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PubMedDate not supplied

The use of endomyocardial biopsy in a serious cardiac abnormality associated with polymyositis: a case report.

Life-threatening cardiac abnormalities in polymyositis (PM) patients may result from myocardial inflammation, fibrosis, or another disease process. Few procedures have reliably identified the pathologic basis for these abnormalities. We report a patient with PM, severe congestive cardiomyopathy, and life-threatening ventricular arrhythmias in whom an endomyocardial biopsy was done. The histopathological findings of fibrosis without inflammation, which were confirmed at autopsy, facilitated the decision to treat the patient symptomatically without intensification of antiinflammatory therapy. Endomyocardial biopsy may be a useful technique to determine the exact nature of serious cardiac abnormalities in PM patients so that appropriate therapy may be given.

Adult↗

PubMed1989

Ventricular fibrillation without apparent heart disease: description of six cases.

Since 1977, six patients (five males and one female), aged 14 to 35 years, resuscitated from ventricular fibrillation, were referred to our department for detailed evaluation, after exclusion of major cardiac pathologic conditions. Four patients had a family history of heart disease. Basic ECGs showed sinus rhythm in all of them. PR interval was prolonged in one. Two patients had complete and one had incomplete right bundle branch block. One patient had inverted t waves in V1-3 and late potentials. Three had an upsloping ST-T segment elevation in V1-2. The cardiothoracic index was less than 0.5 in five and 0.50 in one. In one of the five patients studied, the clinical episode of ventricular fibrillation was reproduced by stimulation of the right ventricular outflow tract during electrophysiologic study. Results of cross-sectional echocardiography and angiography showed predominantly structural and wall motion abnormalities of the right ventricle in five patients and slight wall motion abnormalities of the left ventricle in two. Two patients also had mitral and tricuspid valve prolapse. Coronary arteries were normal in all five patients examined. Results of endomyocardial biopsy showed no abnormalities in one patient, fibrosis in two, and fibrolipomatosis in one. Two patients died during follow-up: autopsy was performed in one and results showed right ventricular cardiomyopathy. Thus in five of these selected patients with apparent idiopathic ventricular fibrillation, some abnormalities, predominantly of the right ventricle, were documented only after detailed investigation; however, clinical history and some nonspecific ECG abnormalities were factors in the diagnostic procedure.

Adolescent↗

PubMed1977

Systolic and diastolic ventricular function in cardiac amyloidosis. Studies in six cases diagnosed with endomyocardial biopsy.

Five cases of cardiac amyloidosis diagnosed after endomyocardial biopsy are described. Systolic function was normal in three cases as assessed by cardiac index, ejection fraction and maximal velocity of contractile element shortening at zero developed pressure. The diastolic dip and plateau wave form was a characteristic but not an invariable finding. Increased ventricular end-diastolic pressure but normal end-diastolic volume indexes in all cases implied a disorder of diastolic compliance. The diastolic pressure-volume plots suggested a sudden decrease in distensibility after normal early filling of the ventricle. The functional defect did not necessarily differ from that of other diseases of heart muscle. Therefore, hemodynamic data should not be relied upon for diagnostic purposes. Electron microscopy was the most reliable aid in making the diagnosis from the endomyocardial biopsy specimens especially in the presence of fibrosis. It may not be valid to assume a diagnosis of cardiac amyloidosis from the finding of amyloid in other organs.

Adult↗

PubMed1986

Chronic anthracycline cardiotoxicity: haemodynamic and histopathological manifestations suggesting a restrictive endomyocardial disease.

Of 38 patients referred with suspected cardiotoxicity after administration of antineoplastic drugs, 11 patients with signs of manifest or latent anthracycline cardiotoxicity were selected for heart catheterisation with endomyocardial biopsy. Ultrastructural abnormalities of the myocytes with myofibrillar loss and cytoplasmic vacuolation were present in most patients and these findings were more pronounced in biopsy specimens from the left ventricle. Surprisingly, light microscopy showed considerable fibrous thickening of the endocardium in 10 of 11 patients, primarily in the left ventricle. These morphological findings together with the echocardiographic and the haemodynamic data suggest that chronic anthracycline cardiotoxicity is a restrictive endomyocardial disease. The biochemical mechanisms responsible for endocardial fibrosis are unknown, but drug induced damage to the endocardium, possibly mediated via hormonal or humoral agents, may feature in the initial phase of the toxic process. The present observations contribute toward the understanding of the pathophysiology of human anthracycline cardiotoxicity.

