[Inflammatory cardiomyopathy and myocarditis]. / Inflammatorische Kardiomyopathie und Myokarditis.

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.

[Alcohol consumption in women and the elderly : When does it induce heart failure?]. / Alkoholkonsum bei Frauen und Senioren : Wann führt er zur Herzinsuffizienz?

The association between alcohol consumption and the etiology and prognosis of cardiovascular diseases has been the focus of attention and also the subject of controversial discussions for many years. This is particularly true for heart failure, which can be induced by coronary artery disease (CAD), arterial hypertension, atrial and ventricular arrhythmias and cardiomyopathies. Acute effects of high doses of alcohol can lead to impairment of the cardiac contraction strength with rhythm disturbances (holiday heart syndrome), transient ischemic attacks and in rare cases to sudden cardiac death. The chronic effects of high alcohol consumption include in particular, ventricular dysfunction, chronic rhythm disturbances, alcoholic cardiomyopathy and CAD. In contrast, light to moderate consumption of alcohol is associated with a reduced risk of CAD and ischemic stroke; however, even moderate alcohol drinking is associated with a greater risk for atrial fibrillation. The unfavorable effects of alcohol occur at much lower levels of acute or chronic consumption in women than in men. In the elderly just as in young people, a moderate alcohol consumption is associated with a lower risk of heart failure.

Reverse epidemiology in different stages of heart failure.

BACKGROUND: In heart failure (HF), traditional cardiovascular risk factors (RF) as body mass index (BMI), total cholesterol (TC) and systolic blood pressure (SBP) are associated with better survival. It is unknown at which time point along the disease continuum the adverse impact of these RF ceases and may 'start to reverse'. We analyzed the distribution of RF and their association with survival across HF stages. METHODS: We pooled data from four cohort studies from the German Competence Network HF. Employing ACC/AHA-criteria, patients were allocated to stage A (n=218), B (n=1324), C1 (i.e., New York Heart Association [NYHA] classes I & II; n=1134), and C2+D (NYHA III & IV; n=639). RESULTS: With increasing HF severity median age increased (63/67/67/70 years), whereas the proportion of females (56/52/37/35%), median BMI (26.1/28.8/27.7/26.6 kg/m(2)), TC (212/204/191/172 mg/dl), and SBP (140/148/130/120 mmHg) decreased (P<0.001 for trend for all). In the total cohort, higher levels of all RF were associated with better survival, even after extensive adjustment for multiple confounders. If analyses were stratified, however, a higher RF burden predicted better survival only in clinically symptomatic patients: hazard ratio (HR) per +2 kg/m(2) BMI 0.91 (95% confidence interval 0.88; 0.95); per +10 mg/dl TC 0.93 (0.92; 0.95); per +5 mmHg SBP 0.94 (0.92; 0.95). CONCLUSION: In this well-characterized sample of patients representing the entire HF continuum, reverse associations were only consistently observed in symptomatic HF stages. Our data indicate that the phenomenon of a "reverse epidemiology" in HF is subject to significant selection bias in less advanced disease.

Suspected inflammatory cardiomyopathy. Prevalence of Borrelia burgdorferi in endomyocardial biopsies with positive serological evidence.

BACKGROUND: Cardiac involvement in Lyme disease, caused by the tick-borne spirochete Borrelia burgdorferi, has been reported to occur in 0.3-4 % of infected patients in Europe. Cardiac manifestations may include conduction disturbances, and also myocarditis, pericarditis, and left ventricular dysfunction. We investigated the prevalence of B. burgdorferi DNA in endomyocardial biopsies from patients with suspected inflammatory heart disease and positive serology for B. burgdorferi. METHODS AND RESULTS: In 64 patients, endomyocardial biopsies were taken after exclusion of coronary heart disease by coronary angiography, and investigated with polymerase chain reaction (PCR) for the presence of B. burgdorferi and cardiotropic viruses. B. burgdorferi DNA was not detected in any of the endomyocardial biopsies. Viruses, particularly parvovirus B19, were detected as infectious agents in 19 (30 %) patients. CONCLUSION: The results of our study demonstrate that PCR analysis of endomyocardial biopsies from patients with suspected inflammatory heart disease, including individuals with dilated cardiomyopathy (DCM) and positive serology for B. burgdorferi, did not reveal the B. burgdorferi genome in any biopsy sample.

