were obtained in the remaining undamaged tissue of the left ventricle of rats with a myocardial infarction when compared with the left ventricle of control or sham-operated rats. Findings concerning intercapillary distance suggest that also in the myocardium which remains intact during the development of the infarction and later hypertrophies, tissue oxygen transport might be impaired, particularly during a stress situation. Results in the right ventricle of rats with myocardial infarction show an opposite trend against rats exposed chronically to simulated high altitude, where in the hypertrophied right ventricle a shorter intercapillary distance occurs and therefore an improvement of tissue oxygen transport might be expected.Dedicated to Professor Dr. Adolf Faller, University of Fribourg, Switzerland, on his 65th birthdayPresented in part at the IXth World Conference of the European Society for Microcirculation, Antwerp, July 5–9, 1976     

Expression of Id2 in the second heart field and cardiac defects in Id2 knock-out mice

The inhibitor of differentiation Id2 is expressed in mesoderm of the second heart field, which contributes myocardial and mesenchymal cells to the primary heart tube. The role of Id2 in cardiac development is insufficiently known. Heart development was studied in sequential developmental stages in Id2 wildtype and knockout mouse embryos. Expression patterns of Id2, MLC-2a, Nkx2.5, HCN4, and WT-1 were analyzed. Id2 is expressed in myocardial progenitor cells at the inflow and outflow tract, in the endocardial and epicardial lineage, and in neural crest cells. Id2 knockout embryos show severe cardiac defects including abnormal orientation of systemic and pulmonary drainage, abnormal myocardialization of systemic and pulmonary veins, hypoplasia of the sinoatrial node, large interatrial communications, ventricular septal defects, double outlet right ventricle, and myocardial hypoplasia. Our results indicate a role for Id2 in the second heart field contribution at both the arterial and the venous poles of the heart.     

肺心病左室舒张功能障碍发病机制的研究进展

近年来肺心病的发病率逐年上升,以往认为肺心病对心脏的影响主要以右心功能障碍为主,不影响左心功能,左心功能障碍主要为原发左心病变所致,但近年来不少文献指出由慢阻肺长期发展引起的肺心病在早期即可合并左心功能障碍,主要以左室舒张功能障碍为主。本文主要从五个不同方面阐述由慢阻肺长期发展引起的肺心病患者左室舒张功能障碍的原因,结合相关文献做一综述。     

Pathomorphology of myocardial circulation: Comparative study in increased left or right ventricle afterload

Comparative study of pathomorphology of myocardial circulation under conditions of increased afterload of the left or right ventricles showed similar changes. All compartments of the coronary bed were plethoric, capillary blood stasis and perivascular edema, more pronounced in arterial vessels, were detected in both cases. These changes equally involved both ventricles and the ventricular septum. Significant differences consisted in local increase in the density of functioning capillaries. The increase was the maximum in hemodynamically overloaded ventricle and ventricular septum, presumably due to increase of their contractile activity. The density of functioning capillaries in the intact (vs. pressure overloaded) ventricle also increased, but to a lesser degree, which could be due to systemic neurohumoral effects. If increased afterload was complicated by the development of heart failure, circulatory disorders in the myocardium progressed. Significant increase in the density of functioning capillaries in all cardiac compartments indicated decreased vascular tone and exhaustion of coronary reserve. This was paralleled by a sharp arterial plethora in case of increased afterload of the left ventricle and sharp blood stasis in the microcirculatory bed in case of increased right ventricle afterload. Reduction of effective perfusion pressure in the presence of coronary dystonia can cause coronary insufficiency and myocardial ischemia in case of increased right ventricle afterload. __________ Translated from Byulleten’ Eksperimental’noi Biologii i Meditsiny, Vol. 145, No. 3, pp. 352–356, March, 2008     

Premortem histologic evidence of chronic vascular rejection obtained from percutaneous endomyocardial biopsy: report of a case

Chronic rejection is characterized by obliterative arteritis of coronary arteries and their branches in the form of myointimal proliferation and diffuse tubular atherosclerosis. Chronic rejection is more difficult to detect than discrete focal obstructive lesions by coronary angiography. We report a case of a 51-year-old woman in whom a biopsy of the right ventricle 14 months after heart transplantation revealed convincing histologic evidence of chronic rejection. A subsequent biopsy of the left ventricle showed subendocardial infarct. Necropsy findings confirmed coronary artery changes of chronic rejection, as well as diffuse subendocardial infarction, which had been suspected clinically. The finding of a small arteriole in a biopsy was fortuitous. However, if such a vessel is present and shows obliterative arteritis, this demonstrates that a premortem histologic diagnosis of chronic rejection is possible.     

