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Cardiac amyloidosis is a rare cause of cardiomyopathy, however, it is part of an underdiagnosed underlying systemic disease. For transthyretin (ATTR) amyloidosis, which is caused by the deposition of incorrectly folded transthyretin, either as the wild-type (wtATTR) or mutated (mATTR) form, novel evidence-based treatment options were recently shown to reduce disease progression as well as hospitalisation rates for heart failure. Thus, it is important to establish early and reliable diagnosis of cardiac involvement with ATTR amyloidosis.Modern non-invasive imaging (cardio magnetic resonance (CMR) and scintigraphic methods) together with immune fixation with determination of free light chains allow fast and reliable clinical screening for cardiac amyloidosis, whereas endomyocardial biopsy and genetics are used for confirmation of the underlying diagnosis. Interdisciplinary teams including hemato-oncology, neurology, nephrology in addition to cardiology are essential to enable personalized targeted treatment strategies., Competing Interests: Birgit Aßmus hat in den vergangenen 3 Jahren Fortbildungs- und Reisekostenunterstützung von Alnylam erhalten. Korinna Feldmann und Christian W. Hamm geben keine Interessenskonflikte an., (© Georg Thieme Verlag KG Stuttgart · New York.)

Current pandemic caused by SARS-CoV-2 inducing viral COVID-19 pneumonia, is categorized in 3 stages. Some biomarkers could be assigned to one of these stages, showing a correlation to mortality in COVID-19 patients. Laboratory findings in COVID-19, especially when serially evaluated, may represent individual disease severity and prognosis. These may help planning and controlling therapeutic interventions. Biomarkers for myocardial injury (high sensitive cardiac troponin, hsTn) or hemodynamic stress (NTproBNP) may occur in COVID-19 pneumonia such as in other pneumonias, correlating with severity and prognosis of the underlying disease. In hospitalized COVID-19 patients' mild increases of hsTn or NTproBNP may be explained by cardiovascular comorbidities and direct or indirect cardiac damage or stress caused by or during COVID-19 pneumonia. In case of suspected NSTE-ACS and COVID-19, indications for echocardiography or reperfusion strategy should be carefully considered against the risk of contamination., Competing Interests: E. Giannitsis: Honorare für Vorträge von Astra Zeneca, Bayer Vital, Boehringer Ingelheim, Roche Diagnostics, Brahms Deutschland und Daiichi Sankyo. Forschungsmittel von Daiichi Sankyo, Roche Diagnostics und durch die Deutsche Herzstiftung e. V. Beraterhonorare von Roche Diagnostics, Brahms Deutschland, Boehringer Ingelheim.C. Salbach: keine., (© Georg Thieme Verlag KG Stuttgart · New York.)

The joint position paper of the working community "Cardiovascular Nuclear Medicine" of the German Society of Nuclear Medicine (DGN) and the working group "Nuclear Cardiology Diagnostics" of the German Cardiac Society (DKG) updates the former 2009 paper. It is the purpose of this paper to provide an overview about the application fields, the state-of-the-art and the current value of nuclear cardiology imaging. The topics covered are chronic coronary artery disease, including viability imaging, furthermore cardiomyopathies, infective endocarditis, cardiac sarcoidosis and amyloidosis., Competing Interests: Die Interessenkonflikte der Autoren sind online deklariert unter: https://leitlinien.dgk.org/, (Georg Thieme Verlag.)

We describe diagnosis, differential diagnosis, multimodality imaging and medical and invasive diagnostic treatment in patients with inflammatory cardiomyopathy and myocarditis under etiological considerations in reference to a landmark position paper of the Working Group Myocardial and Pericardial Diseases of the European Society of Cardiology together with recent developments in diagnosis and treatment. Diagnosis of the symptomatic patient is the assessment of etiology of inflammatory cardiomyopathy, followed by the clinical presentation, course, treatment option and prognosis. Viral myocarditis in its different facets can clearly be separated from autoreactive forms by histological and molecular methods in the endomyocardial biopsy, thus leading to an individualized targeted therapy beyond heart failure treatment.

This manuscript gives a comprehensive overview with regard to cardiac imaging techniques for work-up and management of patients with myocarditis or cardiomyopathies., (© Georg Thieme Verlag KG Stuttgart · New York.)

