Animal model: pulmonary hypertension, cor pulmonale and endocardial fibroelastosis in monocrotaline-intoxicated nonhuman primates.
Explore the source record for details and available documents.
Animals↗
SEARCH · Search PubMed
Results for “ENDOCARDIAL FIBROELASTOSIS”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Development of endocardial fibroelastosis following myocardial infarction.
Explore the source record for details and available documents.
Adult↗
Ross-Konno operation with resection of endocardial fibroelastosis for critical aortic stenosis with borderline-sized left ventricle in neonates.
Explore the source record for details and available documents.
Retraction Notice↗
Determination of left ventricular diastolic chamber stiffness and myocardial stiffness in patients with congenital heart disease.
Left ventricular diastolic indexes were derived in 13 patients aged 5 to 21 years. Three had a normal heart, three had lesions causing volume overload and seven had coarctation of the aorta, including one whose main lesion was severe endocardial fibroelastosis. At cardiac catheterization simultaneous high fidelity pressure (P) and left ventricular volume (V) measurements were obtained and several points in one diastolic cycle taken. With use of a monoexponential formula (P = aebv) for P versus V, dP/dv and the operant chamber stiffness b were obtained. Similarly, with use of sigma = alpha e beta epsilon, d sigma/d epsilon, elastic stiffness (E) and the muscle stiffness constant KE were obtained. Values for b were 0.0273 +/- 0.0065 in normal subjects, 0.017 +/- 0.0043 in those with volume overload, 0.0369 +/- 0.0173 in those with coarctation (without endocardial fibroelastosis) and 0.0192 in the child with endocardial fibroelastosis. The plot of P versus V for coarctation was to the left and steeper than normal and the patients with volume overload had a flattened rightward curve, whereas the curve for those with endocardial fibroelastosis was extremely rightward. The stress-radii curves of the normal subjects and those with coarctation were similar whereas the curves for patients with volume overload and endocardial fibroelastosis were rightward of normal. The value for KE was 8.92 +/- 0.87 for the normal subjects, 8.26 +/- 0.75 for those with volume overload, 9.2 +/- 2.5 for those with coarctation and 22.75 for those with endocardial fibroelastosis. Thus, the pressure-loaded ventricle is stiffer than the normal, which in turn, is stiffer than the volume-loaded ventricle. This response, due to hypertrophy, appears to be appropriate in that diastolic stress was normalized and muscle stiffness was not increased except in the patient with endocardial fibroelastosis.
Adolescent↗
Ultrastructural aspects of endomyocardial biopsy in restrictive cardiomyopathy: evaluation of elastogenesis.
Besides elastic and collagen fibres, numerous smooth muscle (SM) cells were found in the thickened endocardium in 3 cases of restrictive cardiomyopathy and in 1 case of congenital endocardial fibroelastosis. In 3 other cases of congenital endocardial fibroelastosis, fibroblast-like cells were present besides numerous fibres. The former seemed to originate from SM cells. The authors present their opinion that both in congenital and acquired endocardial fibroelastosis, the lesion develops essentially in the same way. The basic phenomenon consists in increased elastoplastic activity of SM cells. In contrast, the aetiology (stimulus) of higher, elastogenous activity of SM cells remains obscure and seems to be different in individual cases.
Adolescent↗
Structural remodeling of the left atrial appendage in patients with chronic non-valvular atrial fibrillation: Implications for thrombus formation, systemic embolism, and assessment by transesophageal echocardiography.
Left atrial appendage (LAA) is frequently the site of thrombus formation in patients with chronic atrial fibrillation (AF). Transesophageal echocardiography and hematologic studies have identified blood flow stasis (spontaneous echogenic contrast) and abnormal coagulation (increased serum fibrinogen) as important predisposing factors to formation of LAA thrombi. However, the third component of the Virchow's triad, i.e., endothelial abnormalities, has not been adequately studied. Accordingly, we studied, at necropsy, the LAA morphology in 46 hearts of patients with (n = 22) and without (n = 24) chronic AF. Compared to patients without AF, those with AF had significantly larger LAA volumes (1.7% 1.1 vs. 5. 4% 3.7 mL, p = 0.0002), and larger luminal surface area of the bisected LAA (4.4% 1.8 vs. 7.1% 4.5 cm(2), p = 0.01). However, both the absolute and relative surface area of the transected pectinate muscles were reduced in patients with AF (2.6% 1.1 vs. 1.8% 1.0 cm(2), p = 0.02 and 38% 15 vs. 21% 14%, p = 0.0003). In addition, in most patients (73%) with chronic AF, the LAA showed significant endocardial thickening with fibrous and elastic tissue (endocardial fibroelastosis) compared to those without AF (13%, p < 0.0001). Endocardial fibroelastosis resulted in a smooth LAA luminal surface and encased the pectinate muscles. These findings suggest that LAA remodeling (dilation, stretching, and reduction in pectinate muscle volume, as well as endocardial fibroelastosis) occurs frequently in chronic AF and may contribute to the increased risk of thrombus formation and systemic embolism. Additionally, the information may have relevance in interpreting transesophageal echocardiographic images of the LAA in patients with chronic AF.
