Autoantibodies against the beta-adrenergic receptor in human myocarditis and dilated cardiomyopathy: beta-adrenergic agonism without desensitization.

The serum of patients with myocarditis and dilated cardiomyopathy contains immunoglobulins capable of causing in vitro acceleration of beating of neonatal rat heart myocytes. This effect is stereoselectively inhibited by (-)-propranolol and is inhibited also by the beta 1-selective adrenergic antagonists, bisoprolol and metoprolol. This effect is not reversed by washing and continues unabated for at least 24 h.

[Quantitative and qualitative findings in endomyocardial biopsies from patients with and without hypertension]. / Quantitative und qualitative Befunde an Endomyokardbioptaten bei Patienten mit und ohne Hypertonie.

Histological and morphometric investigations were made of left-ventricular endomyocardial bioptates which had been obtained from 19 male patients with so-called latent cardiomyopathy as well as from 21 patients with positively verified hypertension and with angiographically excluded coronary artery sclerosis. The patients were additionally divided in two cohorts according to the weight of the left ventricle below and above 170 g. Inflammatory and ischaemic conditions were, above all, histologically recorded from the first group, while microangiopathy was more frequent in the second. Aspects relating to differential diagnosis and possible relationship between microangiopathy and hypertension were discussed in some detail. Endomyocardial biopsy appears to be justified for patients with hypertension (and also for those with so-called latent cardiomyopathy), if other heart diseases are to be ruled out. Subjective perception of hypertension of myocardial cells was compared to measured myocardial cell diameters. Hypertension of myocardial cells was subjectively overestimated in roughly 50% of all cases. Such subjective overestimation did not necessarily lead to histological diagnosis of hypertension. The conclusion was suggested that subjective assessment of hypertension was sufficient for routine checks. No relationship was found to exist between the weight of the left ventricle or presence of hypertension, on the one hand, and diameter of myocardial cells or volume fraction of the interstitium, on the other.

[Submicroscopic indication of pathological cell growth in hypertrophic cardiomyopathy (HCM) in humans]. / Submikroskopische Hinweise auf pathologisches Zellwachstum bei hypertrophischer Kardiomyopathie (HCM) des Menschen.

The increasing application of transvascular endomyocardial biopsy for diagnostic verification of various heart diseases and elucidation of their etiology relies mainly on light microscopical methods. It is shown by hypertrophic cardiomyopathy as an example that also submicroscopical findings may be of value in diagnostic work, prognosis, and investigating research for etiological relationships. Specific morphological markers for idiopathic HCM were not demonstrable; nevertheless, some characteristic alterations such as fibrillary disorder, excessive folding of nuclear-, intercalated disc-, and sarcolemmal membranes, contraction bands, accumulation of mitochondria, glycogen, and lipid droplets, among others as well as sometimes the thickening of vascular walls with its unknown relations to the so-called "small vessel disease" may be helpful when endomyocardial biopsies are examined with the electron microscope too. Though many cytomorphologic findings are similar to those in dilatative cardiomyopathy, this disease probably is not a final state of HCM. In order to prove the actual cause of both heart failures, more intensive investigating research especially at the molecular biological level is required.

Significant X-ray patterns in cardiomyopathy--an approach improving noninvasive diagnosis.

To identify their characteristic patterns, the X-ray dorso-ventral chest images of 118 patients with cardiomyopathy (63--dilated cardiomyopathy, 25--hypertrophic obstructive cardiomyopathy, 30--hypertrophic nonobstructive cardiomyopathy) were analyzed and compared with those of 22 patients without cardiac abnormalities. All 140 patients underwent comprehensive invasive cardiac examinations. In dilated and hypertrophic nonobstructive cardiomyopathy transvasal endomyocardial biopsy was performed. Irrespective of the type and the aetiology of cardiomyopathy, 4 characteristic patterns of X-ray findings could be identified: a class of left heart abnormality (class L) and 3 types of bilateral cardiac abnormalities with various stages of left heart failure (class L + R). An association between the image patterns and haemodynamic parameters (left ventricular end-diastolic pressure, wall mass index, pulmonary mean pressure) was demonstrated which was most obvious in dilated and less evident in hypertrophic obstructive cardiomyopathy. Mitral insufficiency in all types and classes frequently corresponded with the X-ray findings of left atrial enlargement. The X-ray classification method proved to be an efficient approach and superior to the heart-to-lung ratio.