Myocarditis is an inflammatory disease of the heart muscle commonly caused by viral pathogens. Dilated cardiomyopathy is a major long-term sequela of myocarditis and at least in part related to post-viral immune-mediated responses. Establishing a diagnosis of myocarditis represents a major challenge because of the variable clinical picture and the lack of readily available, non-invasive diagnostic tests. In recent years, cardiac magnetic resonance imaging (cMRI) has emerged as a promising additional diagnostic tool in patients with suspected myocarditis: cMRI not only provides important insights into structural and functional abnormalities of the heart but relevant tissue pathologies can also be visualized. The diagnostic accuracy of three tissue criteria, i.e. the edema ratio, early gadolinium enhancement ratio and late gadolinium enhancement, has been characterized in several studies. Endomyocardial biopsy (EMB) is widely considered to be the reference standard for diagnosis of myocarditis. Although limited by sampling error, EMB is the only diagnostic procedure that can be used to confirm myocarditis. Laboratory analyses of EMB may provide information about specific causes of myocarditis and are, at least in part, of prognostic relevance. In a subset of patients the results of EMB may guide therapeutic decision-making. Additional efforts are needed in cardiac imaging, molecular characterization of EMB and evaluation of serum biomarkers to improve the diagnostic work-up in patients with suspected myocarditis and to identify potential novel targets for a cause-specific therapy of myocarditis.

We report the case of a 52 years old patient with dilated cardiomyopathy. Ischemic heart disease was excluded by coronary angiography. However, bilateral hilar lymphadenopathy was present on plain chest radiography. Transbronchial biopsy showed non-necrotizing, epitheloid cell granulomas and thus sarcoidosis was diagnosed. A MRI of the heart confirmed the cardiac involvement by the sarcoidosis with corresponding structural changes.

History and Admission Findings: A 27-year-old man presented with acute dyspnea and a previous respiratory tract infection with progressive dyspnoea and chest pain over 2 weeks. Clinical findings revealed severe cardiac failure with development of cardiogenic shock and need for adrenergic drug therapy for circulatory support., Investigations: The electrocardiogram showed a sinus tachycardia with unspecific T-wave-inversions, the echocardiogram revealed a dilated left ventricle and severely reduced systolic LV-function. An acute coronary syndrome could be excluded by coronary angiogram., Treatment and Course: A myocardial biopsy was taken to exclude giant cell myocarditis. The immediate initiation of a mechanical circulatory support by an Extracorporal Membrane Oxygenator (ECMO) facilitated rapid hemodynamic stabilization and recovery of organ function., Conclusions: A drug-only circulatory support very often does not enable stabilization of a patient in progressive cardiogenic shock and cannot prevent multiorgan dysfunction. Therefore implantation of an assist device facilitates a "bridging-to-recovery" or a "bridging-to transplant" concept in critically ill patients presenting with cardiogenic shock. The bridging also allows for reviewing etiology and evaluation of further treatment options. In case of recovery continuous care in a specialized Heart Failure Clinic can help to maintain the clinical status and offer frequent reevaluation of cardiac status and therapeutic concepts., (© Georg Thieme Verlag KG Stuttgart · New York.)

Intoxication with the beta2-agonist Albuterol may lead to immediate signs of beta-adrenergic stimulation like excitation, tachypnea and tachycardia. Furthermore, it typically causes severe hypokalemia, which then leads to muscle weakness and which predisposes to ventricular arrhythmias. We describe a dog where albuterol intoxication caused runs of fast paroxysmal ventricular tachycardia that persisted after normalization of the hypokalemia. Based on a markedly elevated serum troponin I level acute myocardial damage was identified as cause of the tachyarrhythmia. Repeated Troponin I measurements and Holter-ECGs were the means to document complete cure.

Distinct examinations and resulting therapies are depending on the cardiomyopathy phenotype and the severity of the myocardial compromise. Specific treatment modalities for distinct cardiomyopathies, demand detailed diagnostic procedures in addition to the routine diagnostic workup. Quality analyses for causal treatment strategies of different cardiomyopathies are missing. Most diagnostic procedures are unable to detect the relevant pathophysiological changes that generally occur at the cellular and molecular level of myocardial tissue or individual cells. Therefore, often only additional biopsy-based tissue analyses can provide the necessary basis for causal treatment options., (Georg Thieme Verlag KG Stuttgart, New York.)

The diagnosis "muscular dystrophy" without analysis of the underlying gene defect is nowadays obsolete. With the discovery and cloning of cytoskeleton proteins and intermediate filaments in the muscle fiber membrane, the sarcoplasm and the nucleus which are essential for the normal muscle fiber function, the classification of muscular dystrophies has dramatically improved. Muscular dystrophies are a group of clinically and genetically heterogeneous disorders. By means of immunohistochemistry and molecular genetics more than 40 different disease forms can be distinguished, which are characterised by distinct protein defects or defined gene loci and can be related to typical phenotypes. It is noteworthy that muscular dystrophies may be associated with cardiomyopathy with increased risk of sudden cardiac death. Thus, diagnosis and treatment require experienced investigators and clinicians and regular cardiologic follow-ups, preferably in a specialised muscle center.

