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[A case of sarcoidosis in which sarocoid granulomas were observed only in the heart].

A 64-year-old woman was transferred to the intensive care unit with dyspnea and palpitation on effort. Chest x-ray film showed cardiomegaly and pulmonary congestion. We carefully examined for sarcoidosis as a differential diagnosis of heart failure. Serum lysozyme was mildly high, but human atrial natriuretic peptide (HANP) and brain natriuretic peptide (BNP) were strikingly high. Angiotensin converting enzyme was within normal limit. Chest roentgenogram did not reveal bilateral hilar lymphadenopathy. Atrioventricular conduction block was not observed on electrocardiogram. Echocardiographic examination showed left ventricular global hypokinesis with septal thinning and enlargement. Mitral valve regurgitation was recognized by Doppler evaluation. Coronary arteriography showed normal coronary arteries. Endomyocardial biopsy revealed noncaseous epithelioid granulomas containing, Langhans type giant cell accompanied by fibrosis and lymphocyte infiltration. From these data cardiac sarcoidosis was diagnosed. Gallium scintigraphy showed diffuse uptake only in the heart. Treatment with oral prednisolone 20 mg/day was started. Her symptoms improved by several weeks after the medical treatment. In addition, both the value of HANP and BNP were markedly decreased and echocardiogram showed improvement of cardiac systolic function. In Japan, there is a higher incidence of cardiac sarcoidosis than in the West. The prognosis of this condition associated with cardiac dysfunction is reported to be very poor. When progressive heart failure in older patients is seen, cardiac sarcoidosis should also be kept in mind. Endomyocardial biopsy play an important role as the only accurate technique for the diagnosis of cardiac sarcoidosis.

Cardiomyopathies↗

Spectrum and diagnosis of myocardial rejection.

Right ventricular endomyocardial biopsy has become the mainstay for the diagnosis of acute cardiac rejection. The intelligent inerpretation of endomyocardial biopsy specimens requires knowledge of the artifacts inherent to the procedure as well as specific rejection and nonrejection pathology. Myocardial contraction bands, artifactual tissue spreading, and prior biopsy site changes should not be misinterpreted as evidence of myocyte damage, interstitial edema, or rejection, respectively. The Billingham criteria for acute cardiac rejection (mild, moderate, and severe) are still the most widely utilized, although other schemes for rejection have also shown clinical usefulness. Additionally, there is increasing evidence that some patients may develop a vascular or humoral rejection that may be more difficult to diagnose by endomyocardial biopsy without utilization of special techniques--for example, immunofluorescence. Nonrejection pathology frequently seen post-transplantation includes ischemia or catecholamine effect, interstitial fibrosis, myocardial calcification, cyclosporine-associated endocardial infiltrates (Quilty effect), myocyte hypertrophy, and infections (CMV, toxoplasmosis). Coronary artery disease continues to be the most significant threat to long-term survival. The spectrum of pathologic changes in the vessels range from mild intimal thickening to severe concentric intimal fibrosis involving extramural, as well as intramural, coronaries to lesions virtually identical to native atherosclerosis. Patients with diffuse narrowing involving large and small intramyocardial vessels appear to be at greater risk for myocardial infarction, death, or retransplantation than patients with other types of coronary pathology. Although important, these large vessel changes are rarely identified by endomyocardial biopsy.

Biopsy↗

Relationship between thallium-201 myocardial SPECT and findings of endomyocardial biopsy specimens in dilated cardiomyopathy.