[Etiology, diagnosis, management, and treatment of myocarditis. Position paper from the ESC Working Group on Myocardial and Pericardial Diseases]. / Ätiologie, Diagnose, Management und Therapie der Myokarditis. Positionspapier der ESC Working Group on Myocardial and Pericardial Diseases.

Despite great advances in the pathophysiology and etiology of myocarditis, the clinical diagnosis of myocarditis in daily clinical practise remains challenging. Often the diagnosis was not clear because of the heterogeneity of clinical symptoms and the lack of guidelines for adequate diagnostic requirements and consecutive treatment options. The European Society of Cardiology (ESC) Working Group on Myocardial and Pericardial Diseases established a working group of experts to improve the diagnosis and management of myocarditis and to provide a common consensus statement as a reference for future registries and controlled trials. The goal was to bridge the gap between clinical- and tissue-based diagnosis by formulating a concept concerning essential diagnostics and treatment of these patients that would be accepted across Europe. Only in this manner is it possible to establish a basis for national and international registries and double-blind randomized treatment trials for the etiologically differentiated treatment of myocarditis, which appear promising due to numerous studies in recent years. In this paper, two members from the expert working group summarize the most important aspects of this position paper on the etiology, diagnosis, management, and treatment of myocarditis, which were published in the July 2013 issue of European Heart Joumal.

Polymorphisms in genes encoding nonsarcomeric proteins and their role in the pathogenesis of dilated cardiomyopathy.

Dilated cardiomyopathy (DCM), clinically characterized by contractile dysfunction and ventricular chamber enlargement, is a heterogeneous heart disease leading to progressive systolic heart failure and sudden cardiac death. The etiology of the disease is multifactorial and involves genetic factors, viral infections, autoimmune phenomena, and toxic agents. Within the last two decades, a growing body of evidence has suggested that single-gene mutations play a pivotal role in the development of familial forms of dilated cardiomyopathies. Numerous genes encoding cytoskeletal, sarcomeric, and nuclear proteins have been linked to the pathogenesis of DCM, and most of the respective mutants disrupt the structural integrity of sarcomeres in cardiac myocytes. Frequently, point mutations in cytoskeletal proteins critically diminish force generation and interfere with mechanical transduction within the contractile apparatus of the myocardium, thereby ultimately leading to impaired systolic function. However, hitherto reported sarcomeric gene defects explain the etiology of the disease only in some families, leaving other forms of DCM subentities unresolved. Since one of the major factors in DCM pathogenesis involves autoimmune-mediated damage to cardiac tissue, candidate genes that are involved in controlling immune reactions have currently come into focus in genetic research. We and others have shown that a single-nucleotide polymorphism (SNP) in the gene encoding cytotoxic T-lymphocyte antigen 4 (CTLA4) is associated with the diagnosis of DCM. Cytotoxic T-lymphocyte antigen 4 is an inhibitory receptor molecule expressed on activated T lymphocytes, where it functions as an important negative regulator of T-cell activation by competing with the costimulatory CD28 receptor to bind to B7 receptors localized on the surface of antigen-presenting cells. The observed association between CTLA4 genotypes and DCM suggests that genetic factors contribute to both unbalanced immune responses in the myocardium and the development of left ventricular dysfunction. In this review, we will briefly discuss how these findings may stimulate the search for novel DCM-associated SNPs in human genes expressed in noncardiomyocytes.

Current treatment options in (peri)myocarditis and inflammatory cardiomyopathy.

In inflammatory dilated cardiomyopathy and myocarditis there is--apart from heart failure and antiarrhythmic therapies--no alternative to an aetiologically driven specific treatment. Prerequisite are noninvasive and invasive biomarkers including endomyocardial biopsy and PCR on cardiotropic agents. This review deals with the different etiologies of myocarditis and inflammatory cardiomyopathy including the genetic background, the predisposition for heart failure and inflammation. It analyses the epidemiologic shift in pathogenetic agents in the last 20 years, the role of innate and aquired immunity including the T- and B-cell driven immune responses. The phases and clinical faces of myocarditis are summarized. Up-to-date information on current treatment options starting with heart failure and antiarrhythmic therapy are provided. Although inflammation can resolve spontaneously, specific treatment directed to the causative aetiology is often required. For fulminant, acute and chronic autoreactive myocarditis immunosuppressive treatment is beneficial, while for viral cardiomyopathy and myocarditis ivIg can resolve inflammation and is as successful as interferon therapy in enteroviral and adenoviral myocarditis. For Parvo B19 and HHV6 myocarditis eradication of the virus is still a problem by any of these treatment options. Finally, the potential of stem cell therapy has to be tested in future trials. In virus-negative, autoreactive perimyocardial disease a locoregional approach with intrapericardial instillation of high local doses of triamcinolone acetate has been shown to be highly efficient and with few systemic side-effects.