Cardiac amyloidoses are a heterogeneous group of cardiomyopathies that are resistant to treatment and are associated with a poor outcome. Standard heart failure treatment is usually not well tolerated and the underlying disease remains unaffected. The clinical picture is uncharacteristic. Cardiac amyloidosis is often associated with dysfunction of additional organs. Early cardiac amyloid involvement usually reveals left ventricular hypertrophy, impairment of longitudinal shortening and diastolic ventricular function. Without adequate therapy (bi-)ventricular hypertrophy will progress to severe systolic ventricular function decrease. The combination of low voltage pattern, left ventricular hypertrophy and granular sparkling is characteristic for advanced cardiac amyloid involvement. Cardiac magnetic resonance imaging and scintigraphy yield further information on the pattern and severity of cardiac involvement. In unclear cases (left ventricular) endomyocardial biopsy is necessary. Detection of early cardiac involvement and proper identification of patients at high risk for sudden cardiac death due to rapid progressive amyloidosis is still incompletely defined. Referral to specialized centers is strongly recommended.

The first classifications of cardiomyopathies from 1980 and 1996 described them as heart muscle diseases, with dilated (DCM), hypertrophic (HCM), restrictive (RCM), arrhythmogenic right ventricular (ARVC), and nonclassifiable cardiomyopathies. Furthermore, the World Health Organization/International Society and Federation of Cardiology (WHO/ISFC) classification from 1996 listed among the specific cardiomyopathies inflammatory cardiomyopathy as a new and distinct entity, which was defined histologically as myocarditis in association with cardiac dysfunction. Infectious and autoimmune forms of inflammatory cardiomyopathy were recognized. Viral cardiomyopathy was defined as viral persistence in a dilated heart without ongoing inflammation. If it was accompanied by myocardial inflammation, it was termed inflammatory viral cardiomyopathy (or viral myocarditis with cardiomegaly). This entity was further elucidated in a World Heart Federation consensus meeting in 1999 by quantitative immunohistological criteria (< 14 infiltrating cells/mm(2)) and the etiology by molecular biological methods, e.g., polymerase chain reaction, as viral, bacterial, or autoimmune (= nonmicrobial). The development of molecular genetics, with the discovery of a genetic background in several forms of cardiomyopathies previously alluded to as "of unknown origin", was the origin of a debate on a new classification based on genomics. A genomic/postgenomic classification was postulated taking the underlying gene mutations and the cellular level of expression of encoded proteins into account, thus distinguishing cytoskeleton (cytoskeletalopathies, e.g., DCM or ARVC), sarcomeric (sarcomyopathies as in HCM and RCM) and ion channel (channelopathies, e.g., long or short QT syndrome and Brugada's syndrome) cardiomyopathies. Such a classification of cardiomyopathies was proposed in 2006 by the American Heart Association (AHA), which took the rapid evolution of molecular genetics in cardiology into account. It also introduced several recently described diseases, and is unique in that it incorporated ion channelopathies even without hemodynamic dysfunction as a "primary" cardiomyopathy. The ESC (European Society of Cardiology) Working Group on Myocardial and Pericardial Diseases has deliberately taken a different approach based on a clinically oriented classification in which heart muscle disorders were grouped according to morphology and function. This obviously remains the clinically most useful approach for the diagnosis and management of patients and families with heart muscle disease. In the ESC position statement published in 2008, cardiomyopathies were defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal, and in which coronary artery disease, hypertension, valvular and congenital heart disease are absent or do not sufficiently explain the observed myocardial abnormality. The aim was to help clinicians look beyond generic diagnostic labels in order to reach more specific diagnoses. In parallel, a scientific statement on the role of endomyocardial biopsy in the management of cardiovascular disease was published at the end of 2007 making useful recommendations for clinical practice and providing an understanding for the use of endomyocardial biopsy in an individual patient. Taking the classification of cardiomyopathies and the statement on the role of endomyocardial biopsies in different clinical scenarios together, the clinician is now able to identify genetic, autoimmune and viral causative factors by using a thorough and logical approach to reach a diagnosis in patients with familial and nonfamilial forms of the underlying structural heart muscle diseases.

Molecular biological methods have confirmed the pathogenetic role of enteroviruses, primarily coxsackieviruses of group B (CVB), in the induction and maintenance of inflammatory cardiomyopathy. More recently, adenoviruses, various herpes viruses, and increasingly parvovirus B19 (B19) have been identified as potential cardiotropic agents. While cardiac myocytes are target cells for enterovirus and adenovirus infections with virus-induced cytolysis, B19-associated inflammatory cardiomyopathy is characterized by infection of intracardiac endothelial cells of small arterioles and veins, which may be associated with endothelial dysfunction, impairment of myocardial microcirculation, penetration of inflammatory cells, and secondary myocyte necrosis. Recent observations showed that B19 is involved in intracellular calcium regulation by the viral phospholipase. B19-induced caspase activation can lead to proinflammatory/proapoptotic processes through dysregulation of STAT signaling. These cellular interactions may contribute to mechanisms by which B19 establishes persistent infection in endothelial cells and play a critical role in viral pathogenesis of inflammatory cardiomyopathy.