Adult↗

PubMed2000

[Diffuse left ventricular hypokinesis mimicking dilated cardiomyopathy with multi-vessel coronary vasospasm].

We investigated 7 patients with multi-vessel coronary vasospasm (> or = 75%) and diffuse left ventricular hypokinesis by coronary angiography and echocardiography. Four patients were male and 3 were female and mean +/- SD age was 63.0 +/- 11.2 years. Chief complaints were dyspnea in 3 patients, and chest pain, appetite loss, palpitation and general fatigue in one each. New York Heart Association functional classification was I in one patient, II in 5 and III in one. Mean heart rate was 73.9 +/- 11.6 beats/min. Initial echocardiography showed left ventricular end-diastolic diameter (LVDd) 54.4 +/- 5.5 mm, left ventricular end-systolic diameter (LVDs) 43.7 +/- 4.8 mm and percentage fractional shortening (%FS) 19.7 +/- 2.6%. The left ventricle was not remarkably enlarged despite poor contraction. Coronary vasospasm was induced after acetylcholine injection into the right coronary artery in 6 patients, left anterior descending artery in 7 and circumflex artery in 5. Four patients developed three-vessel coronary vasospasm. Three patients underwent endomyocardial biopsy which showed non-specific mild fibrosis. They were treated with nitrates and/or Ca-antagonists to prevent coronary vasospasm. Follow-up echocardiography was performed in 6 patients after 8.5 +/- 6.6 months. Echocardiography revealed marked improvement in left ventricular contraction (LVDd 49.7 +/- 4.6 mm, LVDs 35.8 +/- 4.4 mm, p < 0.05; %FS 27.9 +/- 4.5%, p < 0.05). These data suggested that left ventricular dilation was not prominent despite the poor contractility in patients with multi-vessel coronary vasospasm and diffuse left ventricular hypokinesis. The left ventricular dysfunction might be hibernating myocardium produced by multiple episodes of coronary vasospasm. Anti-vasospastic agents were effective in these patients.

Acetylcholine↗

PubMed2003

[Reversible left ventricular dysfunction manifesting as hyperkinesis of the basal and the apical areas with akinesis of the mid portion: a case report].

A 60-year-old man underwent emergent cardiac catheterization because of anterior chest oppression. He had been treated with calcium antagonist and nitrate. Coronary angiography showed no organic stenosis. However, left ventriculography revealed that the basal and the apical areas were hyperkinetic and the mid portion was akinetic. Echocardiography showed that the bizarre-shaped left ventricular dysfunction had completely recovered after a week. Serial electrocardiography demonstrated initial ST-segment elevation in the precordial leads followed by T-wave inversion in II, III, a VF and V4-V6. Coronary artery spasm provocation tests were negative. Endomyocardial biopsy revealed focal myocardial depletion and mild fibrosis. The present case may be an atypical form of tako-tsubo-like left ventricular dysfunction in which the left ventricular contraction is symmetric with both hyperkinetic and akinetic areas.

3-Iodobenzylguanidine↗

PubMed2006

[Peripartum cardiomyopathy with antiphospholipid antibody: a case report].

A 32-year-old woman tested positive for lupus anticoagulant when she had fever of unknown origin at 18 weeks of pregnancy. Sixteen days after a normal delivery at 35 weeks, she developed dyspnea and was hospitalized with heart failure. Chest radiography showed severe pulmonary edema. Echocardiography showed dilation and diffuse hypokinesis of the left ventricle. The diagnosis was peripartum cardiomyopathy. The patient responded to diuretic and vasodilator therapy. Endomyocardial biopsy revealed mild myocardial degeneration and interstitial fibrosis. Heart failure due to coronary microthrombosis has been indicated in patients with antiphospholipid antibodies, suggesting such a relationship in this case.

Adult↗

PubMed1989

Progressive dilated cardiomyopathy in a patient with longstanding and complete prednisone-induced hematological remission of idiopathic hypereosinophilic syndrome.

