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PubMed1999

Late potentials and ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy.

We studied 38 patients (mean age 32 +/- 14 years) with arrhythmogenic right ventricular cardiomyopathy (ARVC) to evaluate the clinical significance of histologic features on endomyocardial biopsy specimens as related to signal-averaged electrocardiography (SAECG), spontaneous ventricular arrhythmias, and hemodynamic features. Fifteen patients presented with ventricular tachycardia or fibrillation (sustained ventricular arrhythmias), 23 with other minor arrhythmias. SAECG variables and right ventricular ejection fraction (RVEF) were statistically correlated with the extent of myocardial fibrosis on biopsy in ARVC. An increased percentage of fibrous tissue (> or = 30%) was a significant univariate predictor of late potentials (p = 0.004) and reduced RVEF (p = 0.02). The 18 patients with late potentials had an increased percentage of fibrous tissue (p = 0.01), a reduced RVEF (p = 0.0004), and a higher risk for sustained ventricular arrhythmias (p = 0.05) than the 20 patients without late potentials. RVEF was the most powerful predictor of late potentials (p = 0.004) at multivariate analysis. Moreover, RVEF < or = 50% was associated with an increased risk for development of sustained ventricular arrhythmias (p = 0.02). A SAECG parameter, namely the root-mean-square voltage of the terminal 40 ms at 25 Hz, was an independent predictive factor for the occurrence of sustained ventricular arrhythmias (p = 0.02). Although fibrous tissue may contribute to delayed myocardial activation in ARVC, a reduced RVEF plays an essential role for spontaneous manifestation of sustained ventricular arrhythmias.

Adolescent↗

PubMed1979

Transvascular endomyocardial biopsy in infants and small children. Myocardial findings in 10 cases of cardiomyopathy.

Transvascular endomyocardial biopsy specimens from nine children with congestive cardiomyopathy and one with hypertrophic cardiomyopathy were studied by light microscopy using sections 1 mu thick cut from Epon embedded tissue and by electron microscopy. There was a disparity between the severity of the physiologic impairment and the morphologic abnormalities. Interstitial fibrosis was present only in the one case in which significant viral antibody titers were obtained. The sizes of the cardiac muscle cells varied abnormally in all specimens. Cardiac muscle cells in two patients contained abnormal mitochondria, and a leptomeric fibril was found in one patient. Virologic cultures of the tissues were negative and no viral particles were identified by electron microscopy. An attempt was made to correlate the clinical and pathologic findings.

Basement Membrane↗

PubMed1992

Ventricular premature beats in young subjects without evidence of cardiac disease: histological findings.

This report describes the results of right ventricle endomyocardial biopsies from 26 subjects (mean age 27 +/- 10 years) with premature ventricular beats and normal cardiac anatomy and mechanical function. Light microscopy examination revealed normal myocardium in 10 subjects (38%), acute myocarditis in two (7%), borderline myocarditis in one (3.5%), non-specific histological abnormalities including cellular hypertrophy, fibrosis and degenerative changes in 11 (42%), vasculitis in one (3.5%) and findings compatible with right ventricular dysplasia in the final subject (3.5%). The frequency of ventricular premature beats, as assessed by Holter monitoring, and the results of electrophysiological testing did not correlate with histopathological findings and their severity. These data indicate that some young subjects with premature ventricular beats of unknown origin have abnormal right ventricular biopsy findings. Adequate follow-up will probably demonstrate the clinical utility of these observations.

Acute Disease↗

PubMedDate not supplied

Endomyocardial biopsy in diagnosis of myocardial diseases.