Purpose: Since limited data is available using MR imaging in cardiac amyloidosis, the purpose of our study was to evaluate morphological and functional differences of the heart using cardiac MRI., Materials and Methods: 19 consecutive patients (14 males, 5 females, mean age 59 +/- 6 years) with histologically proven cardiac amyloidosis were evaluated with MRI at 1.5 T. Results were compared with data of 10 healthy, age-matched control subjects (5 males, 5 females, mean age 60 +/- 6 years). Functional and morphological data including late enhancement (LE) was acquired., Results: Compared to the control group, patients with cardiac amyloidosis had thickened atrial walls and dilated atriums. Both ventricles and the interventricular septum were thickened. The LV hypertrophy was focal in 11 / 19 (58 %) and global in 4 / 19 (21 %) of patients. A myocardial edema occurred in 2 / 19 patients with cardiac amyloidosis (11 %). An edema of the myocardium was visible in 2 / 19 (11 %) of patients. The LV ejection fraction was statistically significantly decreased. The prevalence of LE was 74 % (14 / 19 of patients). LE was detected predominantly in the LV anterior wall and in the interventricular septum. Within the segments LE was located predominantly in a subendocardial location. Between patients with and without LE no statistically significant differences of functional and morphological results were able to be established., Conclusion: There are three major outcomes of our assessment: 1. The LV hypertrophy is focal in the majority of patients with cardiac amyloidosis. 2. No statistically significant differences can be established in regard to the functional and morphological features between patients with and without LE. 3. Myocardial edema is a possible feature in cardiac amyloidosis.

Cardiomyopathies are an important and diverse group of heart muscle diseases in which the heart muscle itself is structural or functional abnormal. This often results in severe heart failure accompanied by arrhythmias and/or sudden death. Clinical and morphological diversity of cardiomyopathies can reflect the broad spectrum of distinct underlying molecular causes or genetic heterogeneity. In addition, modifying genes, life style and additional factors were reported to influence onset of disease, disease progression and prognosis. The individual patient's phenotype may reflect a summation and/or interaction of the underlying mutation with other genetic or environmental factors. During the last years major advances have been made in the understanding of the molecular and genetic basis of this type of disease. Nevertheless, much more progress in the identification of underlying mutations, susceptibility genes and modifier genes is important and indispensable for the development of new etiology orientated forms of therapy.

Amyloidoses are a heterogeneous group of multisystem disorders, which are characterized by an extracellular deposition of amyloid fibrils. Typically affected are the heart, liver, kidneys, and nervous system. More than half of the patients die due to cardiac involvement. Clinical signs of cardiac amyloidosis are edema of the lower limbs, hepatomegaly, ascites and elevated jugular vein pressure, frequently in combination with dyspnea. There can also be chest pain, probably due to microvessel disease. Dysfunction of the autonomous nervous system or arrhythmias may cause low blood pressure, dizziness, or recurrent syncope. The AL amyloidosis caused by the deposition of immunoglobulin light chains is the most common form. It can be performed by monoclonal gammopathy. The desirable treatment therapy consists of high-dose melphalan therapy twice followed by autologous stem cell transplantation. Due to the high peritransplantation mortality, selection of appropriate patients is mandatory. The ATTR amyloidosis is an autosomal dominant disorder caused by the amyloidogenic form of transthyretin, a plasmaprotein that is synthesized in the liver. Therefore, liver transplantation is the only curative therapy. The symptomatic treatment of cardiac amyloidosis is based on the current guidelines for chronic heart failure according to the patient's New York Heart Association (NYHA) state. Further types of amyloidosis with possible cardiac involvement comprise the senile systemic amyloidosis caused by the wild-type transthyretin, secondary amyloidosis after chronic systemic inflammation, and the beta(2)-microglobulin amyloidosis after long-term dialysis treatment.

Arrhythmogenic right ventricular dysplasia was diagnosed in 2000 in this 44-year-old male patient with a history of syncope. An internal defibrillator was implanted. Six years later the patient was readmitted with severe heart failure, and cardiac sarcoidosis was diagnosed by myocardial biopsy. Response to a course of glucorticoids was favourable. We herein review diagnostic strategies and therapeutic options in this rare disorder.