Adolescent↗
Abnormal morphological patterns of the left ventricular myocardium in the critical stenosis of the aortic valve and the intact ventricular septum.
The evaluation of prognostic factors in infants with critical stenosis of the aortic valve and intact ventricular septum is often misleading due to a complex interaction among lesions in the mitral and aortic valves, and the left ventricular myocardium. The clinical parameters on the left ventricular function, such as ejection fraction and left ventricular end-diastolic volume, are of particular interest as their effects on survival are very controversial. We performed a clinicopathologic analysis of two autopsied cases of this disease. Besides the morphological hallmarks of the aortic and mitral valves, these two cases showed two extreme types of pathology in the left ventricular myocardium, which might have significant impacts on the clinical evaluation of the left ventricular function. Case 1 showed endocardial fibroelastosis associated with abnormal intertrabecular spaces (so-called spongy myocardium), obscuring accurate estimation of the left ventricular end-diastolic volume. Case 2 showed ischemic necrosis of the apical part of the left ventricular myocardium. This infarct was associated with acute and chronic subendocardial ischemia and mild endocardial fibroelastosis. Aggravation of the left ventricular failure could be caused by the recent ischemic insult. The evaluation of the left ventricular function, therefore, should include the evaluation of the morphologic status of the myocardium as regards to whether there is ischemia, endocardial fibroelastosis or hypertrophied trabeculae in addition to stenotic lesions in the aortic and mitral valves.
Aortic Valve Stenosis↗
Neonatal surgical aortic commissurotomy: predictors of outcome and long-term results.
BACKGROUND: The objective of this study was to review our institutional experience concerning neonatal aortic commissurotomy and to identify predictors of outcome. METHODS: From January 1, 1990, to January 1, 2005, 65 neonates were referred for critical aortic stenosis. Among these 36 consecutive patients underwent surgical valvotomy by the same surgeon and 16 patients had balloon valvotomy. We examined clinical records to establish determinants of outcome and illustrate long-term results. RESULTS: In the surgical group 6 patients had nonstenotic parachute mitral valve, 6 had aortic coarctation, 13 had endocardial fibroelastosis; and 15 had depressed fractional shortening. In the balloon valvotomy group most infants had associated mitral stenosis (n = 7), small aortic annulus (n = 5), multiple anomalies (n = 2), endocardial fibroelastosis (n = 9), and depressed fractional shortening (n = 13). In the surgical group global mortality was 19%; 47% in infants with depressed fractional shortening and 0% in infants with normal fractional shortening. Mortality in the balloon valvotomy group was 56%. Low fractional shortening, low mean aortic gradient, endocardial fibroelastosis, and small aortic annulus were associated with fatal outcome. In the surgical group 6 patients needed Ross operation at a median interval of 4 years from commissurotomy. In the balloon valvotomy group 3 patients needed early mitral replacement and 5 aortic valve surgery. At a median follow-up of 6 years, all children are in New York Heart Association class I, all have a normal ventricular function, 2 have aortic restenosis, and 5 have at least moderate aortic incompetence. CONCLUSIONS: In a selected population of neonates with isolated aortic stenosis, surgical valvotomy offers an excellent long-term outcome to patients with a normokinetic left ventricle, while patients with a hypokinetic left ventricle have a poor outcome. Balloon valvotomy offers poor results when performed in patients with complex anomalies.
Aortic Valve Stenosis↗
[The contribution of pathology sections to the Italian Heart Transplant Project in the first 5 years of its activities (1985-1990)].