In the past years, diagnosis of myocardial diseases became less difficult. A major contribution in this direction has the recent development of diagnosis methods. One of the most recent diagnosis procedures is the endomyocardial biopsy, which manages directly through the histologic examination to establish a clear diagnosis in myocardial diseases. This method provides faithful morphological data about the pathological changes in the myocardium. The aim of our study was to underline the importance of endomyocardial biopsy in an accurate diagnosis of different cardiomyopathies. In the period of 12 years (1986-1998), in the Cardiovascular Department of the First Medical Clinic Târgu-Mureş, Romania, were admitted 137 patients suffering from different cardiomyopathies. We performed a number of more than 100 endomyocardial biopsies on 25 of them, obtaining in each case 3 to 5 samples of endomyocardial tissue from the right ventricle. In all cases the Cordis bioptome was introduced in the right ventricle via femoralis vein. The complications were minor: ventricular extrasystoles. In all cases, we succeeded to obtain enough tissue for the histopathological examination, from different areas of RV. Histologic examination showed in all cases characteristic aspects of different cardiomyopathies. helping to clarify the diagnosis. After performing Endomyocardial Biopsy, we record an important increase of dilated cardiomyopathy percentage of diagnosis, from 40% to 72%. In patients with dilated cardiomyopathy, we observed a good correlation between hemodynamical data (Ejection Fraction, Left Ventricle End-Diastolic Pressure, etc.) and the index of myocardial damage, especially the degree of interstitial fibrosis. Using this method it is possible to distinguish different types of cardiomyopathies, such as: primary dilated cardiomyopathy, eosinophilic cardiomyopathy, myocarditis, idiopathic hypertrophic cardiomyopathy. Because the complications of this procedure are insignificant, and because of its important benefit for an accurate diagnosis, we recommend to apply this method for different cardiomyopathies.

Adult↗

PubMed1984

Cardiac histologic findings in patients with life-threatening ventricular arrhythmias of unknown origin.

Percutaneous endomyocardial biopsy (right ventricle in 10, left ventricle in 2) was performed in 12 patients, aged 9 to 57 years, with serious ventricular arrhythmias occurring in the setting of normal cardiac anatomy and mechanical function. Light microscopic examination of tissue revealed histologic abnormalities in 11 patients, including myocardial cellular hypertrophy in 7, interstitial fibrosis in 5, endocardial fibrosis in 2, myocardial degenerative changes in 1 and increased interstitial cellularity in 1. One patient had histologic evidence of acute lymphocytic myocarditis. Thus, a majority of patients with serious ventricular arrhythmias and normal cardiac anatomy had histologic abnormalities, bringing into question the concept of primary electrical heart disease or idiopathic ventricular tachycardia.

Adolescent↗

PubMed2001

Myocardial ultrasonic tissue characterization for estimating histological abnormalities in hypertrophic cardiomyopathy: comparison with endomyocardial biopsy findings.

AIMS: In this study, we investigated the clinical usefulness of ultrasonic tissue characterization with integrated backscatter for the evaluation of myocardial histological abnormalities in comparison with endomyocardial biopsy findings in patients with hypertrophic cardiomyopathy. METHODS: Twenty patients with hypertrophic cardiomyopathy and 20 normal subjects were enrolled in this study. We measured two parameters for the ultrasonic tissue characterization with integrated backscatter: the magnitude of the cardiac-cycle-dependent variation in integrated backscatter signals (cdv-IB) and the mean value of integrated backscatter signals calibrated by the pericardium (cal-IB). These parameters were measured at both the interventricular septum and the left ventricular posterior wall. Histological findings of right ventricular endomyocardial biopsy specimens were analyzed by computer image analyzer. RESULTS: cdv-IB was significantly lower and cal-IB significantly higher in both the interventricular septum and the left ventricular posterior wall in patients with hypertrophic cardiomyopathy compared with normal subjects. In patients with hypertrophic cardiomyopathy, the degree of myocardial disarray, interstitial fibrosis, and nonhomogeneity of myocyte size showed positive correlations with cal-IB and negative correlations with cdv-IB. CONCLUSIONS: Ultrasonic tissue characterization with IB enables the noninvasive evaluation of myocardial histological abnormalities in patients with hypertrophic cardiomyopathy.