We investigated efficacy and safety of different energy sources and application techniques for the treatment of atrial fibrillation in an experimental acute sheep model. In particular, we focused on thermal damage to the adjacent structures and tissues. We also attempted to evaluate the efficacy of different application techniques such as endocardial or epicardial approaches. Overall 64 young Merino sheep were examined. It could be shown that endocardial ablation with different energy sources on cardiopulmonary bypass consistently caused histomorphologically and electrophysiologically transmural lesions. Depending on the energy source, different amounts of endocardial damage were induced. Cryoapplication produces the smallest endocardial laceration without thrombus formation. Dry radiofrequency energy and microwave produced very wide and diffuse endocardial damage with carbonisation and disruption of the endothelium. Epicardial ablation on a beating heart (off-pump) with bipolar radiofrequency was consistently effective. Due to the energy flow between the two jaws of the bipolar clamp, no collateral damage was observed. All other energy sources were unable to produce transmural lesions epicardially (off-pump) because the nearby blood flow rewarmed or recooled the myocardium and caused the so called "heat sink phenomenon". Depending on the energy source, different histomorphological changes in the esophagus could be observed. Changes in intraluminal-measured esophageal temperatures were not observed during ablation.

The heart can be the primary target for a viral, bacterial or parasitic infection (primary myocarditis/inflammatory cardiomyopathy). It can also participate in the "collateral damage" due to toxins, chemo- and cytokines, autoreactive antibodies or the native and acquired immune response through T- and B-cells, monocytes and macrophages (secondary myocarditis/inflammatory cardiomyopathy), when it is not the dominant organ of the disease. Infective agents show remarkable organ specificity: viral infections, toxic and autoreactive processes affect primarily the myocardium and the pericardium, whereas bacterial infections prefer endothelial surfaces and cause endocarditis and, less frequently, pericarditis. They are even discussed as part of the inflammatory process involved in coronary artery disease. Infective agents and their adequate diagnosis and treatment are discussed for these clinical entities according to current guidelines and clinical pathways.

Anamnesis: A 68-year-old woman was admitted to the emergency unit with symptoms of an acute coronary syndrome. She complained about a feeling of chest oppression for two hours. The cardiac history was unremarkable. Her husband had died few hours before the onset of her cardiac symptoms., Investigations: Blood pressure was 80/40 mmHg, heart rate 110/min. Cardiac enzymes were normal. The ECG showed significant ST elevation in V2 to V6. Laevoangiocardiography revealed a severe reduction of left ventricular systolic function with an ejection fraction of 27 %. Akinesia was seen in the anterior wall region as well as apical, inferior, and poserolateral. Coronary heart disease could be excluded., Diagnosis, Therapy and Course: Hemodynamic support with catecholamines was needed for one day. After two days hemodynamic recovery occurred. The creatine kinase peak level was surprisingly low (357 U/l). Echocardiographies performed in short intervals showed a continuous, rapid recovery of the ventricular function. Two weeks later the left ventricular function was normal. There was no evidence of myocarditis. In consideration of the history of emotional stress, absence of coronary lesions, a low CK peak and rapid recovery of left ventricular function, Tako-Tsubo cardiomyopathy was diagnosed. Medical treatment with beta blockers was initiated: the further clinical course was unremarkable., Conclusion: Tako-Tsubo cardiomypathy should be considered in patients with findings and symptoms of an acute coronary syndrome.

Inflammation of the myocardium is a common cardiac disease. Molecular and immunhistological techniques help to assure myocarditis in terms of persistence of chronic viral infection and myocardial inflammation activity. The connection between viral myocarditis and development of dilative cardiomyopathy is of great interest. Thus, new possibilities arise for assessment of the disease prognosis and the strategy of specific immunosuppressive, immunomodulating and antiviral therapy.