The purpose of this study was to clarify which myocardial histological findings associated with dilated cardiomyopathy (DCM) are reflected in quantitative 201Tl myocardial SPECT. We obtained studied SPECT images from 21 patients with DCM 10 minutes and 2 hours after they received an injection of 111 MBq 201Tl at rest. We calculated the percent coefficient of variation of myocardial 201Tl counts [%CV(TI)], the washout rate (WR), standard deviation of WR [SD(WR)], extent score (ES) and severity score (SS). We used image analysis to measure % fibrosis, % myocytes, the ratio of fibrous tissue to myocyte tissue (F/My), myocyte size and standard deviation of myocyte size [SD(My)] in left ventricular endomyocardial biopsy specimens. The %CV(Tl) was correlated with % fibrosis and F/My. The ES and SS also correlated with F/My. The correlation between SD(WR) and SD(My) was significant. The present findings suggest that %CV(Tl), ES and SS of rest 201Tl SPECT reflect myocardial fibrosis and that the standard deviation of washout reflects the distribution of myocyte size.

Biopsy↗

Peripartum cardiomyopathy: the value of endomyocardial biopsy in diagnosis, prognostication, and therapy.

We describe the case of a 21-year-old black female who was readmitted postpartum because of increasing exertional dyspnea, orthopnea, pleuritic chest pain, and pedal edema. The patient underwent a successful course of clinical treatment for peripartum cardiomyopathy consisting of a regimen of digoxin, diuretics, captopril, and heparin. The results of an endomyocardial biopsy done at readmission were normal: there was no evidence of inflammation, necrosis, or fibrosis; the endocardium, intramural arterioles, mitochondria, and ultrastructure were normal, as was the amount of glycogen; nuclear chromatin were evenly dispersed; and no antibodies were found. Previous studies have shown that approximately half of patients who suffer from peripartum cardiomyopathy recover, while half develop a more severe form of dilated cardiomyopathy. We venture to speculate that normal endomyocardial biopsy findings during the acute stage of the disease may be predictive of recovery. With more certainty, we propose that histologic findings from material taken during an acute episode can and should guide the course of therapy.

Journal Article↗

Endomyocardial biopsy findings in patients with atrioventricular block in the absence of apparent heart disease.

The purpose of this study was to examine the histopathological findings of right ventricular endomyocardial biopsies from ten patients less than 60 years of age (47 +/- 9.8 (mean +/- SD) years) with documented atrioventricular block but without apparent heart disease. They underwent electrophysiological testing, echocardiography, coronary angiography, and right ventricular endomyocardial biopsy. Biopsy specimens were assessed for morphologic changes in myocyte diameter, fibrosis, disarray, and degeneration. Electrophysiological testing demonstrated atrioventricular nodal block in 2, intra-His bundle block in 2, and infra-His bundle block in 6 patients. Histology revealed evidence of myocardial fibrosis with either myocyte hypertrophy or disarray in 7 of the 10 patients. The results of electrophysiological testing did not correlate with the histopathological findings or severity. In one patient, heart failure appeared during the follow-up period. We conclude that patients with atrioventricular block of unknown etiology have histological abnormalities of the ventricular endomyocardium in addition to the conduction system disturbances. We consider such cases as constituting one of the disease groups of cardiomyopathy, and suggest that it is necessary to follow up the clinical course in these patients.

Adult↗

Decreased expression of tumor necrosis factor-alpha and regression of hypertrophy after nonsurgical septal reduction therapy for patients with hypertrophic obstructive cardiomyopathy.

BACKGROUND: Nonsurgical septal reduction therapy (NSRT) is a novel therapeutic strategy for patients with hypertrophic obstructive cardiomyopathy (HOCM). Although the clinical benefits of this technique appear to be clear, the structural and functional changes that lead to improvements in cardiac function are not completely defined. In these studies, we sought to define the effect of NSRT on myocardial function as well as various markers of hypertrophy including the expression of tumor necrosis factor (TNF)-alpha, a cytokine capable of producing fibrosis, left ventricular hypertrophy (LVH), and cardiomyopathy. METHODS AND RESULTS: We performed endomyocardial biopsies of the RV side of the septum and echocardiograms on 15 HOCM patients at baseline and after successful NSRT. Comparative analysis on paired myocardial samples were performed to determine the effects of NSRT on LVH, end-diastolic volume and chamber stiffness, myocyte size, collagen content, and TNF-alpha levels. At baseline, myocardial TNF-alpha levels were increased in all patients. After NSRT, myocyte size, collagen content, and TNF-alpha were significantly decreased. These changes were accompanied by an increase in left ventricular volumes and a reduction in LVH and chamber stiffness. CONCLUSIONS: We suggest that pressure overload in HOCM patients contributes to the development of hypertrophy. These data provide the initial experimental evidence to suggest that TNF-alpha may play a pathogenetic role in the hypertrophy of pressure overload.