A female patient is described in whom the diagnosis of idiopathic hypereosinophilic syndrome (HES) with heart disease and peripheral neuropathy was made at the age of 32 years. Although prednisone induced a prompt and longstanding complete hematological remission, progressive and eventually intractable heart failure developed, and the patient died 6 years later. Endomyocardial biopsy at diagnosis showed infiltration with intact and disintegrated eosinophils and Charcot-Leyden crystals. Echocardiographic follow-up (including Doppler-Echocardiography) revealed mitral regurgitation with thickening and impaired motility of the posterior mitral leaflet, as well as progressive dilated cardiomyopathy. At autopsy, a diffuse interstitial fibrosis with patchy prominence in an eccentric hypertrophic and highly dilated heart was found. There were no significant endocardial thickening and no mural thrombi. In contrast to the findings of the initial endomyocardial biopsy, autopsy revealed no eosinophilic infiltrate. In this case, eosinophil-induced heart disease manifested as dilated cardiomyopathy, without endocardial fibrosis as originally described by Löffler. We speculate, that eosinophils have been deposited predominantly in the myocard at an early stage of disease, and - activated locally - secreted their granule proteins producing an initial damage to capillary endothelial cells and myocytes. After prednisone-induced clearance of eosinophils from blood and tissues, progressive, self-perpetuating interstitial fibrosis of the myocard and loss of myocytes eventually resulted in end-stage dilated cardiomyopathy.

Adult↗

PubMed2001

Recurrence of giant cell myocarditis in cardiac allograft.

BACKGROUND: Giant cell myocarditis causes essentially irreversible fulminant left ventricular dysfunction with associated conduction abnormalities and congestive failure. Response to immunosuppressive therapy is poor and cardiac transplantation is the only viable treatment option. The histologic hallmarks of giant cell myocarditis include a polymorphous inflammatory response with numerous multinucleated giant cells and extensive myocyte necrosis in a geographic pattern. There were 38 patients who received a cardiac transplant for giant cell myocarditis in the Giant Cell Myocarditis Registry. Among these patients, there were 9 recurrences of disease in the allograft. Concern has been expressed that recurrence of giant cell myocarditis in the allograft might be a contraindication for cardiac transplantation in the future. METHODS: In our single-center analysis we describe the clinical and histologic findings of 5 patients transplanted for giant cell myocarditis at the Cleveland Clinic. RESULTS: All but 1 of the patients were New York Heart Association (NYHA) class 4 with an average cardiac index (CI) of 1.52 liters/min x m(2). Of the 5 patients transplanted, 1 developed recurrent giant cell myocarditis. Routine right ventricular endomyocardial biopsy at 1 week exhibited severe multifocal myocardial fibrosis in addition to mild acute vascular rejection and mild grade 1A cellular rejection. Follow-up biopsy in this patient indicated grade IIIA moderate acute rejection in addition to multinucleated giant cells. Two distinct inflammatory processes were noted consisting of foci of T-cell inflammation identified by immunohistochemistry to be consistent with rejection, and a second inflammatory process with few mononuclear cells staining for macrophage or T-cell markers with eosinophils and myocyte necrosis consistent with giant cell myocarditis. Follow-up right ventricular endomyocardial biopsies (RVBXs) in this patient have subsequently demonstrated improvement in the degree of inflammatory infiltrate without vascular or significant cellular rejection. Vascular rejection was noted in 1 of the remaining 4 patients and was treated successfully with muramab-CD3 and plasmapheresis. CONCLUSIONS: Giant cell myocarditis should be expected to recur in the allograft and often does so concurrently with rejection. However, the disease in the allograft responds to therapy in a favorable manner, which differs dramatically from that in the native heart. This might be the result of detection of the disease at an earlier stage than in the native heart, or the immunosuppression milieu in the allograft. The favorable response to therapy suggests that the likelihood of recurrence of giant cell myocarditis should not be considered a barrier to transplantation.

Adult↗

PubMed1997

Cell-mediated cytotoxicity in hearts with dilated cardiomyopathy: correlation with interstitial fibrosis and foci of activated T lymphocytes.