This review may serve as a basis for evaluating publications on the topic "myocardial biopsy for myocarditis and dilated cardiomyopathy" in the clinical practice. The literature is particularly analyzed to answer the question, whether an endomyocardial catheter biopsy is indicated in patients with these myocardial disorders in the clinical routine besides its unequivocal scientific value. The judgment of the biopsy samples has been based on the classically histological and for years on the additional immunohistochemical and molecular biological-virological examination. The analysis of the literature data shows that outside scientific studies there is no indication to perform myocardial biopsy, or in other words, this procedure is not suitable for diagnosis, therapy, detection of early stages or prognostic evaluation in the disease spectrum "myocarditis, inflammatory heart disease, dilated cardiomyopathy". Reasons are the subjectivity in the judgment and interpretation of bioptic findings resulting in considerable interobserver variability, a missing standardization in biopsy performance, methods of examination and diagnostic criteria, the bioptic sampling error, missing therapeutic and prognostic consequences and potentially severe complications in performing myocardial biopsies. So far, the specificity of inflammatory changes in patients with dilated cardiomyopathy has not been proven in controlled blinded studies. The bioptic changes could be understood also as an unspecific inflammatory process in front of increasing pathophysiological evidence for myocardial inflammation in any form of heart failure. In addition, regarding the specific etiology of dilated cardiomyopathy, primarily a genetic, noninfectious or autoimmunologic origin plays an increasing role. The favorable clinical course and the very good prognosis of the acute, clinically diagnosed or supposed viral myocarditis should also be taken into account for the evaluation of myocardial biopsy. It should also be considered that the proof of causality between acute myocarditis and dilated cardiomyopathy is still lacking. Regarding the diagnosis "inflammatory cardiomyopathy" and multiple inflammatory subsets among patients with dilated cardiomyopathy or unclear regional contraction disorder, there is no adequate clinical validation of different diagnostic methods, criteria and interpretations so far. It is missleading to replace the well-established clinical diagnosis myocarditis by the bioptic diagnosis "inflammatory cardiomyopathy". However, endomyocardial catheter biopsy is clearly indicated in rare patients with fulminant myocarditis, giant-cell myocarditis and myocardial storage disease. Its probably underestimated role in sarcoid heart disease still needs to be clarified by systematic studies.

Cardiac involvement is well known in a number of skeletomuscular diseases but not in facio-scapulohumeral muscular dystrophy (FSHD). We report on a 71 year old woman with progressive cardiac insufficiency in FSHD, which was also confirmed by molecular analysis in one of the two daughters affected by the disease. Autopsy of the deceased patient showed the typical changes in skeletal muscles including focal inflammatory infiltrates in the diaphragm and, in addition, cardiac muscular involvement. The histological changes resembled those seen in primary cardiomyopathy despite the normal muscle mass volume. Both clinically and morphologically, the cardiac disease was the cause of death in this patient with FSHD.

Sarcoidosis is a systemic disease, in which early inflammatory organic changes, which are often reversible under treatment, may take a subclinical course. In scarred residual tissue improvement id rare. Continuing functional loss or even organ failure is possible. The involvement of the heart is of essential prognostic value. Early onset of treatment with corticosteroids before irreversible functional loss of the heart occurs leads to an excellent prognosis. Technical progress in the imaging techniques have led to great improvement of the graphical presentation of cardiac structure and function, but not one of these techniques has yet been approved of for early diagnosis. Still, a combination of different diagnostical measures in addition to the evaluation of clinical symptoms often allows early diagnosis. A decisive factor is "to consider it" and a timely start of further diagnostics if certain symptoms are presented. Alternatives to the specific treatment with cortisone are still lacking. Symptomatic cardiac treatment is mainly oriented on the therapy of dilatory cardiomyopathies, since there are no reliable systematic studies available on questions concerning, for example, antiarrhythmic treatment or indications for the implantations of pacemakers in connection with cardiac sarcoidosis.

Adult polyglucosan body disease (APBD) is a rare genetic disorder, inherited in an autosomal recessive mode. The disease is caused by mutations of the gene coding for the glycogen-branching enzyme, which is essential for branching of polyglucose chains in the normal glycogen molecule. The age of clinical manifestation of the disease mostly is between 40 and 60 years and its course is slowly progressive. Characteristic globular deposits (polyglucosan bodies, PGB) can be detected in biopsies of skin and skeletal muscle as well as in the peripheral and central nervous system. Biochemically, PGBs consist of poorly branched glycogen molecules with abnormally long polysaccharide chains. We report the case of a 50-year-old female patient with APBD who suffered from neurological symptoms such as spastic tetraparesis, urinary incontinence, hypesthesia and dementia. She died unexpectedly of cardiac failure. At autopsy a severe cardiomyopathy with abundant PGBs in the heart muscle fibres could be proven as the cause of death. This observation shows that in addition to the known deposition of PGBs in nervous system and skeletal muscle, an involvement of the heart has to be considered in APBD as well.

Sarcoidosis is a systemic disease of young adults. Cardiac involvement is rarely diagnosed clinically. It presents with arrhythmias, conduction disorders, increasing myocardial insufficiency, or sudden death. We report a 42-year-old man with cardiac conduction disturbances of unknown cause who died of sudden cardiac death. Sarcoidosis with prominent cardiac involvement was diagnosed only at autopsy. Cardiac sarcoidosis should be considered in young patients with unexplained conduction disorders.

