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[Primary endocardial fibroelastosis].

Endocardial fibroelastosis is a disease characterized by a prominent proliferation of dense collagenous and elastic tissue in the endocardium with the occurrence of congestive heart failure and death in early life. There are, however, many recorded instances of infants surviving the initial illness and living up to or beyond puberty. The clinicopathological factors for good prognosis include the increase in myocardial mass of ventricle evaluated by ventriculography and the histopathologic evidence for compensatory myocardial cell hypertrophy in the biopsied myocardium.

Child↗

Myocardial fiber diameter as a good indicator of outcome in Batista's operation.

BACKGROUND: Despite the initial promissory results of partial left ventriculectomy, or Batista's operation, the postoperative mortality associated with the procedure has been too high. We described a histopathologic study performed to identify histological parameters that could help to determine outcomes of patients undergoing this procedure. METHODS AND RESULTS: Myocardial fiber diameter, myocardial fibrosis, thickness of the compact wall, and number of cells presenting from the endocardium to epicardium were analyzed in 32 patients with idiopathic dilated cardiomyopathy who underwent Batista's operation. Data were grouped by patients who died < or = 6 months and patients who survived for > 6 months after the surgical procedure. Additional analyses were performed to compare results according the causes of death and to test the application of these results to biopsy. RESULTS: Myocardial fiber diameter was the only index that could distinguish the two groups. Myocardial fiber diameter < 22 microm distinguished the group of patients who survived the 6-month postoperative period from patients who died during that time with sensitivity of 85.7 and specificity of 72.2. The subendocardial region of the compact wall and the trabecular portion of the wall exhibited comparable results. CONCLUSION: Our results indicate that the myocardial fiber diameter of samples from the trabecular or subendocardial compact wall regions may help predict the outcome of left ventriculectomy. Samples from the trabecular or subendocardial compact wall regions were used for analysis. Further prospective studies involving left ventricular endomyocardial biopsies are necessary to confirm if the use of myocardial fiber diameter in the selection of patients for surgery improves the index of success of Batista's operation. Other factors that are involved remain unclear.

Biopsy↗

Early endocardial formation originates from precardiac mesoderm as revealed by QH-1 antibody staining.

The formation of endocardial endothelium in quail embryos was investigated using in vivo and in vitro systems. At stage 7+ (2 somite), the initial emergence of endothelial cells within the bilateral heart forming region (HFR) was detected in quail embryos by immunohistochemistry with QH-1 (an anti-quail endothelial cell marker) and confocal microscopy. We consistently observed more QH-1 positive cells in the right HFR than the left. At stage 8 (4 somite), the HFR, including QH-1 positive cells, were located in the splanchnic mesoderm after formation of the coelom. During stage 8, the HFR migrated along the margin of anterior intestinal portal in association with the endoderm. By stage 8+ (5 somite), the two HFR had fused at the midline and formed a plexus of QH-1 positive endothelial precursor cells. The definitive endocardium developed as a single, hollow, tube within this plexus. Posteriorly, QH-1 positive cells of the HFR established vascular-like connections with QH-1 positive cells that had formed outside (peripheral to) the HFR. During migration and subsequent determination, the precardiac mesoderm is continuously associated with the basement membrane of the anterior endoderm. To determine the role of endoderm on endocardial endothelial cell formation and development, precardiac mesoderm from stage 5 embryos, which does not express QH-1 antigen, was explanted onto the surface of collagen gels. When co-cultured with endoderm, the outgrowth of free cells from the mesoderm was much more extensive, many of which invaded the gel and expressed the QH-1 antigen; mesoderm cultured without endoderm did not seed nor express QH-1 antigen. These findings suggest that the segregation of endothelial and myocardial lineages may occur by an endoderm-mediated, mesenchymal formation.

Animals↗

[Myocardial sarcoidosis--pathological studies on 7 autopsy cases with particular reference to histological variations].