OBJECTIVES: The purpose of this study was to investigate the expression of perforin and T-cell intracellular antigen-1, two crucial components of lymphocyte-mediated cytotoxicity, in endomyocardial biopsies from patients with idiopathic dilated cardiomyopathy. BACKGROUND: Previous reports have demonstrated the presence of myocardial interstitial fibrosis and increased infiltrating lymphocytes in patients with dilated cardiomyopathy. However, the pathogenic significance of these lymphocytic infiltrates remains unclear. METHODS: Endomyocardial biopsies from 134 patients with idiopathic dilated cardiomyopathy were histologically and immunohistologically analyzed. Monoclonal antibodies against diverse T-lymphocyte antigens, perforin and T-cell intracellular antigen-1 were used with the highly sensitive avidin-biotin complex technique. Positive cells were counted in at least 10 high power field. RESULTS: Perforin and T-cell intracellular antigen-1 were immunohistologically detected in all biopsies. Immunoreactivity was restricted to the cytoplasm and was granular in nature, indicating specific staining of cytoplasmic granules. Correlations were established between the expression of perforin and T-cell intracellular antigen-1 and the abundance of foci of various T-lymphocyte subpopulations and, most importantly, the degree of interstitial fibrosis on routine histologic examination (p = 0.015). CONCLUSIONS: Cytotoxic activity is clearly present in endomyocardial biopsies from patients with idiopathic dilated cardiomyopathy. Local activation-that is, focal accumulation of T lymphocytes-seems to be important for the generation of lymphocyte-mediated cytotoxicity. The interstitial fibrosis commonly seen in dilated cardiomyopathy may be caused by cytotoxic T-lymphocyte damage to the myocardium.

Adult↗

PubMed1988

[Diastolic dysfunction and its relation to myocardial fibrosis in essential hypertension].

This study elucidated diastolic left ventricular dysfunction and whether myocardial interstitial fibrosis correlates with diastolic dysfunction in mild to moderate systemic hypertension (HT). Six normotensive subjects, 18 hypertensive patients without left ventricular hypertrophy (LVH) and 10 hypertensive patients with significant LVH were evaluated. M-mode echocardiography was used to determine fractional shortening (FS), isovolumic relaxation time (IRT), and left ventricular filling volume during rapid and slow filling periods and the atrial contraction period (RFV, SFV, ACV). The quotients of the left ventricular filling volume and the end-diastolic volume and stroke volume were also calculated. Simultaneous biventriculography was used to determine the end-diastolic thickness of the interventricular septum and posterior wall. Right ventricular endomyocardial biopsies were performed to calculate the percentages of fibrosis. The FS was normal in all groups. The percentages of fibrosis in the two HT groups were significantly greater than those in the normals. The IRT of the HT groups was significantly greater, and the RFV, RFV/EDV and RFV/SV were significantly less than those of the normals. Multiple regression analysis showed that the wall thickness and the percentages of fibrosis correlated significantly with IRT, RFV, RFV/EDV and RFV/SV. The standard coefficients of correlation of wall thickness and the percentages of fibrosis were 0.333 and 0.239 in respect to IRT, and -0.304 and -0.473 in respect to RFV. There were significant correlations between the percentages of fibrosis and RFV (r = -0.675), RFV/EDV (r = -0.664) and RFV/SV (r = -0.602) in the normals and in cases of HT without LVH.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗

PubMed1985

Findings on endomyocardial biopsy in infants and children with dilated cardiomyopathy.

Fifteen infants and children with dilated cardiomyopathy underwent transvascular endomyocardial biopsy. The light and electron microscopic findings were reviewed to evaluate the presence of lymphocytes as an indicator of active myocarditis. Both ventricles were biopsied in 13 patients, and the right ventricle only was biopsied in 2. None of the endomyocardial specimens obtained by biopsy revealed an inflammatory process. Interstitial fibrosis, myofiber hypertrophy, degeneration and necrosis were found. Ultrastructural abnormalities of the mitochondria, T tubules or Z bands were noted in approximately one-third of patients. Persistent, active myocarditis is an uncommon cause of dilated cardiomyopathy in children. Immunosuppressive therapy, which may be harmful, should be considered only after myocardial inflammation has been documented by endomyocardial biopsy.