History and Clinical Findings: A 40-year-old man with histologically proven sarcoidosis, known for 15 years, which had involved the myocardium was hospitalized because of intractable heart failure (NYHA class IV). An implantation of an intracardiac defibrillator for ventricular arrhythmias (Lown type IVa) had preceded. On physical examination severe dyspnea at rest cough and fever were noted., Investigations: The erythrocyte sedimentation rate (88/104 mm), C-reactive protein (250 mg/l) and white cell count (20/nl) were markedly increased. Serum sodium (113 mmol/l), potassium (3.0 mmol/l) and chloride (64 mmol/l) were markedly reduced, while creatinine (2.5 mg/dl) and urea (82 mg/dl) were elevated due to renal failure. The chest radiogram demonstrated central venous congestion, cardiomegaly and right pericardial infiltration. There were no obvious changes due to sarcoidosis and computed tomography did not indicate pulmonary involvement by sarcoidosis. The echocardiogram revealed severe impairment of left ventricular function with an ejection fraction of ca. 14%., Diagnosis, Treatment and Course: Heart failure (NYHA class IV), caused by a dilated cardiomyopathy of sarcoidosis, was accompanied by pneumonia which responded to antibiotics. But the chronic heart failure failed to improve on drug treatment and cardiac transplantation was undertaken. The explanted myocardium was examined histologically, immunologically and virologically. Antibodies were demonstrated against vascular endothelium, sarcolemma and endocardium (IgG and IgA), but not by PCR against cytomegalovirus, enterovirus and adenovirus. The transplantation was without complication and, under azathioprine and methylprednisolone, one rejection had occurred until now. The patient has been working full-time since 2 years in his previous occupation of lorry driver. There has been no evidence of renewed sarcoidosis activity., Conclusion: Sarcoidosis may involve the myocardium in up to 25% of cases. Clinically relevant symptoms are even more rare. Sarcoidosis should be included in the differential diagnosis of unexplained serious arrhythmias or cardiomyopathy, particularly in young persons. Cardiac transplantation may have to be contemplated if drug or pacemaker treatment fails to control heart failure.

Cell death can be induced by 2 different mechanisms: necrosis and apoptosis. Necrosis, on the one hand, is usually caused by unphysiological stress factors such as hyperthermia or hypoxia, apoptosis, on the other hand, is part of the normal organ development and controls for example immune responses. Morphologically, necrosis is characterized by swelling of cells and their organelles leading to the disruption of the cell membrane, which in turn causes an inflammatory reaction in the surrounding tissue. Morphological and biochemical criteria (Figure 1, Table 1) of apoptosis are the condensation of chromatin leading to the development of apoptotic bodies or membrane-enclosed vesicles containing oligonucleosomal DNA fragments. Important diagnostic tools of cell death (Table 2), such as the TUNEL test (Figure 2) or gel electrophoresis of extracted DNA (Figure 3) are based on the above mentioned biochemical characteristics, but a reliable differentiation of apoptotic versus necrotic processes is not always possible. Experimental studies in animals and studies in various diseases of the cardiovascular system were able to show that apoptosis in myocytes can be induced, an issue that has long been discussed controversially. Ischemia, reperfusion, and myocardial infarction were also shown to lead to apoptosis in cardiomyocytes, whereas cell destruction was caused mainly by necrosis. Several authors (Table 3) demonstrated apoptotic indices in cardiomyocytes of patients with dilatated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and patients with acute infarction from 0.25 to 35% by the use of the TUNEL test. Others were able to demonstrate an elevated expression of Fas-receptor in cells of atheroslerotic plaques in patients with atherosclerosis and high indices of apoptotic cardiomyocytes in patients with chronic heart failure. We investigated endomyocardial biopsies of patients with inflammatory cardiomyopathy, DCM without inflammatory reaction but the presence of adenoviral or cytomegaloviral genome and idiopathic DCM using the TUNEL test. The percentage of apoptotic cardiomyocytes in biopsies of patients with DCMi was 1.03 and in biopsies of patients with adenoviral genome 0.25, whereas in all other groups no apoptosis was found. If apoptosis plays a major role in myocardial diseases such as heart failure, arrhythmia and others, blocking this mechanism will have to be considered as a therapeutical strategy. Therefore, studies on the extent of apoptotic processes in diseased versus healthy cardiac tissue are of great importance.

Cardiac involvement in AL-amyloidosis due to a multiple myeloma is present in up to 90% of cases. We present two patients with cardiac symptoms in whom a hematologic disease could be diagnosed because of suspicious cardiac finding. The leading symptom was dyspnea. The routinely performed laboratory tests, especially the erythrocyte sedimentation rate and the electrophoresis, were normal. After exclusion of coronary artery disease an infiltrative cardiomyopathy was suspected because of the echocardiographic examination with marked left ventricular hypertrophy, the restrictive flow pattern at the mitral valve and the electrocardiogram with a low voltage in limb leads and absent R waves in left precordial leads. Further, hematologic workup confirmed the production of light chains due to a myeloma. If the echocardiographic examination and the electrocardiogram raises the suspicion of an infiltrative cardiomyopathy as the cause of dyspnea, an immunofixation should be done in spite of normal laboratory tests to confirm or rule out the presence of a light chain disease due to a myeloma.