Adult↗

PubMed1987

Interobserver variability in the pathologic interpretation of endomyocardial biopsy results.

Controversy exists over the role of endomyocardial biopsy in evaluating patients with dilated cardiomyopathy, particularly in detecting myocarditis and in assessing prognosis. Interobserver variability, if high, could explain conflicting reports. To assess this possibility, we submitted biopsy specimens from 16 patients with dilated cardiomyopathy to seven cardiac pathologists. The same slides were independently reviewed by each and assessed for fibrosis, hypertrophy, nuclear changes on a 0 to 3+ scale, mean lymphocyte count per high-power field, and myocarditis. The prevalance of significant fibrosis ranged from 25% to 69%, hypertrophy from 19% to 88%, nuclear changes from 31% to 94%, and abnormal lymphocyte count from 0 to 38%. One or more pathologists diagnosed definite or possible myocarditis in 11 of the 16 patients. Of these 11 patients, three pathologists agreed about three and two pathologists agreed about five. Myocarditis was diagnosed by a single pathologist in three cases. We conclude that interobserver variability is high in interpreting biopsy specimens from patients with dilated cardiomyopathy and that quantitative and standardized methods are needed to increase diagnostic consistency.

Adult↗

PubMed1994

Histomorphometric features predict 1-year outcome of patients with idiopathic dilated cardiomyopathy considered to be at low priority for cardiac transplantation.

Cardiac transplantation for patients with idiopathic dilated cardiomyopathy (IDC) and poor left ventricular function usually is postponed until symptoms have become intolerable. However, the short-term prognosis of this subset of patients has been defined poorly. Accordingly, the 1-year outcome was investigated in 30 patients with IDC with an ejection fraction < or = 25% who showed a stabilized clinical condition at assessment for transplantation and were therefore considered at low priority for surgery. During follow-up, 10 patients (group A) showed a poor outcome: 2 died suddenly, and 8 had hemodynamic failure (4 of whom underwent transplantation and 4 of whom died from heart failure while on the waiting list). The remaining 20 patients (group B) had a benign outcome. At assessment for cardiac transplantation, clinical and electrocardiographic features, left ventricular dimension, and ejection fraction were similar between the two groups. However, group A patients had higher left ventricular end-diastolic pressure (p < 0.03) and lower cardiac index (p < 0.02) and stroke volume index (p < 0.03) with respect to group B patients. In addition, the former had a lower myofibril volume fraction (p < 0.001) and a higher nuclear area (p < 0.001) compared with the latter. Multivariate analysis selected myofibril volume fraction (p < 0.001) and nuclear area (p < 0.005) as the only independent predictors of a poor 1-year outcome. The combination of myofibril volume fraction < or = 89% and nuclear area > 50 microns 2 was found in all group A patients (sensitivity 100%) but in only 2 group B patients (specificity 90%). It is concluded that in patients with IDC considered at low priority for cardiac transplantation: (1) the 1-year freedom from a cardiac event is lower than that currently expected with surgery; (2) histomorphometric features, that is, the concurrency of low myofibril volume fraction and increased nuclear area, predict short-term outcome; and (3) endomyocardial biopsy at assessment for cardiac transplantation might improve the rationalization of the timing of the procedure.

Adult↗

PubMed1990

A case of biopsy-proven myocarditis progressing to autopsy-proven dilated cardiomyopathy.

A 22-year-old man presented with congestive heart failure following flulike symptoms. The diagnosis of acute myocarditis was confirmed by endomyocardial biopsy, which revealed mild infiltration of inflammatory cells. A favorable response to beta-adrenergic receptor blockade was seen, and the patient was discharged without symptoms. Five months later, however, congestive heart failure recurred, and intracardiac thrombi were demonstrated. The patient died after two months. Postmortem examination revealed left ventricular dilatation with slight interstitial fibrosis; the diagnosis was dilated cardiomyopathy. Thus, progression of biopsy-proven myocarditis to dilated cardiomyopathy 10 months after the onset of disease was documented.