Implementation of pharmacotherapy guidelines in heart failure: experience from the German Competence Network Heart Failure.

AIM: To evaluate the implementation of current pharmacotherapy guidelines of heart failure and to identify factors associated with high pharmacotherapy guideline adherence in heart failure patients. METHODS AND RESULTS: We pooled data from seven studies performed in the context of the German Competence Network Heart Failure selecting patients with chronic systolic heart failure and left ventricular ejection fraction (LVEF) <45% (n = 2,682). The quality of pharmacotherapy was evaluated by calculating the guideline adherence indicator (GAI), which considers three (GAI-3) or five (GAI-5) of the recommended heart failure substance classes and accounts for respective contraindications. GAI-3 was categorized as perfect (GAI = 100%: 71% of the cohort), medium (GAI = 50-99%: 22%), and poor adherence (GAI <50%: 7%). In ordinal regression, the following factors were positively associated with perfect adherence: history of revascularization (odds ratio 1.59, 95% confidence interval 1.27-1.98), prior ICD implantation (2.29, 1.76-2.98), and LV ejection fraction <30% (1.45, 1.19-1.76), whereas age (per 10 years; 0.82, 0.77-0.89), NYHA III/IV (0.15, 0.12-0.18), unknown duration of heart failure (0.69, 0.53-0.89), and antidepressant medication (0.61, 0.42-0.88) were negatively associated with perfect adherence. Better GAI-3 at baseline predicted favorable changes of LV ejection fraction and end-diastolic diameter after 1 year. One-year mortality risk was closely related to GAI-3 in both groups of NYHA functional class I/II (excellent vs. poor GAI-3: 7.2 vs. 14.5%, log rank = 0.004) and class III/IV (13.5 vs. 21.5%, log rank = 0.005). CONCLUSIONS: This large pooled analysis showed that a high level of guideline adherence is achievable in the context of clinical studies. Those receiving and tolerating optimal pharmacotherapy experience a better prognosis. Nevertheless, the implementation of heart failure medication needs further improvement in female and elderly patients, especially those in NYHA functional class >II and patients with LVEF ≥30%.

Pericardial effusion of HIV-infected patients ? Results of a prospective multicenter cohort study in the era of antiretroviral therapy.

BACKGROUND: Patients with human immunodeficiency virus (HIV) infection have an increased risk of cardiovascular diseases. Previous publications described pericardial effusion as one of the most common HIV-associated cardiac affiliations. The aim of the current study was to investigate if pericardial effusion still has a relevant meaning of HIV-infected patients in the era of antiretroviral therapy. METHODS: The HIV-HEART (HIV-infection and HEART disease) study is a cardiology driven, prospective and multicenter cohort study. Outpatients with a known HIV-infection were recruited during a 20-month period in a consecutive manner from September 2004 to May 2006. The study comprehend classic parameters of HIV-infection, comprising CD4-cell count (cluster of differentiation) and virus load, as well as non-invasive tests of cardiac diseases, including a thorough transthoracic echocardiography. RESULTS: 802 HIV-infected patients (female: 16.6%) with a mean age of 44.2 ± 10.3 years, were included. Duration of HIV-infection since initial diagnosis was 7.6 ± 5.8 years. Of all participants, 85.2% received antiretroviral therapy. Virus load was detectable in 34.4% and CD4 - cell count was in 12.4% less than 200 cells/µl. Pericardial effusions were present in only two patients of the analysed population. None of the participants had signs of a relevant cardiovascular impairment by pericardial effusion. CONCLUSIONS: Our results demonstrate that the era of antiretroviral therapy goes along with low rates of pericardial effusions in HIV-infected outpatients. Our findings are in contrast to the results of publications, performed before the common use of antiretroviral therapy.