[Differential assessment of myocardial diseases: hemodynamic-angiocardiography studies and etiologic classification]. / Zur differenzierten Beurteilung myokardialer Erkrankungen: Hämodynamisch-angiokardiographische Untersuchungen und ätiologische Zuordnung.

Subject of our study are 293 patients with diseases of the myocardium, 212 males and 81 females at the average age of 45.2 years who were examined invasively including biopsy of the endomyocardium on account of clinical symptoms and changes of the ECG. Patients with hypertension, vitia, coronary stenoses as well as with hypertrophic cardiomyopathy were excluded. The aims of our study were a precised assessment of the degree of severity by a score from invasive parameters, the characterization of so-called latent or beginning cardiomyopathies and their relations to the dilatative cardiomyopathy as well as the aetiological classification by the biopsy of the endomyocardium. The score resulted from the number and the degree of individual pathological parameters, in which case the degree of the deviation was confirmed with points between 0 and 3. By summarizing of the score three degrees of severity were defined, to which clinical, electrocardiographic and bioptic results were coordinated. In the degree of severity 1 were 170 patients, in the degree of severity 2 42 patients, in the degree of severity 3 81 patients. In the higher degree of severity dyspnoea was significantly more frequently stated. In all degrees of severity the decrease of function was nearly of the same frequency. Of the degree of severity 3 died 28 patients, of the degree of severity 2 1 patient. In the degree of severity 1 by the biopsy of the endomyocardium in greater than 80% aetiological clues were detected, only in 35% in the degree of severity 3. Inflammatory changes were relatively frequently observed in all degrees of severity.(ABSTRACT TRUNCATED AT 250 WORDS)

[Comparative analysis of enzyme activities in endomyocardial biopsy samples of patients with various cardiomyopathies]. / Vergleichende Analyse der Enzymaktivitäten in Endomyokardproben von Patienten mit unterschiedlichen Herzmuskelerkrankungen.

41 patients have been investigated using invasive techniques including left ventricular endomyocardial biopsy. One of six specimens per patient was investigated biochemically. 8 pts. (group A) suffered from dilated cardiomyopathy (DCM), 13 (group B) from sarcoidosis of the lungs, 20 (group C) from so-called latent cardiomyopathy (LCM). The histological diagnoses were: normal (3 pts.), small vessel disease (4 pts.), unknown etiology (4 pts.), post myocarditis (6 pts.), residual myocarditis (12 pts.), active myocarditis (5 pts.), suspected toxic myocardial disease (6 pts.), Fabry's disease (1 pt.). The enzyme/isoenzyme activities of malate dehydrogenase (MDH), aspartate amino transferase (ASAT) and lactate dehydrogenase (LDH) were measured and compared in different hemodynamic and histologic groups. The enzyme activities from patients with impaired left ventricular function were increased. Independent from left ventricular function the activities of MDH, ASAT and LDH as well mitochondrial isoenzymes were decreased in active and residual myocarditis. The isoenzyme LDH M was increased significantly in active myocarditis and suspected toxic myocardial disease. The measurements of LDH enzyme/isoenzyme profile in myocardial tissue obtained by endomyocardial biopsy seem to support the histological diagnosis of active myocarditis.

[Roentgenologic data patterns and hemodynamics in biopsy-proven dilated myocardial diseases]. / Röntgenologische Befundmuster und Hämodynamik bei bioptisch gesicherten dilatativen Myokarderkrankungen.