Adrenergic beta-Antagonists↗

PubMed1989

The morphologic spectrum of dilated cardiomyopathy and its relation to immune-response genes.

Endomyocardial biopsies from 174 patients with dilated cardiomyopathy (DC) were examined. Eight patients with histologically proven myocarditis were excluded from the study. A peculiar pattern of oversized and bizarre nuclei was observed in only some of the remaining patients. Two groups were identified: those with and without this feature (groups A and B, respectively). Myocyte width, nuclear diameter and nuclear/sarcoplasmic ratio were significantly higher in group A. The mean respective values were 36 +/- 5 mu, 14 +/- 3 mu and 0.41 +/- 0.08 for group A versus 20 +/- 8 mu, 7 +/- 2 mu and 0.37 +/- 0.08 for group B. Interstitial fibrosis was similarly present in groups A and B. Endocardial thickness was significantly increased in all patients, with group A showing the highest mean value. The morphologic features showed no correlation with the clinical condition of the patients at time of presentation. HLA typing was performed in 50 consecutive patients, 38 from group A and 12 from group B. DR4 and DR5 antigens were significantly more frequent in DC patients than in a normal population control (400 blood donors), while DR3 was less frequent. Group A was more strongly associated with the DR5 antigen than group B (55.3 vs 25.0%, respectively). It was less strongly associated with the DR4 antigen compared with group B (21.5 vs 41.7%, respectively). No difference was observed between the 2 groups concerning negative association with the DR3 antigen. Endomyocardial biopsies from DC patients reveal marked morphologic changes from patient to patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1984

Right ventricular endomyocardial biopsy in undetermined form of Chagas' disease.

Right ventricular endomyocardial biopsy was carried out in thirty three patients with undetermined form of Chagas' disease. Fragments obtained by this method were analysed under light microscopy with hematoxilin-eosin, and Masson trichromic stains. Thirteen (39.4%) patients showed normal myocardial fragments and twenty patients (60.6%) had them altered. Alterations included fiber degeneration, volume changes, interstitial edema, inflammatory infiltrates and fibrosis. These data permit to conclude that only part of patients with this form of Chagas' disease have an incipient myocardial attack and that the alterations found in the fragments obtained are mild. The remaining patients would be either individuals with chagasic infection without cardiac disease or have spontaneous healing. This should be considered in the future treatment of the disease.

Adult↗

PubMed1984

Left ventricular function and myocardial structure in aortic valve disease before and after surgery.

47 patients with aortic valve disease were studied by left ventricular micromanometry and cineangiography before and 18 months after successful valve replacement. There were 27 patients (17 with aortic stenosis, ten with aortic insufficiency) with moderate hypertrophy (angiographic mass less than 180 g/m2) and 20 patients (ten with aortic stenosis, ten with aortic insufficiency) with severe hypertrophy (angiographic mass greater than or equal to 180 g/m2). In the patients with moderate hypertrophy ejection fraction was slightly although not significantly smaller than in 14 controls. Peak systolic circumferential wall stress was, however, significantly increased. In the patients with severe hypertrophy ejection fraction was significantly decreased although afterload was similar to that in the patients with moderate hypertrophy. This indicated a depressed contractile state in the patients with mass exceeding 180 g/m2. After surgery peak systolic wall stress returned to normal levels in both groups. Among the patients with severe preoperative hypertrophy only the patients with aortic stenosis showed a normal ejection fraction after surgery, whereas in the patients with aortic insufficiency ejection fraction improved but remained depressed compared to the controls. In a second study, comparison of left ventricular function with myocardial structure (endomyocardial biopsies) was carried out in 30 patients with aortic valve disease (group 1: 18 with aortic stenosis, group 2: twelve with aortic insufficiency). Both groups were restudied 18.8 and 17.4 months, respectively, following successful aortic valve replacement. Preoperative biplane ejection fraction (60 and 57%), angiographic mass (183 and 186 g/m2), muscle fibre diameter (31.1 and 30.8 mu), interstitial fibrosis (17.1 and 17.4%) and left ventricular fibrous content (31.0 and 32.2 g/m2) were similar in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Stenosis, Subvalvular↗

PubMed1983

Quantitative morphologic findings of the myocardium in idiopathic dilated cardiomyopathy.