Differential diagnosis of pericardial effusion after stem cell transplantation in acute myeloic leukemia.

The pathology underlying a pericardial effusion in a 24-year-old patient, who had suffered from acute myeloic leukemia 5 years previously and undergone chemotherapy followed by whole body radiation prior to allogeneic stem cell transplantation, could be identified by the careful analysis of pericardial cytology and epicardial biopsy guided by flexible pericardioscopy. Molecular, histological, cytochemical and immunological examination of the effusion and the epicardial biopsy for a viral or bacterial infection despite known CMV reactivation, or an effusion induced by radiation or graft-versus-host reaction, could be ruled out as possible causes of pericardial tamponade. The infiltration of CD 117-positive cells in the biopsied cardiac tissue revealed recurrent acute myeloic leukemia now also affecting the heart and the pericardium. An intrapericardial instillation of 1000 mg triamcinolone acetate at day 1 and 50 mg/m(2) cisplatin at day 3 effectively prevented the recurrence of tamponade, but could not prevent a lethal outcome 3 weeks later.

[Tumor markers in the assessment of malignant and benign pericardial effusion]. / Tumormarker im Perikarderguss bei malignen und nichtmalignen Perikardergüssen.

BACKGROUND: The differential diagnosis of pericardial effusion is often challenging because different etiologies can be discussed. Of particular therapeutic and prognostic importance is the definitive differentiation of malignant pericardial effusion from benign effusions. The definitive diagnosis of malignant pericardial effusion is established by a positive cytological examination of the pericardial fluid. However, pericardial fluid cytology, although specific has variable sensitivity. Tumor markers are often investigated after pericardiocentesis but their utility as an aid for the diagnosis of malignant pericardial effusion is not well established. The aim of this study was to measure the concentrations of the tumor markers CEA, CA 19-9, CA 72-4, SCC and NSE in malignant and non-malignant pericardial effusions and to assess their diagnostic utility in differentiating malignant from benign pericardial effusion. METHODS: We investigated the pericardial fluid of 29 patients with proven malignant pericardial effusion and 25 patients with non-malignant pericardial effusion. The etiology of the pericardial effusion was defined by pericardial cytology, epicardial histology and PCR for cardiotropic viruses from pericardial and epicardial tissue acquired by pericardioscopy. The group with non-malignant pericardial effusion comprised 15 patients with autoreactive effusion and 10 patients with viral pericardial effusion. We analyzed the following tumor markers in the pericardial fluid: carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, carbohydrate antigen (CA) 72-4, squamous cell carcinoma (SCC) antigen and neuron-specific enolase (NSE). RESULTS: Of the tumor markers tested the mean concentrations of the CEA, CA 72-4 and CA 19-9 were significantly higher in malignant pericardial effusions than in non-malignant effusions (CEA 450.66 ±1620.58 µg/l vs. 0.72 ±1.49 µg/l, p<0.001; CA 19-9 1331.31 ±3420.87 kU/l vs. 58.85 ±17.53 kU/l, p=0.04; CA 72-4 707.90 ±2397.55 kU/l vs. 0.48 ±2.40 kU/l, p<0.001). ROC curve analysis showed that pericardial fluid CA 72-4 yielded an area under the curve (AUC) of 0.85 (95% confidence interval 0.74-0.95), followed by CEA with 0.80 (95% confidence interval 0.68-0.92). Pericardial fluid CA 72-4 levels >1.0 kU/l had 72% sensitivity (95% confidence interval 53%-87%) and 96% specificity (95% confidence interval 80%-99.9%) and CA 72-4 levels >2.5 kU/l had 69% sensitivity (95% confidence interval 49%-85%) and 96% specificity (95% confidence interval 80%-99.9%) in differentiating malignant pericardial effusions from effusions due to benign conditions. CONCLUSION: Malignant pericardial effusions are associated with significantly higher pericardial concentrations of the tumor markers CEA, CA 72-4 and CA 19-9. Of the tested tumor markers, measurement of CA 72-4 levels in pericardial fluid offered the best diagnostic accuracy. Based on our data evaluation of every patient with unexplained pericardial effusion and negative pericardial fluid cytology should include the measurement of pericardial fluid CA 72-4 levels. Under these circumstances the elevation of pericardial fluid CA 72-4 levels should include malignancy as a probable diagnosis.