All the transplantation units within the Italian Heart Transplantation Project are supported by a section of pathology, devoted to the study of the recipient's heart, to patient monitoring by means of a schedule of endomyocardial biopsies, and, if that was the case, to examine the donor's heart and to analyse the causes of death. When successes and failures of the first five years of the Project's activity are weighed up, good results are observed: of the 847 operations performed (orthotopic, heterotopic and heart-lung transplants, and re-transplants) an actuarial survival rate of 77% at 5 years has been achieved. The sections of pathology believe to have contributed significantly to these results, examining as many as 10,446 endomyocardial biopsies. The indications for transplantation were: dilated cardiomyopathy (48.5%); ischemic (35.3%); valvular (5.9%) and congenital (2.4%) heart disease; hypertrophic cardiomyopathy (2.2%); endocardial fibroelastosis (1.7%); restrictive cardiomyopathy (1.4%); anthracycline cardiotoxicity (0.8%); myocarditis (0.8%); cardiac tumours (0.5%) and arrhythmogenic cardiomyopathy (0.2%). Distribution of recipients by sex and age varied according to the indications for transplantation: males were more common among the patients transplanted for ischemic (97%) and valvular (84%) heart disease, as well as for dilated (82%) and hypertrophic (78%) cardiomyopathy, whereas the opposite was true for endocardial fibroelastosis (males constituting 21%) and cardiac tumours (25%). Mean age at transplantation ranged from 49 years (ischemic heart disease) to 6 years (endocardial fibroelastosis). In the follow-up period, a 17.5% death rate was recorded; the main causes of death were the early failure of the transplanted heart (27 pts), postoperative complications (16), hyperacute rejection (4), acute rejection (18), infections (the singular most frequent cause of death, 35 pts), the proliferative endoarteritis of coronary branches (the so-called chronic rejection, that caused 21 deaths and required 14 re-transplants) and the development of neoplasms (11). The actuarial survival curve drops to 89% after the first postoperative month, abates to 82% at the end of the first year, and progressively decreases to 77% at the end of the fifth follow-up year. Rejection monitoring required an average number of 12.5 endomyocardial biopsies per recipient, and allowed 1.7 rejection episodes per patient to be diagnosed. The fewer were the rejection episodes occurring in a unit, the higher was the percentage of deaths due to infections.(ABSTRACT TRUNCATED AT 400 WORDS)
Age Factors↗
Endocardial fibrosis and hydrops fetalis in a premature infant.
We describe a premature neonate with endocardial fibrosis associated with hydrops fetalis. The infant did not have any other cardiac disorder and expired with congestive heart failure when five days old. Endocardial fibroelastosis has been previously described in neonates but is rare and is usually associated with other cardiac malformations. In only two previous reports was endocardial fibroelastosis associated with hydrops and in both of these cases there were also valvular deformities. We also discuss the possible significance of this association.
Diseases in Twins↗
[Emergency aneurysmectomy for persistent ventricular tachycardia].
Aneurysms are a later consequence of acute myocardial infarction, developing in about 20% of patients with transmural infarcts. Pathologically aneurysms have two general forms: the first type has large endocardial fibroelastosis overlying the infarct size, the second type contains little or no endocardial fibroelastosis but often contains a large amount of mural thrombus. These pathologic types correlate with clinical complications. The first type is related to recurrent sustained ventricular arrhythmias, the second type to systemic embolism. The importance of endocardial pathology in pathogenesis of ventricular: arrhythmias is also suggested by recent endocardial stripping procedures. We report the case of a patient 63 years old, with ischemic left ventricular aneurysm complicated with multiple morphologic sustained ventricular tachycardia resistant to drug treatment. Standing hemodynamic shock and anginal symptoms, was performed left ventricular aneurysmectomy in state of emergency. The surgical treatment was successful. A programmed ventricular stimulation prior to hospital discharge, not induced ventricular tachycardia, also Holter monitoring was normal. The patient was discharged after 18 days. After 4 months he is well, and no arrhythmia was demonstrated during ambulatory controls.
Emergencies↗
Retrospective study of congenital heart defects in 151 dogs.