Human disease caused by echinococcus granulosus is rare in Northern Europe, but is seen increasingly due to migration of labour and of tourism to endemic areas e. g. Southern Europe and the Middle East. Echinococcosis of the heart occurs in only 0.5 to 2% of cases of echinococcosis. Diagnosis is difficult because of the long latency between infection and manifestation of disease, and also because symptoms are unspecific. It is a diagnosis "to think of". Apart from a thorough history serological tests and medical imaging (ultrasound, X-rays, computed tomography) are used in the diagnosis of echinococcosis. Surgery is the preferred therapy, if not feasible medical treatment with benzimidazoles (Albendazole and Mebendazole) is effective. We describe a case of echinococcosis of the heart as the only manifestation of the disease: a 34-year-old male Turkish patient came in severe cardiac shock due to cardiac tamponade to the emergency department. Immediate heart surgery revealed a pericardium filled with cysts of echinococcus granulosus, infiltrating the left ventricle of the heart (Figure 1). The cysts were removed and the patient was put on a 2-year course of Mebendazole. The patient is free of relapse for more than 6 years now and he is considered to be cured.

Hemochromatosis is an autosomal-recessive disease which causes iron-overload of various organs including liver, pancreas and heart. This report analyzes the course of hemochromatosis in two patients (a 28-year-old man and a 57-year-old woman) in whom hemochromatosis was detected because of severe cardiomyopathy. Initial symptoms were edema, anasarca and dyspnea. Further examinations showed pleural effusion, decreased left-ventricular-function, skin pigmentation, diabetes mellitus and liver cirrhosis. Although phlebotomy treatment and iron-chelation therapy with deferoxamine initially resulted in some improvement, both patients died from cardiomyopathy three months after diagnosis. The reports of these two cases underline that hemochromatosis-associated cardiomyopathy is often irreversible if severe congestive heart failure is present. In cardiac decompensation heart transplantation has to be considered as early as possible.

In situ hybridization studies have shown that myocardial enterovirus infections may be detected in acute and chronic enterovirus-induced myocarditis, indicating the possibility of myocardial enterovirus persistence in the human heart. As well, such infections can be observed in patients with dilated cardiomyopathy, a condition which may evolve from chronic myocarditis. The discovery of possible enterovirus persistence in the human heart is supported by the finding in different strains of immunocompetent mice, demonstrating that coxsackievirus B3, typically a cytolytic enterovirus, is capable of evading immunological surveillance in a host-dependent fashion, thus inducing a persistent type of heart muscle infection. Progress is currently being made in unraveling the molecular mechanisms of enterovirus persistence, the diversity of host and virus genetics and their impact on the nature and severity of myocardial injury. Apart from providing an etiologic diagnosis, there are therapeutic implications from in situ demonstration of myocardial enterovirus infection. Evaluation of specific antiviral agents, for example interferons, may lead to the development of new therapeutic strategies capable of providing protection against myocardial enterovirus infection.

Clinical and morphological findings recorded in an 81-year-old woman patient with a 45-year history of rheumatoid arthritis treated with long-term chloroquine therapy are reported. The cause of death was low cardiac output syndrome, which upon autopsy and postmortem toxicological analyses was classified as vacuolar chloroquine-induced cardiomyopathy. The pathogenesis and the morphological and clinical characteristics of cardiotoxic effects of chloroquine are reviewed.

Described in this paper are pathomorphological alterations to the heart postmortem recorded from 100 deceased with rheumatoid arthritis. Vasculitis of subepicardial or intramural rami of coronary arteries was recorded from twelve cases and had caused multiple myocardial necrosis in ten of these. These developments had led to progressive cardiac insufficiency which actually then was the direct cause of death. Formal pathogenesis of vasculitis-related multifocal myocardial necrosis in concomitance with rheumatoid arthritis had never been described in the literature before, although such reference would have been justified for the clinical consequences for which it deserves to be emphasised as a nosological entity in its own right. Isolated rheumatic nodes were detected in the myocardium of five of the decreased and could be attributed to rheumatic arteritis in two cases. Those myocardial rheumatic nodes, according to the author's view, represent the severest form of necrotising rheumatic vasculitis. Pathognomonic pericarditis was found in four cases (2 pericarditis nodularis and 2 pericarditis rheumatica). Pathognomonic nodular valvulitis was identified in another four cases. Systemic secondary amyloidoses were found to be present in 24 of the above 100 deceased with chronic polyarthritis. The heart was involved in 23 of these 24 cases (95.8%).