The present report consists of seven autopsy cases of myocardial sarcoidosis terminating in death 1 month to 5 years after the onset of symptoms. Histologically, they were classified into the following 4 types: a) diffuse nonspecific granulation, b) specific granuloma with giant cells, c) diffuse fibrosis with giant cells, and d) nonspecific fibrosis. Types a) and b) demonstrate active changes, while c) and d) are sequelae of active inflammation. Morphological changes of sarcoidosis were found not only in myocardium but also in both pericardium and endocardium. Although no final conclusions could be obtained as to whether the giant cells appearing in myocardium were of mesenchymal or myogenic origin, the authors favor the latter concept. Changes compatible to sarcoidosis found in other organs in acute cases were scarce and old. On the contrary, more active and variable changes were encountered in protracted cases. The authors consider giant cell myocarditis to be one type of sarcoidosis occurring in the heart.

Adult↗

Elastic tissue in pseudoxanthoma elasticum. Ultrastructural study of endocardial lesions.

A 56-year-old woman had pseudoxanthoma elasticum (PXE). Ultrastructural findings in the endocardium showed increased collagenogenesis and elastogenesis. The newly formed elastic fibers appeared morphologically normal, but showed a notable propensity to become calcified. The internal structure of the calcified areas was essentially similar to that of the adjacent uninvolved portions of the elastic fibers. Some of the heavily calcified fibers showed loss of internal structure and fragmentation. These findings support the concept that in PXE, calcification involves morphologically unaltered elastic fibers that subsequently may undergo secondary degenerative changes, such as loss of internal structure and fragmentation.

Calcinosis↗

Lipid droplets in the heart interstitium: concentration and distribution.

A morphological study of the sudanophilic lipids in dog and human hearts was carried out. The hearts of 5 mongrel dogs and 20 auricular appendices of human patients were utilized. The dog hearts were fixed in situ by perfusion with 2% glutaraldehyde, and the human tissue was fixed by immersion. For the ultrastructural study, some blocks of every portion of the specimen were postfixed in osmium and embedded in Epon. The topographical and quantitative study was done on frozen 20-mu sections of tissue stained with Sudan IV in propylene glycol and counterstained with hematein (Geigy). The results indicate that a significant quantity of visible triglycerides is extracellular and located in the interstitial spaces of the heart. The dimensions of these lipid droplets vary from 0.5 mu to more than 20 mu. The highest concentration was found in the epicardial region of the left ventricle, but a significant amount was found in the endocardium and in the middle portion of the myocardium. These observations indicate that a sizable portion of the heart interstitium is occupied by free droplets of triglycerides. This substrate pool is probably mobile and readily available as a source of energy.

Adipose Tissue↗

[Complications of cardiac pacemakers].

Since the number of patients with pacemakers increases and the further decentralisation of the care of these patients is planned the survey has the purpose to inform as many physicians as possible about the complications of the pacemaker therapy. In the several paragraphs the problems of the regular and anticipated exhaustion of the battery, defects of the electronic constituents of the pacemaker, fracture of the cable, dislocation of the electrodes, disturbances of the transmission between endocardium and myocardium, disturbances of rhythm under pacemaker-therapy, disturbing influences by intra- and extracorporal causes, surgical complications and implantations in the false place are reported on. Knowledge and early recognition of the complications in the pacemaker-therapy will furthermore favourably influence the long-term prognosis of this group of patients.

Cardiac Surgical Procedures↗

Myocardial and endocardial lesions in rabbit after acute poisoning with Intration and after treatment with some detoxifying agents.

The hearts of rabbits killed 24 hours after intoxication with Intration in the dose of LD50 and rabbits treated at the same time with PAM or atropine were studied pathomorphologically. Twenty-four hours after administration of Intration toxic lesions of the myocardium and endocardium were observed, which were more pronounced in rabbits treated simultaneously with the detoxifying agents as compared with those which received only Intration. The intensity of the changes was due to overdosage of the detoxifying agents, especially PAM which was particularly harmful.

Animals↗

[Early afterdepolarization and cesium chloride induced ventricular arrhythmias].

This study is to demonstrate the role of early afterdepolarization (EAD) in the cesium chloride induced long QT interval and ventricular arrhythmias by recording monophasic action potential (MAP) of right ventricular endocardium with contact electrode in 12 closed chest dogs. The results suggested that EAD could produce triggered premature ventricular beats and ventricular tachycardias (VT) in situ. The first ectopic ventricular beat of EAD induced VT usually appears close to the peak of the U wave. Short-long-short interval sequence preceding episodes of VT occurred in 89.6% of VT.