The case records of 151 dogs diagnosed with congenital heart disease were reviewed retrospectively. The most common defect was aortic stenosis, accounting for 35 per cent of all cases, followed by pulmonic stenosis (20 per cent), ventricular septal defect (12 per cent), patent ductus arteriosus (11 per cent), mitral valve dysplasia (8 per cent), tricuspid valve dysplasia (7 per cent), endocardial fibroelastosis (1.9 per cent) and tetralogy of Fallot (0.6 per cent). Fifty-one breeds were represented, with golden retrievers, German shepherd dogs and boxers predominating. No overall sex predilection was obvious. Seventy-five per cent of the dogs were asymptomatic at presentation. The defects most often associated with presenting symptoms, such as dyspnoea, syncope, ascites, failure to grow and depression, were mitral valve dysplasia, atrial septal defect, tricuspid valve dysplasia and endocardial fibroelastosis. The latter presented with the most severe signs of heart failure. In some cases of aortic stenosis and pulmonic stenosis, where the defect could not be accurately visualised with two-dimensional echocardiography, Doppler echocardiographic examination was needed for definitive diagnosis.
Animals↗
Two chambered left ventricle. Three new varieties.
A short review of two chambered left ventricle is given, and this rare condition is briefly compared and contrasted with the more common condition involving the right ventricle. Three cases are described, each of which presents a new variety of subdivision of the left ventricle; in two of these the condition was first shown by angiocardiography. The first case had left heart hypoplasia with dysplastic stenosis of the mitral and aortic valves and severe endocardial fibroelastosis of the main left ventricular cavity. The second case also showed dysplasia of the left sided valves with pronounced endocardial fibroelastosis, and in addition there was a congenital malformation of the left lung. The third case showed a bizarre ventricular subdivision without other congenital malformations. The role of the intertrabecular myocardial sinusoids in the pathogenesis of some types of two chambered ventricle is discussed and this is considered to have been of significance in the first two cases. The third case possibly represented an intramyocardial aneurysm of obscure aetiology.
Female↗
Fetus with two identical reciprocal translocations: description of a rare complication of consanguinity.
We report on a 24-week fetus with multiple organ anomalies secondary to biparental inheritance of an apparently balanced t(17;20) reciprocal translocation. The pregnancy was terminated following the discovery by ultrasound of an abnormal heart and micrognathia. At autopsy, the following anomalies were found: Pierre-Robin sequence, hypoplasia of the right ventricle with muscular hypertrophy, and endocardial fibroelastosis, hypoplastic lungs, dysplastic left kidney, bilateral pelvicalyceal dilatation, central nervous system periventricular heterotopias and right sided club foot. Given the endocardial fibroelastosis and cleft palate, Eastman-Bixler syndrome (Facio-cardio-renal) is a possible diagnosis. The parents were first cousins and each had an identical t(17;20)(q21.1;p11.21) translocation. The fetal karyotype was 46,XX,t(17;20)(q21.1;p11.21)mat,t(17;20)(q21.1;p11.21)pat. While there are a few reports of consanguineous families where both the mother and father had the same reciprocal translocation and offspring with unbalanced karyotypes, we were unable to find any reports of a fetus/child with double identical reciprocal translocations. We propose that although the fetus had an apparently balanced karyotype, inheriting only the translocated chromosomes led to the unmasking of a recessive syndrome. It seems most likely that a gene (or genes) was disrupted by the breaks but the parents might also be heterozygous carriers of a recessive gene mutation since the fetus must be homozygous by descent for many loci on both chromosomes 17 and 20 (as well as on other chromosomal segments). It was not possible to totally exclude segmental uniparental disomy as a cause of the anomalies as no recombinations were detected for chromosome 17. However, there is no evidence to suggest that chromosome 17 is imprinted and UPD 20 was excluded thus making an imprinting error unlikely.
Abnormalities, Multiple↗
Balloon dilatation of the aortic valve in the fetus. A case report.
Fetal echocardiography has recently caused an impact on the treatment of congenital heart disease and in the field of therapeutic, cardiological intervention. The present study reports on a case of critical aortic stenosis, diagnosed in utero at 27 weeks' gestation, and in which balloon dilatation was attempted to improve the poor prognosis associated with this condition. Since the endocardium at this stage of development was apparently normal, this therapeutic intervention was attempted to avoid irreversible damage to the left ventricle. Although hydrops disappeared and the myocardium hypertrophied, endocardial fibroelastosis progressed and the neonate died within the first day of life, after surgical aortic valvotomy. More data are necessary to clarify whether endocardial fibroelastosis is really a consequence of high pressure in the left ventricle resulting from stenosis of the aortic valve or whether it is a disease, the progression of which is unavoidable once it takes hold.