Adolescent↗

PubMed1994

Role of myocarditis in athletes with minor arrhythmias and/or echocardiographic abnormalities.

We report the clinical and instrumental data, including the endomyocardial biopsy findings, of six young athletes presenting with minor arrhythmias and/or echocardiographic abnormalities. In one of them, a left ventricular dilation with moderate depression of the systolic function had been attributed to an athlete's heart. A diagnosis of arrhythmogenic right ventricular dysplasia had been made in three others, one with right ventricular dilation and apical hypokinesia, and two with ventricular arrhythmias with QRS morphology of left bundle branch block. A myocarditis could be unequivocally established in four athletes (two with and two without fibrosis). In the remaining two, with a clinical history strongly suggesting a previously acute myocarditis, the endomyocardial biopsy specimen revealed a nonspecific fibrosis compatible but not definitely pathognomonic of a healed myocarditis. Our report suggests that a myocarditis may be a cause of minor rhythm disturbances and/or echocardiographic abnormalities in athletes. A prevalent localization of the inflammatory process in the right ventricle with or without the occurrence of ventricular arrhythmias with left bundle branch block morphology can mimic an arrhythmogenic right ventricular dysplasia. An early diagnosis of myocarditis in athletes is useful to avoid the risk of fatal arrhythmias, also considering that rest still keeps on being one of the most effective strategies in myocarditis management.

Adolescent↗

PubMed2002

A case of hypereosinophilic syndrome presenting mid-ventricular obstruction.

A 59-year-old man with hypereosinophilic syndrome (HES) who had been maintained with low-dose prednisolone for 5 years developed the characteristic features of hypertrophic cardiomyopathy. Left ventricular endomyocardial biopsy revealed no eosinophilic infiltration but extensive myocardial fibrosis. Cardiac involvement in HES presents as endocardial fibrosis, resulting in a clinical presentation of restrictive cardiomyopathy. HES heart disease can also present dilated cardiomyopathy, but myocardial hypertrophy has only rarely been noted in conjunction with HES. This report concerns a patient with HES who had clinical and hemodynamic evidence of asymmetric septal hypertrophy with mid-ventricular obstruction.

Cardiomyopathies↗

PubMed1987

Longitudinal changes in left ventricular architecture and function in peripartum cardiomyopathy.

Left ventricular (LV) function was quantitated in 14 patients with peripartum cardiomyopathy, in 10 using 2-dimensional (2-D) echocardiography and in 4 radionuclide ventriculography, and values were compared with those in 11 normal women in the immediate postpartum period. LV end-diastolic and end-systolic volume indexes, LV wall mass index and ejection fraction were calculated during the acute phase of the illness and serially through long-term follow-up (mean 24 months). During the acute phase of illness there was marked LV dilatation: mean end-diastolic volume index was 95 +/- 22 ml/m2, vs 67 +/- 9 ml/m2 in control subjects (p less than 0.005), and mean end-systolic volume index was 66 +/- 18 ml/m2, compared to 27 +/- 5 ml/m2 in control subjects (p less than 0.001). Mean heart rates and mean systolic pressures in the patients with peripartum cardiomyopathy and the control subjects were similar, 91 +/- 24 vs 79 +/- 14 beats/min and 120 +/- 14 vs 117 +/- 10 mm Hg, respectively. LV wall mass index was higher, 139 +/- 38 vs 96 +/- 8 g/m2 (p less than 0.005), and ejection fraction much lower, 29 +/- 5% vs 67 +/- 5% (p less than 0.001), in control subjects. Five patients underwent endomyocardial biopsy during the acute illness, which showed interstitial fibrosis but no evidence of active inflammation. There was rapid and early improvement in LV function in 13 of 14 patients. Changes in LV volume, mass and ejection fraction all followed an exponential time course during LV remodeling.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1986

[Malignant familial cardiomyopathy with sudden death, typical asymmetric septal hypertrophy and dilated cardiomyopathy-like features].