Adult↗

[A case of right ventricular dilated cardiomyopathy, which involved left ventricle].

A case of right ventricular dilated cardiomyopathy which also involved the left ventricle was reported. On health screening, a 16-year old woman was pointed out to have multifocal PVC and cardiomegaly. Subsequently, she was admitted to our hospital because of general fatigue. CTR was enlarged to 54.9% on chest X-ray. ECG showed LBBB-type PVC, right axis deviation, low voltage and T wave changes. On UCG, RVdD was dilated to 40 mm and LVdD was 37 mm. There was no finding of abnormality of the tricuspid valve. On cardiac catheterization, there was no shunt disease. Intracardiac pressure was normal. The end-diastolic volume index (ml/m2) of RV and LV was 196.7 and 67.4, respectively. And ejection fraction (%) was 20 and 40. Ventriculography revealed diffuse dilatation of the right ventricle. And lowered contractility existed not only in the right ventricle but also in the anterior and apical segment of the left ventricle. T(1)201 myocardial perfusion imaging showed irregular perfusion defect of the left ventricle. Endomyocardial biopsy revealed marked hypertrophy, partial atrophy, disarrangement of myocyte and interstitial fibrosis of the right ventricle. This case was considered to be right ventricular dilated cardiomyopathy. It seemed to be an intermediate form of dilated cardiomyopathy since it also involved the left ventricle. It was an interesting case to illustrate the spectrum of expression of cardiomyopathy.

Adolescent↗

[Acute latent myocarditis appearing with ventricular fibrillation after intranasal administration of adrenaline. Description of a case].

We report a case of a 17 year old sports woman, who had a ventricular fibrillation episode with cardiogenic shock during endonasal anaesthesia containing epinephrine. She was so transferred to our department (in shock): the 2-D echo showed biventricular hypokinesia without dilatation (LVEF less than 25%). Endomyocardial biopsy performed 5 days later showed active lymphocyte myocarditis with interstitial fibrosis. There were serum antibodies anti-Echo 9 and Coxsackie B 1, 2, 3. Immunoassay, urinary catecholamines and glucagon test were normal. The clinical picture was resolved within 15 days using intravenous isoprenaline and/or dopamine initially followed by oral diuretics and digoxin; the therapy was broke off at the time of discharge. We believe that the vasoconstriction and/or the oxygen wasting effect caused by epinephrine revealed latent myocarditis which had not been shown up even by intensive physical training.

Adolescent↗

Reversal of oxalosis cardiomyopathy after combined liver and kidney transplantation.

Few data have been published on the course of oxalosis cardiomyopathy after combined liver and kidney transplantation in hyperoxaluria patients with myocardial involvement. We report the case of a primary hyperoxaluria type 1 patient with renal failure who developed end-stage cardiomyopathy. Left venticulography showed severe diffuse hypokinesia and left ventricular ejection fraction was calculated at 12%. Endomyocardial biopsy demonstrated platelike calcium oxalate crystals within the myocardium and the connective tissue, and mild perivascular fibrosis. The patient was first considered for combined liver-heart-kidney transplantation, but as his cardiac function improved slightly with an intensive dialysis program, combined liver and kidney transplantation was performed. Normal cardiac function was demonstrated at 1-year follow-up, and comparative endomyocardial biopsy showed regression of the myocardial oxalate deposits. This case adds stronger clinical, hemodynamic, and histopathological evidence that severe oxalosis cardiomyopathy may be reversed after combined liver and kidney transplantation.