The chest plain images of 63 patients suffering from histologically proved dilated myocardial diseases: primary cardiomyopathy (27), active and residual myocarditis (23), toxic cardiomyopathy (3), endomyocardial fibrosis (2), thesaurismotic cardiomyopathy (2), obstructive intramural fine vessel diseases (6) were analysed and correlated with invasively determined parameters: end-diastolic volume index, wall mass index, left ventricular end-diastolic pressure, pulmonary artery mean pressure, ejection fraction. The x-ray findings proved to occur in four different patterns which were defined as left heart involvement and three different patterns of bilateral left and right heart involvement representing various stages of left heart failure. This kind of image evaluation with functional classification is characterized by increased sensitivity, accuracy and information in detail in comparison with the heart lung index or other usual procedures.

[Diagnostic contribution of endomyocardial biopsy in suspected latent cardiomyopathy]. / Diagnostischer Beitrag der Endomyokardbiopsie bei Verdacht auf latente Kardiomyopathie.

Twenty-eight patients with suspected diagnosis of latent cardiomyopathy are reported. In all patients left ventricular endomyocardial biopsy (five or more specimens) was performed after noninvasive and invasive investigations. The indication to investigate invasively arose from clinical symptoms in combination with uncertain ECG disturbances. Coronary heart disease, latent myocarditis and latent cardiomyopathy were to be excluded. Hemodynamic parameters proved to be normal or borderline at rest. Angiocardiography and coronary angiography revealed no pathological findings. But in all cases the histological results of endomyocardial biopsy were characterized by pathological criteria as follows: inflammatory myocardial process, narrowing or obliterating diseases of intramural small arteries, exogenous-toxic myocardial disturbances, idiopathic cardiomyopathy, endomyocardial fibrosis with endocardial thrombus and infiltrations of eosinophiles. Four cases are demonstrated as examples.

[The importance of echocardiography for the diagnosis of cardiomyopathies in comparison to cardio-computer tomography and to complex invasive heart diagnostics]. / Die Bedeutung der Echokardiographie für die Diagnostik der Kardiomyopathien im Vergleich zur Cardio-Computer-Tomographie und zur komplexen invasiven Herzdiagnostik.

112 patients with various forms of cardiomyopathy (typical-subaortic--hypertrophic obstructive; atypical--medioventricular--hypertrophic obstructive; hypertrophic nonobstructive; apical hypertrophic; dilatative) were subjected to echocardiographic examination of the morphological and functional state of the heart. The results were compared with the results of computer heart tomography and comprehensive invasive examination. The high diagnostic reliability of ultrasonic diagnostics has been confirmed. Invasive examination is necessary in hypertrophic nonobstructive and in dilatative cardiomyopathies. Computer heart tomography is suitable for complementing findings and for clarifying contradictory results of examination, especially in apical forms of cardiomyopathy.

[Hypertrophic cardiomyopathy with midventricular and subvalvular obstruction in both heart ventricles]. / Hypertrophische Kardiomyopathie mit medioventrikulärer und subvalvulärer Obstruktion in beiden Herzkammern.

Typical (subaortic) and atypical (midventricular or apical) obstructions of both ventricles in HOCM are rare events. This combination could be demonstrated in a 21-year-old female patient by heart catheterization and angiocardiography. Simultaneous registration via 2 catheters in each ventricle showed pressure gradients with two steps in both ventricles: RV apical/RV midventricular 70-100 mm Hg, RV midventricular/subvalvular 25 mm Hg; LV apical/LV midventricular 70 mm Hg, LV midventricular/LV subvalvular 30 mm Hg; no valvular pressure gradients. Morphology of the obstructions is demonstrated by angiography: severe bilateral septal hypertrophy with midventricular systolic obstruction, systolic obstruction of right ventricular infundibulum by hypertrophy of the crista supraventricularis, systolic apical separation in the right ventricle; stenosis of the left ventricular outflow tract by SAM, systolic apical separation in the left ventricle by hypertrophy of papillary muscles.