This study assesses the relation between quantitative morphologic findings and left ventricular contractile function in patients with idiopathic dilated cardiomyopathy. Left ventricular endomyocardial catheter biopsy specimens were obtained from 73 patients during diagnostic heart catheterization. All patients had normal coronary arteriograms but abnormal electrocardiograms. Twenty-six patients had normal left ventricular function (ejection fraction greater than or equal to 55%), whereas 47 patients had contractile dysfunction (ejection fraction less than or equal to 54%). Myocardial fiber diameter, volume fraction of interstitial fibrosis, and intracellular volume fraction of myofibrils were determined by light microscopic morphometry. Results of light microscopic morphometry were confirmed by electron microscopic morphometry in 12 patients. The coefficient of variation (analysis of several biopsies from the same patient) was 6% for determination of fiber diameter, 43% for interstitial fibrosis, and 3% for volume fraction of myofibrils. Fiber diameter (r = -0.32, p less than 0.01) and fibrosis (r = -0.47, p less than 0.001) showed a negative correlation, the volume fraction of myofibrils (r = 0.55, p less than 0.001) and calculated myofibrillar mass per 100 g of myocardium (r = 0.64, p less than 0.001) a positive correlation with the ejection fraction. Thus, (1) sampling error is low for determination of fiber diameter and myofibrils but high for evaluation of fibrosis, and (2) a reduction in the volume fraction of myofibrils and an increase in fibrosis are morphologic correlates of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy.

Adult↗

PubMed1992

Endomyocardial biopsy approach in cases with ventricular arrhythmias.

Right ventricular endomyocardial biopsies were performed in patients with ventricular tachycardia (VT: n = 20) and idiopathic premature ventricular contraction (PVC: n = 23). Active myocarditis was found in one case and postmyocarditic change in 9 cases. Significant pathology according to our definition was found in 9 out of the total of 43 cases (20.5%). The pathological features of the 9 cases were interstitial fibrosis in 5, disarrangement of muscle bundles in 6, degeneration of myocytes in one and increase in fatty tissue in 7. Increase in fatty tissue was characteristically observed in 6 out of the 20 cases with VT and it was noteworthy that the VT cases with left bundle branch block configuration showed high incidence of the fatty tissue (5/12 cases, 42%).

Adolescent↗

PubMed1986

Peripartum heart disease: an endomyocardial biopsy study.

Endomyocardial biopsies were performed in 11 African women in Nairobi who presented with the clinical features of peripartum cardiomyopathy. The samples were studied by light and electron microscopy. In five patients there was evidence of a "healing myocarditis", that is the presence of a mild inflammatory cell infiltration within the myocardium with foci of necrosis and variable amounts of hypertrophy and fibrosis. Of the nine patients who were followed up, three out of four with myocarditis had persistent heart failure and four out of five without myocarditis improved. Peripheral blood T lymphocyte cell subsets were measured in nine patients by means of monoclonal antibodies. A high helper:suppressor T cell ratio was found in three patients. Almost half of this group of patients with peripartum cardiomyopathy had myocarditis in their biopsy specimens. The myocarditis may have been due to an inappropriate immunological reaction in some patients.

Adolescent↗

PubMed1986

Familial hypertrophic cardiomyopathy mimicing typical dilated cardiomyopathy.