Adult↗

Endomyocardial biopsy foretells ventricular function recovery after coronary artery bypass grafting.

OBJECTIVE: Patients with ischemic heart failure may benefit from coronary artery bypass grafting. The histopathological variables associated with improvement in ejection fraction 6 months after surgery were assessed. METHODS: This study comprised 24 patients indicated for coronary artery bypass grafting, ejection fraction < 35%, functional class II-IV heart failure, and mean age 59 +/- 9 years. Endomyocardial biopsies were performed during and 6 months after surgery. Extension of the fibrosis, number of cells with myocytolysis, and hypertrophy of the muscle fiber were quantified by using a system of image analysis. Clinical and functional review was repeated within 6 months. RESULTS: A significant improvement in heart failure functional class was observed in 16 patients after 6 months of follow-up (from NYHA functional class 2.8 +/- 0.7 to 1.7 +/- 0.6; P < 0.001), but the ejection fraction did not change (25 +/- 6 % vs. 26 +/- 10%). Hypertrophy of the muscle fiber was similar in the specimens biopsied in the pre- and postoperative periods (21 +/- 4 vs. 22 +/- 4 microm), but the extension of fibrosis (8 +/- 8 vs. 21 +/- 15% area) and the number of cells with myocytolysis (9 +/- 11 vs. 21 +/- 15% cell) significantly increased. However, the composition of a histological score combining those 3 variables indicated a greater increase in the ventricular function of those with a lower degree of preoperative histopathological alterations. CONCLUSION: Patients with ischemic cardiomyopathy undergoing coronary artery bypass grafting improved their ventricular function when the preoperative adverse histopathological alterations were of a lower degree.

Adult↗

[Early diastolic dysfunction of the left ventricle and its relation to histopathological findings in patients with diabetes mellitus].

The relation of left ventricular diastolic function and the histopathological findings of the myocardium in patients with diabetes mellitus were observed using echocardiography and endomyocardial biopsy. The subjects consisted of six diabetic patients (mean age 49.3 years) and eight normal control subjects (mean age 44.8 years). Coronary angiography had no significant stenotic lesions in their coronary arteries. Their diabetic complications were mild to moderate in severity. Echoes from the left ventricular margin of the septum and from the posterior left ventricular wall were traced on a digitizing board; then the isovolumic relaxation period, rapid filling period, slow filling period and atrial contraction period were determined to calculate fractional shortening (FS), isovolumic relaxation time (IRT), and three filling volumes (RFV, SFV and ACV). The quotients of the left ventricular filling volume and stroke volume were also determined. Right ventricular endomyocardial biopsies were performed to determine the diameters of myocytes, the percentage of fibrosis and the eccentricity e, as a parameter of the degree of myocardial dysarrangement. The results were as follows: IRT was significantly longer and RFV/SV was significantly greater in patients with diabetes mellitus than those among the controls. Also the diameters of myocytes and the percentage of fibrosis were significantly greater, while the eccentricity e was less compared to that of the controls. There were no significant correlations, among IRT, RFV/SV, the diameters of myocytes, and eccentricity e, but the percentage of fibrosis significantly correlated with IRT and RFV/SV (r = 0.62, r = -0.63). IRT and RFV/SV were mainly responsible for the percentage of fibrosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Functional and structural abnormalities in patients with dilated cardiomyopathy.