Twenty-one members of a family were traced for three generations including the propositus, who had typical dilated cardiomyopathy (DCM)-like features. Clinical, radiological, electrocardiographic and echocardiographic examinations were performed for 13 of 16 still living. Five of 21 members died including three of sudden death. Among living 16 members, five had congestive heart failure, greater than grade III of the NYHA, and cardiomegaly on chest radiography in five. Left ventricular hypertrophy, abnormal Q waves, and ST-T changes were observed on electrocardiography in two, five and eight members, respectively. Echocardiography revealed asymmetric septal hypertrophy (ASH) in four, two of whom had systolic anterior motion (SAM) of the mitral valve. Two had features simulating DCM, and five had mitral valve prolapse. Among six patients with ASH or features simulating DCM, four had cardiac catheterization, coronary angiography and endomyocardial biopsy. None had significant coronary stenosis, but all had high left ventricular end-diastolic pressure (LVEDP) (23 +/- 7 mmHg). Endomyocardial biopsy showed moderate myocardial cellular hypertrophy, interstitial myocardial fibrosis, nuclear changes and myocardial disarrangement, with features characteristic of HCM. In this family with malignant HCM, patients had various clinical features including typical HCM, simulated DCM, DCM coexisted with mitral valve prolapse, and sudden death occurred frequently.

Adolescent↗

PubMed1999

Ultrasonic tissue characterization in patients with dilated cardiomyopathy: comparison with findings from right ventricular endomyocardial biopsy.

AIM: The clinical usefulness of integrated backscatter (IB) imaging was compared with right ventricular endomyocardial biopsy for assessing myocardial damage in patients with dilated cardiomyopathy (DCM). METHODS: We examined 15 patients with DCM and 20 healthy controls. In addition to the conventional M-mode echocardiographic parameters, we determined the cyclic variation in IB values (CV-IB) obtained from parasternal short axis views of the left ventricle just under the transducer for both the interventricular septum (IVS) and the left ventricular posterior wall (PW). The per cent fibrosis area (%) and the transverse diameter of myocytes (microm) were measured in right ventricular endomyocardial biopsy specimens by computer image analysis. To analyze the relationship between pathological findings and CV-IB, we divided patients into four subgroups on the basis of the pathological characteristics of endomyocardial biopsy specimens as follows: degeneration dominant group (n = 5), fibrosis dominant group (n = 5), dilated phase hypertrophic cardiomyopathy (n = 2), and mixed type (n = 3). RESULTS: CV-IB in the IVS and the PW was lower in patients with DCM (8.8 +/- 2.9, 8.3 +/- 2.7 dB, respectively) than in normal subjects (14.4 +/- 2.9, 13.6 +/- 2.6 dB, respectively). Biopsy findings showed a mean per cent fibrosis area of 24.0 +/- 12.3%, and a mean myocyte diameter of 14.3 +/- 2.9 microm in patients with DCM. CV-IB was correlated with both of these findings: per cent fibrosis area (r = -0.56 in IVS, r = -0.56 in PW) and myocyte diameter (r = 0.67 in IVS, r = 0.71 in PW). CV-IB was decreased in all DCM subgroups compared with normal subjects, but there was no significant difference between subgroups. CONCLUSIONS: CV-IB was correlated with both the extent of fibrosis in myocardial tissue and the myocyte diameter. These findings suggest that ultrasonic tissue characterization is a good indicator of the severity of fibrosis and myocyte atrophy in patients with DCM.

Biopsy↗

PubMed2005

Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy.

We describe the case of a 17-year-old boy with Becker's muscular dystrophy (BMD) presenting with rapid progression from hypertrophic cardiomyopathy to heart failure within 2 years. Initial echocardiogram showed severe hypertrophy of left ventricle (LV) and right ventricle (RV) with normal chamber size, and preserved LV systolic function. Microscopic study of cardiac muscle obtained by endomyocardial biopsy of the interventricular septum showed severe hypertrophy of the muscle fibers and interstitial fibrosis. Follow-up echocardiogram 2 years after the first examination exhibited marked dilated LV and RV with severe LV global hypokinesia. Follow-up endomyocardial biopsy demonstrated increased interstitial cellular matrix. Immunohistochemical staining for dystrophin revealed significant loss of dystrophin along the sarcoplasmic membrane of the right biceps brachii muscle, compatible with BMD.

Adolescent↗