Adult↗
Dilated cardiomyopathy in infants and children.
The outcome of medical treatment of dilated cardiomyopathy in infants and children was reviewed to develop a predictive index for selection of patients likely to benefit from cardiac transplantation. The clinical findings, laboratory investigations, treatment and outcome of 20 patients (Group 1) less than 2 years of age at presentation and 12 patients (Group 2) greater than 2 years of age at onset were compared. Of 20 Group 1 patients, 5 (25%) died. Available autopsies (four patients) showed endocardial fibroelastosis. Of 15 survivors, 10 showed improvement in cardiac status and 5 remained unchanged. Ninety-three percent of survivors had dilated cardiomyopathy consistent with endocardial fibroelastosis by angiocardiography. All 12 Group 2 patients died. In addition to age at presentation and poor outcome, Group 2 differed from Group 1 in having a higher incidence of other family members with cardiomyopathy, more significant rhythm disturbances at presentation and a more rapid course to death. Risk factors of poor outcome in both groups included persistent cardiomegaly and the development of significant arrhythmias by Holter electrocardiographic monitoring. Cardiac transplantation is recommended for children with dilated cardiomyopathy presenting after age 2 years who survive 1 month. Those patients less than 2 years old at presentation whose condition has not improved after 1 year and who have persistent cardiomegaly or complex ventricular arrhythmias may also benefit from transplantation.
Actuarial Analysis↗
Molecular diagnosis of myocarditis and dilated cardiomyopathy in children: clinicopathologic features and prognostic implications.
Myocarditis is the most common cause of heart failure in children. We investigated viral etiology of myocarditis/dilated cardiomyopathy (DCM) in children and correlated molecular findings with pathologic and clinical data. Polymerase chain reaction (PCR) or reverse transcription (RT)-PCR were used to analyze 59 endomyocardial biopsies from 48 consecutive young (<18 yrs) patients (pts) with clinical and histologic diagnosis of myocarditis and DCM, employing primers designed to amplify specific sequences of various DNA and RNA viruses. Nucleic acids were successfully extracted in 41 pts and viral genomes were found in 20 (49%): 12 out of 26 pts (46%) with myocarditis, 6 out of 13 (46%) pts with DCM, and both patients with endocardial fibroelastosis. Enteroviruses were more common in DCM (72%), whereas adenoviruses and enteroviruses shared the same rate (36%) in myocarditis. The mumps virus genome was detected in the two pts with endocardial fibroelastosis. More diffuse inflammatory infiltrates and myocyte damage as well as more impaired left ventricular end diastolic volume and shortening fraction were noted in viral positive cases. PCR positive pts had a worse outcome, resulting in transplantation or death. Three out of 8 pts with viral myocarditis who underwent cardiac transplantation had recurrent PCR-proven graft viral infection. Viral myocarditis/DCM appeared to be a more severe disease than nonviral forms. Enteroviruses were more common in DCM, whereas adenoviruses were as frequent as enteroviruses in myocarditis. Persistence of viral infection was associated with disease deterioration. Viral myocarditis relapsed after transplantation.
Adolescent↗
Endocardial abnormalities in left ventricular aneurysms. A clinicopathologic study.
To correlate morphologic subgroups of left ventricular aneurysms with the major clinical manifestations (ventricular arrhythmias, congestive heart failure, and systemic emboli), we studied 73 patients with such aneurysms--34 at autopsy and 45 after surgical excision. Sixty-three patients had a history of congestive heart failure, 5 had systemic emboli, and 28 had recurrent ventricular tachycardia. Two types of endocardial abnormalities were noted: thick endocardial plaque composed of fibrous elastic tissue that contained little or no thrombus (type I, 30 patients); and aneurysms with little or no endocardial fibroelastosis (type II, 43 patients), some with layers of thrombus obliterating the aneurysmal cavity. Emboli only occurred in 5 patients with type II aneurysms who had mural thrombi. Recurrent ventricular tachycardia occurred in 20 patients with type I aneurysms but only in 8 with type II (p less than 0.001), showing a relation between endocardial fibroelastosis and ventricular tachycardia. Structural differences in ventricular aneurysms thus appear to have pathophysiologic significance. Our findings have implications for the mechanisms of ventricular tachycardia in postinfarction patients and for its prevention by endocardial resection.
Adult↗