Pulmonary atresia with intact ventricular septum and right ventricular dependent coronary circulation through the “vessels of Wearn”

We present an autopsy case of a male baby born at 35 weeks of gestation with pulmonary atresia with intact ventricular septum (PAIVS), who had coronary blood flow that was dependent on outflow from the right ventricle through the vessels described by Wearn. At 7 weeks of age, he underwent single ventricle palliation consisting of ligation of a patent ductus arteriosus and placement of a modified Blalock-Taussig shunt. The patient experienced a perioperative myocardial infarction, requiring extracorporeal membrane oxygenation. Progressive hemodynamic decline resulted in death 8 days after surgery. Autopsy revealed acute and remote infarctions in both ventricles and fibromuscular dysplasia of the subepicardial and intramural coronary arteries. In 1926, Grant first reported the association between PAIVS and secondary dysplasia of the heart vasculature and hypothesized that the high pressure resulted in dilation of the myocardial sinusoids. Confusion secondary to the unmeritorious dismissal of the myocardial sinusoids has obscured the pathogenesis of PAIVS and led to several publications suggesting second heart field abnormalities as a disease model for PAIVS. We discuss the pathogenesis of PAIVS, the ventriculocoronary arterial connections and the sinusoidal relationship to the vessels described by Wearn, and we attempt to correct the solecism plaguing the nomenclature of myocardial vasculature.     

Neuroendocrine activation and diagnostics in pulmonary embolism: Translational studies

Acute pulmonary embolism (PE) is a severe and potentially fatal disease which acutely augments the right ventricle (RV) strain. Development of RV dysfunction (RVD) in the disease process is synonymous with an overall poor prognosis. The diagnosis of PE is usually established by a combination of clinical assessment, D-dimer test and medical imaging with either lung scintigraphy or pulmonary multidetector computer tomography (MDCT) angiography. Which of the two methods to use in PE diagnostic has not been determined and very limited data comparing these modalities are available. Assessment of RV function is cumbersome due to complex geometry. RVD is usually established by echocardiography which is observer dependent, has low reproducibility, and requires expertise. Therefore, a simple and reproducible biochemical method to assess RVD in patients with PE would be desirable. Brain natriuretic peptide (BNP), pro-atrial natriuretic peptide (pro-ANP), cardiac troponin I (TnI), and endothelin-1 (ET-1) have been the most studied plasma biomarkers in the context of risk stratification in PE. BNP is mainly produced in the ventricles of the heart. It is released from the left ventricle in response to increased filling pressure and is increased in chronic left heart failure. Pro-ANP is primarily produced in the atria, is released by atrial distention and is elevated in chronic pulmonary hypertension and could be an early marker for RVD. Plasma level of ET-1 has been shown to correlate with pulmonary pressure and is released from endothelial cells in the pulmonary vessels. Additionally, increases in circulating levels of ET-1 have been reported in an experimental animal model of PE. TnI is part of a complex of regulatory proteins in the cardiac myofilaments and is released upon myocyte injury. It is related to short term clinical outcome, prolonged hypotension, and cardiogenic shock after myocardial infarction and is a predictor of 30-day mortality and RVD using echocardiography in patients with PE. Our hypothesis was therefore that the neuroendocrine activation of BNP, pro-ANP, ET-1, and TnI alone or in combination could serve as markers of RVD in patients with PE. The use of plasma biomarkers would be much simpler than reproducible medical imaging methods such as magnetic resonance imaging (MRI), radionuclide based methods etc.     

IDIOPATHIC RIGHT VENTRICULAR DILATION

Two autopsy cases which showed marked depletion of the right ventricular musculature of the heart accompanied with marked infiltration of the adipose tissue were reported. The first cases was an 18-year-old female who died of right sided congestive heart failure after about 4-years clinical course. The autopsy disclosed marked dilation of the right atrium and ventricle. The entire free wall of the right ventricle was markedly thin. Microscopically, most of the myocardial fibers of the right ventricle were replaced by fat and fibrous tissue. The second case, a 15-year-old boy, whose identical twin was previously diagnosed as arrhythmogenic right ventricular dysplasia designated by F ontaine et al. , died suddenly during exercise. He showed no cardiac symptoms but electrocardiogram was abnormal. Autopsy revealed majority of the myocardial fibers of the right ventricular free wall were replaced by fatty tissue. In both cases, fatty infiltration was mainly noticed at the epicardial side and some myocardial fibers remained in the fatty tissue showed hypertrophic and/or degenerative changes. Review of the literature on similar cases showing depletion of the right ventricular musculature including so-called adult's Uhl anomaly, ARVD and dilated right ventricular myocardiopathy was conducted and the relationship of the present cases with these lesions was discussed.     