Action Potentials↗

Enhanced expression of the tie receptor tyrosine kinase in endothelial cells during neovascularization.

We have recently cloned a novel human receptor tyrosine kinase, tie, from human leukemia cells showing megakaryoblastoid differentiation. We report here that the 4.4-kb tie messenger RNA (mRNA) is present in all human fetal and mouse embryonic tissues. By in situ hybridization, the tie mRNA was localized to the endothelia of blood vessels and endocardium of 9.5- to 18.5-day mouse embryos. However, tie was not expressed by endothelial cells of developing hepatic sinusoids. Increased tie mRNA signal was seen in proliferating ovarial capillaries during hormone-induced superovulation. Only a weak tie signal was obtained from adult skin, except during wound healing, when the proliferating capillaries in the granulation tissue contained abundant tie RNA. These results suggest that tie may have a role in neovascularization.

Amino Acid Sequence↗

LONG-TERM ELECTRICAL STIMULATION OF THE HEART IN STOKES-ADAMS DISEASE.

Ten patients, between the ages of 39 and 75 years, underwent subcutaneous implantation of an electrode pacemaker for relief of recurrent syncopal episodes of Stokes-Adams disease. Their prolonged course of severe disability on medical therapy is in marked contrast to their new life, free of syncopal attacks, on pacemaker therapy. Experience in the management of these patients has shown that the electrode catheter, introduced intravenously to stimulate the right ventricular endocardium, is the best temporary therapy in a patient with uncontrollable syncopal attacks, and it should be used in all patients undergoing the surgical procedure of implantation. Pacemaker therapy is applicable to patients in sinus rhythm as well as to those in complete heart block. Observations on the effect of heart rate on cardiac output have shown the need of a faster rate in the presence of congestive failure for the maintenance of an adequate cardiac output. The favourable course of patients after implantation of a pacemaker indicates that this is the treatment of choice in Stokes-Adams disease.

Arrhythmia, Sinus↗

[Surgical treatment of ventricular tachycardia in patients with post-infarction aneurysms].

4 patients (P) with recurrent, sustained ventricular tachycardia (VT) resistant to medical treatment, underwent surgery for cure of this arrhythmia. Each P had episodes of VT lasting 30 or more seconds, 3 of them had episodes of ventricular fibrillation. In all cases rhythm disturbances were secondary to post myocardial infarction aneurysm. Coronary angiography showed in all P total occlusion of LAD, in 2 cases significant lesion in RCA were found. 1 P had lung cancer. All P underwent aneurysmectomy and an excision of the altered endocardium by Harken's method. The endocardial excision was performed without endocardial mapping. 2 P had concomitant CABG to RCA. In the P with lung cancer lobectomy was performed. There were 2 ++non-arrhythmic death. The P with lung cancer died because of sepsis due to lung abscess. One P died because of heart failure (preoperative EF 10%), 6 months after the surgery. The 2 survivors remained free of VT during a follow-up period 8 months. In conclusion, endocardial excision by Harken's method is efficient in treating recurrent sustained VT, resistant to medical treatment, in patients with post myocardial infarction aneurysm. The surgical procedure can be performed without intraoperative endocardial mapping.

Endocardium↗

[Detection of bacterial molecular markers in the tissue of cardiac valves in normal and pathological states by gas chromatography and mass spectrometry].

Samples from cardiac valves of 31 patients were analyzed by gas chromatography and mass spectrometry. The algorithm of mass spectrometric parameters was developed, which permitted the determination of about 200 known microbial fatty acids, aldehydes and sterols, sufficient for the detection and quantitative determination of more that 170 taxons of clinically significant microorganisms on the genus or species levels. The quantitative and qualitative differences in the composition of microbial markers of endocardial valves in normal and pathological states, particularly in cases of infectious endocarditis, were detected. The participation of 37 microbial taxons in the process was confirmed. The level of endocardium colonization in infectious endocarditis reached from 2 to 7 x 10(9) microbial cells/g of valvular tissue (which exceeded twofold the equivalent concentrations of the marker in the normal state). In terms of quantity, the leading role was played by Cardiobacterium hominis.