An autopsied patient who showed typical dilated cardiomyopathy (DCM)-like features and was pathologically diagnosed with hypertrophic cardiomyopathy (HCM) is presented. The patient, a 60-year-old male at the time of death, died of intractable congestive heart failure. At autopsy the heart weighed 570g and showed marked left ventricular (LV) dilatation with a thin wall (ventricular septum/free wall of the LV = 7 mm/8 mm). There was no evidence of significant stenosis in the extramural coronary arteries. Massive fibrosis was found in the middle and outer thirds of the ventricular septum and anterior wall of the LV (48% in the ventricular septum and 9% in the free wall of the LV). As myocytes were not present in the area with massive fibrosis, percent area of disarray was calculated excluding the area of massive fibrosis and found to be 30% in the ventricular septum. Based on the marked increase in the percent area of disarray, this case was diagnosed as HCM. The patient's 37-year-old son showed asymmetric septal hypertrophy on echocardiography (ventricular septum/posterior wall of the LV = 15 mm/11 mm), marked LV hypertrophy on electrocardiography, and diffuse and marked disarray by endomyocardial biopsy. There were also LV dilatation (LV diastolic dimension = 51 mm) and hypokinesis of the LV; as a result, a diagnosis of HCM with features of DCM was made.

Adult↗

PubMed1990

High incidence of significant pathology in endomyocardial biopsy and familial occurrence in cases with arrhythmia and/or conduction disturbance.

In our series of studies of cardiomyopathy with endomyocardial biopsy in 1500 cases, we have noticed that there were a certain number of patients with arrhythmias and/or conduction disturbance who showed no evidence of hypertrophic or dilated form of caridiomyopathy but showed significant pathology in the biopsy. Significant pathology was determined when the biopsy findings from the right atrium and the right ventricle showed apparent pathology such as interstitial fibrosis, myocyte degeneration, and fragmentation of muscle bundles. Among 226 biopsied patients in whom arrhythmia and/or conduction disturbance were the main clinical feature, 85 patients (38%) demonstrated significant pathology in their biopsy. Familial occurrence was note-worthy among these patients. We propose that these cases should be grouped as nonhypertrophic, nondilated, and nonrestrictive cardiomyopathy, and suggest the term electric disturbance type of cardiomyopathy (ECM).

Arrhythmias, Cardiac↗

PubMed1993

Ventricular expression of brain natriuretic peptide in hypertrophic cardiomyopathy.

BACKGROUND: Brain natriuretic peptide (BNP), as a cardiac hormone, is expressed together with atrial natriuretic peptide (ANP) in the ventricles in congestive heart failure. However, the ventricular expression of BNP in hypertrophic cardiomyopathy (HCM) with normal systolic function is still unclear. METHODS AND RESULTS: The study population consisted of 39 HCM patients with asymmetric septal hypertrophy and 10 control subjects without any specific cardiac disease. Eleven cases of HCM were obstructive (HOCM), and the other 28 cases were nonobstructive (HNCM). All of these patients had a normal ejection fraction. Immunohistochemical analysis of endomyocardial biopsy specimens with specific monoclonal antibodies showed BNP immunoreactivity in the HOCM group (5/10, 50%) but not in the HNCM group (0/22) or in control subjects (0/5). In HOCM, left ventricular end-diastolic pressure was significantly higher in the BNP-positive patients than the BNP-negative patients. Histological changes such as myocardial fiber disarray, hypertrophy of myocytes, and fibrosis were greater in BNP-positive patients than BNP-negative patients in HCM. However, the expression had no significant relation with other clinical parameters. The elevation of the BNP plasma level versus control subjects was marked in both HOCM (85-fold) and HNCM (23-fold). By contrast, the elevation of the ANP plasma level versus control subjects was mild in HOCM (5.7-fold) and HNCM (4.2-fold). The ratio of BNP level to ANP level was higher in HOCM (4.16) than in HNCM (1.46) and control subjects (0.28), and it was higher than the ratio previously reported for severe congestive heart failure (1.72). CONCLUSIONS: These findings suggest that BNP is expressed in the ventricular myocytes of HCM with normal systolic function. In HOCM, ventricular expression of BNP may be augmented in response to both obstruction and diastolic dysfunction.

Adult↗