Passive diastolic properties of the left ventricle were determined in 10 control subjects and 12 patients with dilated cardiomyopathy. Simultaneous left ventricular angiography and high fidelity pressure measurements were performed in all patients. Left ventricular chamber stiffness was calculated from left ventricular pressure-volume and myocardial stiffness from left ventricular stress-strain relations with use of a viscoelastic model. Patients with dilated cardiomyopathy were classified into two groups according to the diastolic constant of myocardial stiffness (beta). Group 1 consisted of seven patients with a normal constant of myocardial stiffness less than or equal to 9.6 (normal range 2.2 to 9.6) and group 2 of 5 patients with a beta greater than 9.6. Structural abnormalities (percent interstitial fibrosis, fibrous content) in patients with dilated cardiomyopathy were assessed by morphometry from right ventricular endomyocardial biopsies. Heart rate was similar in the three groups. Left ventricular end-diastolic pressure was significantly greater in patients with cardiomyopathy (18 mm Hg in group 1 and 22 mm Hg in group 2) than in the control patients (10 mm Hg). Left ventricular ejection fraction was significantly lower in groups 1 (37%) and 2 (36%) than in the control patients (66%). Left ventricular muscle mass index was significantly increased in both groups with cardiomyopathy. The constant of chamber stiffness (beta*) was slightly although not significantly greater in groups 1 and 2 (0.58 and 0.58, respectively) than in the control group (0.35). The constant of myocardial stiffness beta was normal in group 1 (7.0; control group 6.9, p = NS) but was significantly increased in group 2 (23.5). Interstitial fibrosis was 19% in group 1 and 43% (p less than 0.001) in group 2 (normal less than or equal to 10%). There was an exponential relation between both diastolic constant of myocardial stiffness (beta) and interstitial fibrosis (IF) (r = 0.95; p less than 0.001) and beta and fibrous content divided by end-diastolic volume index (r = 0.93; p less than 0.001). It is concluded that myocardial stiffness can be normal in patients with dilated cardiomyopathy despite severely depressed systolic function. Structural alterations of the myocardium with increased amounts of fibrous tissues are probably responsible for the observed changes in passive elastic properties of the myocardium in patients with dilated cardiomyopathy. The constant of myocardial stiffness (beta) helps to identify patients with severe structural alterations (group 2), representing possibly a more advanced stage of the disease.

Adult↗

Histologic evidence of myocardial damage in apparently healthy subjects with ventricular arrhythmias and myocardial dysfunction.

The association of ventricular arrhythmias and myocardial dysfunction could be considered an early step toward cardiomyopathy; therefore, we studied 28 patients in NYHA class I and II, characterized by complex ventricular arrhythmias (VA) on 24-h Holter monitoring and volumetric and/or contractile abnormalities on a standard two-dimensional echocardiogram (2-D echo). All patients underwent radioisotopic angiography, 20 patients complete hemodynamic study, and 15 patients endomyocardial biopsy. Ambulatory ECG monitoring showed the presence of frequent premature ventricular contractions in 14 patients (50%) and episodes of ventricular tachycardia in 16 patients (57%). 2-D echo showed mono- or biventricular enlargement and dyssynergies in 25 patients (89%) (left ventricle in 6, right ventricle in 11, both in 8). Two patients showed only left ventricle enlargement and one patient isolated left ventricular dyssynergies. Radioisotopic angiography showed mono- or biventricular ejection fraction reduction in 24 patients (85%) and regional dyssynergies in 24 patients (85%) in accordance with 2-D echo. Hemodynamic study showed in all patients normal coronary arteries, and right and left angiography confirmed enlargement and/or regional dyssynergies. Endomyocardial biopsy was abnormal in 11 of 15 patients: various degrees of hypertrophy, parcellar fibrosis, and adipogenic infiltration were found. Our preliminary data suggest that the simultaneous occurrence of ventricular arrhythmias and ventricular dyssynergies and/or enlargement in patients without apparent clinical heart disease may represent an early stage of dilated cardiomyopathy.

Adolescent↗

Ischemic cardiomyopathy: endomyocardial biopsy and ventriculographic evaluation of patients with congestive heart failure, dilated cardiomyopathy and coronary artery disease.