Adolescent↗

[Adherence of pacemaker electrodes to the endomyocardium--a postmortem study].

The weight of extraction of a pacemaker electrode from the endomyocardium was evaluated in 29 hearts (31 pacemakers). The maximum weight of extraction was 2375 g, the mean being 760 g. The weight of extraction depends on the duration of the implantation of the pacemaker, and on the thickness of the right ventricle, but there is no relationship to the shape of the pacemaker electrode, nor to the site of insertion in the right ventricular endomyocardium. In half the cases, pieces of endomyocardium adhered to the extracted electrode, the largest piece being 5 cm x 0.5 cm x 0.5 cm. The epicardium was not injured in any of these cases. It was often noticed that scarred adhesions existed between the cable of the pacemaker and the endocardium and/or the intima of the blood vessels. During traction in vivo, strangulation of the right heart and the blood vessels may occur.

Aged↗

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.

Adolescent↗

tek, a novel tyrosine kinase gene located on mouse chromosome 4, is expressed in endothelial cells and their presumptive precursors.

A search for protein tyrosine kinases expressed during murine cardiogenesis resulted in the isolation of a novel tyrosine kinase, designated tek, which maps to mouse chromosome 4 between the brown and pmv-23 loci. The deduced amino acid sequence of tek predicts that it encodes a putative receptor tyrosine kinase that contains a 21 amino acid kinase insert and which is most closely related in its catalytic domains to FGFR1 and the product of the ret proto-oncogene. In situ hybridization analysis of adult tissues, as well as sectioned and whole-mount embryos, showed that tek is specifically expressed in the endocardium, the leptomeninges and the endothelial lining of the vasculature from the earliest stages of their development. Moreover, examination of the morphology of tek-expressing cells, and staging of tek expression relative to that of the endothelial cell marker von Willebrand factor, revealed that tek is expressed prior to von Willebrand factor and appears to mark the embryonic progenitors of mature endothelial cells. tek encodes a novel putative receptor tyrosine kinase that may be critically involved in the determination and/or maintenance of cells of the endothelial lineage.

Amino Acid Sequence↗

[Histopathological changes post cryoablation in canine myocardium].

OBJECTIVE: The purpose of this study was to observe histopathological changes post cryoablation in canine myocardium, to characterize the specific ablation lesion post cryoablation. METHODS: Cryothermal ablation was applied on myocardium (both epicardium and endocardium) of 14 mongrel dogs with different ablation parameters (-25 degrees C x 4 min, -50 degrees C x 4 min, -75 degrees C x 4 min, -75 degrees C x 2 min, -75 degrees C x 6 min, -75 degrees C x 8 min). Lesion dimensions and histopathologic changes were observed. RESULTS: The discrete, sharply delimited lesions were detected in cryoablated myocardium. Histologically, cryoablation in all temperatures studied induced heterogeneous necrosis of the myocardium. Lesion dimensions are related to freezing time and temperature. CONCLUSION: Cryoablation is a feasible and preferably choice for clinical application due to its controllable myocardium lesions.

Animals↗

Hypereosinophilic syndrome evolving to acute lymphoblastic leukemia.

We report a case of hypereosinophilic syndrome (HES) which later evolved into acute lymphoblastic leukemia (ALL). A 37-year-old man showed typical clinical manifestations of HES: pulmonary infiltrates, erythematous skin rash, deep vein thrombosis, endomyocardial fibrosis, and diffuse central nervous system dysfunctions. Although he was treated with prednisolone and hydroxyurea, marked eosinophilia persisted and lymphoblasts gradually increased in the bone marrow. He died of severe disseminated fungal infection after anti-leukemic therapy. Autopsy revealed marked fibrous thickening of the endocardium, bilateral common iliac vein thrombosis, and chronic hepatitis with fibrosis. Neither eosinophilic nor leukemic cell infiltration was seen in any tissue at autopsy. Including this case, 24 patients with ALL and hypereosinophilia have been reported in English-language literature. We discuss the relationship between eosinophilia and ALL, and the mechanisms, particularly the role of eosinophil cationic protein (ECP), in causing various organ system dysfunctions in HES.

Adult↗