Idiopathic right ventricular dilation. Special reference to "arrhythmogenic right ventricular dysplasia" and analogous lesions

Two autopsy cases which showed marked depletion of the right ventricular musculature of the heart accompanied with marked infiltration of the adipose tissue were reported. The first cases was an 18-year-old female who died of right sided congestive heart failure after about 4-years clinical course. The autopsy disclosed marked dilation of the right atrium and ventricle. The entire free wall of the right ventricle was markedly thin. Microscopically, most of the myocardial fibers of the right ventricle were replaced by fat and fibrous tissue. The second case, a 15-year-old boy, whose identical twin was previously diagnosed as arrhythmogenic right ventricular dysplasia designated by Fontaine et al., died suddenly during exercise. He showed no cardiac symptoms but electrocardiogram was abnormal. Autopsy revealed majority of the myocardial fibers of the right ventricular free wall were replaced by fatty tissue. In both cases, fatty infiltration was mainly noticed at the epicardial side and some myocardial fibers remained in the fatty tissue showed hypertrophic and/or degenerative changes. Review of the literature on similar cases showing depletion of the right ventricular musculature including so-called adult's Uhl anomaly, ARVD and dilated right ventricular myocardiopathy was conducted and the relationship of the present cases with these lesions was discussed.     

Double outlet right ventricle in a calf

Double outlet right ventricle is a conotruncal malformation where both great arteries (aorta and pulmonary trunk) arise from the right ventricle. A 2-month-old Holstein calf was slaughtered due to severe respiratory distress. At necropsy, the heart was enlarged, globose, and had ventricular and atrial septal defects. The only outlet for the left ventricle was a large ventricular septal defect located at 6 cm distance from the heart apex and involved atrial septum too. The right ventricle was enlarged and markedly thickened with a left to right free wall ratio of 2.5:2 and prominent papillary muscles. The aorta arose from the right ventricular infundibulum adjacent to pulmonary trunk. Two valvular hematomas were observed on the edge of the right atrioventricular valve. The lungs were rubbery with ecchymotic and petechial hemorrhages and did not collapse after removing from thoracic cavity. Enhanced lobular pattern was evident on both the capsular and cut surfaces of the liver. Histopathological examination of the lungs revealed thickening of alveolar septa, hemorrhages, and infiltration of hemosiderophages within alveoli. Periportal hepatocellular fatty changes, substitution of centrilobular and midzonal hepatocytes by red blood cells, and dilation of midzonal and periportal sinusoids were seen in the liver. To our knowledge, this particular combination of cardiac defects has not been previously described in domestic animals and the pathological lesions observed in the calf may be resulted as a sequel to left–right blood shunting and heart failure.     

Pathomorphology of Postinfarction Myocardial Ischemia during Atherosclerotic Obstruction of Coronary Arteries

Pathomorphological criteria of early postinfarction angina included segmentary atherosclerotic obstruction of the upper segments of the coronary artery supplying the infarction area, recurrent acute myocardial infarction, maximum decrease in vascularization of the left posterior ventricular wall; and individual changes in angioarchitectonics of the heart promoting hibernation of the myocardium. Pathognomonic morphological criteria of silent postinfarction myocardial ischemia included diffuse extensive atherosclerotic obstruction of lower segments in the coronary artery supplying the infarction area and total hypervascularization of the myocardium, first acute myocardial infarction of the left ventricular anterior wall, and maximum decrease in vascularization of the anterior and posterior wall in the left ventricle. These coronary-myocardial relationships contribute to stunning of the myocardium. Zones of hypokinesia and akinesia were revealed in the left ventricle, which reflects the phenomenon of resting myocardium associated with isolation of heart angioarchitectonics.__________Translated from Byulleten’ Eksperimental’noi Biologii i Meditsiny, Vol. 139, No. 3, pp. 346–351, March, 2005     

致心律失常性右室心肌病心力衰竭期的病理特点分析

目的 通过分析致心律失常性右室心肌病(ARVC)心力衰竭期的病理改变,以进一步了解其临床分期与病理表型的关系.方法 从2004-2007 年在阜外心血管病医院接受心脏移植的心力衰竭病例中,收集病理诊断为ARVC的受体心脏8例,测量心脏重量,评价左右心室心腔扩张、心肌细胞肥大、脂肪浸润、纤维化、附壁血栓和伴发心肌炎等指标,注意左心室受累情况,并进行病理分型.结果 8例中的7例为经典型(即右心室改变为主),1例为左优势型(左心室改变为主),未见双室型病例.组织学均为纤维脂肪型,未见单纯脂肪型病例.经典型病例的右心室中、重度扩张,少数有室壁瘤形成,其中6例伴左心室受累,受累左心室轻、中度扩张,心肌广泛间质纤维化,部分病例伴替代性疤痕,而脂肪浸润量小,多局限于心外膜下.左心室心肌细胞肥大普遍.而左优势型的左心室重度扩张,弥漫间质纤维化和局部透壁性脂肪浸润.8例中3例左心室明显肥厚,3例查见双室附壁血栓,1例伴局灶性心肌炎.结论 ARVC心力衰竭期的左心室受累多见而严重,左心室间质纤维化突出,心肌细胞肥大明显,但脂肪替代少见和局限.左、右心室多扩张,可见附壁血栓,应注意与扩张型心肌病等鉴别.