OBJECTIVES: This study was designed to define clinical and pathophysiologic similarities and differences between patients with ischemic and idiopathic dilated cardiomyopathy. BACKGROUND: Significant coronary artery disease in patients with new onset congestive heart failure due to dilated cardiomyopathy has important prognostic and therapeutic implications. METHODS: Clinical, histologic, ventriculographic and hemodynamic features of patients with dilated cardiomyopathy who underwent coronary angiography were reviewed. RESULTS: Patients with ischemic cardiomyopathy (n = 21) compared with those with idiopathic cardiomyopathy (n = 40) had similar presenting symptoms, durations of illness, and coronary risk factor profiles, with the exception of a greater prevalence of cigarette smoking (71% vs. 39%, p = 0.028) and male gender (100% vs. 70%, p = 0.014). Endomyocardial biopsy specimens from patients with ischemic cardiomyopathy demonstrated a greater prevalence of replacement fibrosis (48% vs. 8%, p = 0.001) and a lesser degree of histologically assessed myocyte hypertrophy (mean grade 0.5 +/- 0.7 vs. 1.3 +/- 1.3, p = 0.015). Although ventriculographically determined regional dyskinesia was present in both groups, there was a higher prevalence of two or more adjacent segments in the ischemic cardiomyopathy group (50% vs. 10%, p = 0.03). This ischemic group had hemodynamic variables associated with a worse prognosis: higher pulmonary artery wedge pressure (23 +/- 10 vs. 15 +/- 9 mm Hg, p = 0.006) and lower cardiac index (2.0 +/- 0.5 vs. 2.3 +/- 0.5 liters/min per m2, p = 0.044). Also, in this group, patients had a mean of 2.6 +/- 0.7 diseased vessels; 15 (71%) of 21 patients had triple-vessel disease and 18 (86%) of 21 had at least one occluded or suboccluded artery. CONCLUSIONS: 1) Patients with ischemic and idiopathic cardiomyopathy may be clinically indistinguishable unless coronary angiography is performed. 2) A greater prevalence of replacement fibrosis and a lesser degree of myocardial hypertrophy in patients with ischemic cardiomyopathy may account for the greater extent of hemodynamic decompensation observed at presentation.

Biopsy↗

[Hypertrophic non-obstructive cardiomyopathy assessed by dipyridamole thallium single photon emission computed tomography: comparisons with hypertrophic cardiomyopathy with a dilated heart].

Thallium-201 (Tl) single photon emission computed tomography (SPECT) after dipyridamole infusion (0.56 mg/kg) was performed in 23 patients with hypertrophic non-obstructive cardiomyopathy (HNCM) and in seven patients with HCM simulating dilated cardiomyopathy (HCM-DCM) to clarify the mechanism and clinical significance of decreased coronary vasodilatory reserve. The coronary vasodilatory reserve in the hypertrophied area assessed by SPECT was compared with the findings of echocardiography, left ventriculography and endomyocardial biopsy. 1. Eleven patients with HNCM had no perfusion defects in the hypertrophied area (group I), but the other 12 patients (52.2%) had such defects (group II). All seven patients with HCM-DCM had perfusion defects in the anterior or septal walls (group III). Redistribution was observed in 11 of the 12 patients in group II and in three of the seven patients in group III. 2. The regional washout rate was relatively low in the upper septum in group II and in the anterior wall and upper septum in group III. Thus, coronary vasodilatory reserve in the hypertrophied area was decreased in groups II and III. 3. Echocardiographically, the degree of hypertrophy did not differ between groups I and II, but the latter had significantly greater left ventricular diastolic dimension (42.3 mm vs 49.5 mm: p less than 0.05) and lower percent fractional shortening (%FS) (43.7% vs 35.6%: p less than 0.05). However, group III showed thinner left ventricular wall, much greater diastolic dimension (60.9 mm vs 49.5 mm: p less than 0.05), and lower %FS (24.0% vs 35.6%: p less than 0.05) than did those of group II. 4. On left ventriculography, those in group II showed larger left ventricular end-diastolic volume index (93.9 ml/m2 vs 79.7 ml/m2: p less than 0.05) than that of group I. Left ventricular ejection fraction showed the same tendency, but this was not statistically significant. 5. On endomyocardial biopsy, the specimens of the patients in group II had significantly higher percent fibrosis than did those of group I (11.4% vs 6.8%: p less than 0.05). These findings suggest that the mechanism of decreased coronary vasodilatory reserve in the hypertrophied area may be related to myocardial fibrosis, and this decrease may induce left ventricular dysfunction and compensatory dilatation.

Adult↗

The myocardial fibrosis in patients with dilated cardiomyopathy. The application of image analysis in the myocardial biopsies.

BACKGROUND: The aim of our study is to investigate whether myocardial fibrosis measured by image analysis may be considered as an important and accurate index of dilated cardiomyopathy and its prognosis. METHODS: The study group consisted of 24 patients with dilated cardiomyopathy, which was diagnosed by echocardiography, radionuclide ventriculography, cardiac catheterization, and left ventricular endomyocardial biopsy. The patients' overall disability was conventionally expressed with the criteria for functional capacity. Using image analysis, the percentage of fibrosis in a total of 35 myocardial biopsies was accurately measured, followed by a study comparing the percentage of myocardial fibrosis and the clinical parameters (left ventricular ejection fraction and overall functional capacity), showing the degree of each patient's heart failure. RESULTS: A correlation was found among fibrosis, left ventricular ejection fraction, and overall functional capacity. The cases with small values of fibrosis (< 10%) have big values of ejection fraction and belong to Class I of overall functional capacity. The cases with big values of fibrosis (> 10%) belong to Classes III and IV of overall functional capacity and have small values of ejection fraction. The results of the comparative study were graphically presented and considered significant. CONCLUSION: Myocardial fibrosis measured by image analysis might be considered an important prognostic index of dilated cardiomyopathy.

Adult↗

Rapid histological changes in endomyocardial biopsy specimens after myocarditis.

The course and response to treatment in acute lymphocytic myocarditis are conventionally monitored by endomyocardial biopsy performed every 3-12 weeks. A patient with a short history (five days) of acute myopericarditis of unknown aetiology presented in cardiogenic shock with evidence of severe systolic dysfunction on the echocardiogram. The initial biopsy specimen showed histologically unequivocal myocarditis. Repeat endomyocardial biopsy after four days of treatment with steroids and azathioprine showed substantial histological improvement, a reduction in cellular infiltrate and myocardial necrosis, and interstitial fibrosis. Serial biopsies at 2 weeks and then 1, 2, 4, 5, 8, and 14 months after the initial biopsy showed progressive clearing of cellular infiltrate, increasing interstitial fibrosis, and compensatory myocyte hypertrophy by 4 months. At 14 months scattered lymphocytes persisted but myocyte abnormalities had resolved completely. The patient remained symptom free and systolic function was normal during this recovery period. Early endomyocardial biopsy (within one week of diagnosis) may yield useful histological information on the response to treatment in patients with myocarditis. It may not be necessary to wait the customary 3-4 weeks to repeat the biopsy. This case shows the chronology of histological changes and emphasises that a return to normal myocardial function may precede resolution of the histological abnormalities, which may persist in part or may resolve totally after the acute episode.

Acute Disease↗

A case of pediatric cardiomyopathy with severely restrictive physiology.

A rare case of a 6-year-old male with idiopathic familial cardiomyopathy manifesting severely restrictive physiology is reported. The patient showed congestive heart failure with dilatation of both atria with a normal ventricular cavity. A square-root configuration was revealed in the ventricular pressure tracings. His elder brother had died of hypertrophic cardiomyopathy at the age of 3 years. Endomyocardial biopsy disclosed marked disorganization of muscle bundles with hypertrophy of the myocytes and interstitial fibrosis. The patient died suddenly during hospitalization. Autopsy revealed diffuse hypertrophy of both the ventricular walls and the ventricular septum with extensive myocardial disorganization and interstitial fibrosis. These advanced myopathic changes in the myocardium may have been related to the restrictive physiology in this case.

Bundle-Branch Block↗