Diabetic cardiomyopathy--fact or fiction?

Epidemiologic as well as clinical studies confirm the close link between diabetes mellitus and heart failure. Diabetic cardiomyopathy (DCM) is still a poorly understood "entity", however, with several contributing pathogenetic factors which lead in different stages of diabetes to characteristic clinical phenotypes. Hyperglycemia with a shift from glucose metabolism to increased beta-oxidation and consecutive free fatty acid damage (lipotoxicity) to the myocardium, insulin resistance, renin-angiotensin-aldosterone system (RAAS) activation, altered calcium homeostasis and structural changes from the natural collagen network to a stiffer matrix due to advanced glycation endproduct (AGE) formation, hypertrophy and fibrosis contribute to the respective clinical phenotypes of DCM. We propose the following classification of cardiomyopathy in diabetic patients: a) Diastolic heart failure with normal ejection fraction (HFNEF) in diabetic patients often associated with hypertrophy without relevant hypertension. Relevant coronary artery disease (CAD), valvular disease and uncontrolled hypertension are not present. This is referred to as stage 1 DCM. b) Systolic and diastolic heart failure with dilatation and reduced ejection (HFREF) in diabetic patients excluding relevant CAD, valvular disease and uncontrolled hypertension as stage 2 DCM. c) Systolic and/or diastolic heart failure in diabetic patients with small vessel disease (microvascular disease) and/or microbial infection and/or inflammation and/or hypertension but without CAD as stage 3 DCM. d) If heart failure may also be attributed to infarction or ischemia and remodeling in addition to stage 3 DCM the term should be heart failure in diabetes or stage 4 DCM. These clinical phenotypes of diabetic cardiomyopathy can be separated by biomarkers, non-invasive (echocardiography, cardiac magnetic resonance imaging) and invasive imaging methods (levocardiography, coronary angiography) and further analysed by endomyocardial biopsy for concomitant viral infection. The role of specific diabetic drivers to the clinical phenotypes, to macro- and microangiopathy as well as accompanying risk factors or confounders, e.g. hypertension, autoimmune factors or inflammation with or without viral persistence, need to be identified in each individual patient separately. Thus hyperglycemia, hyperinsulinemia and insulin resistance as well as lipotoxicity by free fatty acids (FFAs) are the factors responsible for diabetic cardiomyopathy. In stage 1 and 2 DCM diabetic cardiomyopathy is clearly a fact. However, precise determination of to what degree the various underlying pathogenetic processes are responsible for the overall heart failure phenotype remains a fiction.

Intrapericardial procedures for cardiac regeneration by stem cells: need for minimal invasive access (AttachLifter) to the normal pericardial cavity.

In view of the only modest functional and anatomical improvements achieved by bone marrow-derived cell transplantation in patients with heart disease, the question was addressed whether the intracoronary, transcoronary-venous, and intramyocardial delivery routes are adequate. It is hypothesized that an intrapericardial delivery of stem cells or activators of resident cardiac stem cells increases therapeutic benefits. From such an intrapericardial depot, cells or modulating factors, such as thymosin ß4 or Ac-SDKP, are expected to reach the myocardium with sustained kinetics. Novel tools which provide access to the pericardial space even in the absence of pericardial effusion are, therefore, described. When the pericardium becomes attached to the suction head (monitored by an increase in negative pressure), the pericardium is lifted from the epicardium ("AttachLifter"). The opening of the suction head ("Attacher") is narrowed by flexible clamps which grab the tissue and improve the vacuum seal in the case of uneven tissue. A ridge, i.e.,"needle guidance", on the suction head excludes injury to the epicardium, whereby the pericardium is punctured by a needle which resides outside the suction head. A fiberscope can be used to inspect the pericardium prior to puncture. Based on these procedures, the role of the pericardial space and the presence of pericardial effusion in cardiac regeneration can be assessed.

[The heart in viral infections]. / Das Herz bei viralen Infektionen.

Between 10 and 20% of patients with histologically proven inflammatory disease of the heart muscle develop a chronic disorder after acute myocarditis which results in dilated cardiomyopathy with increasing cardiac insufficiency. Viral infections are a frequent cause of inflammatory heart muscle diseases and thus also responsible for myocardial damage in the initial phase. In the past, evidence for enterovirus, adenovirus, and cytomegalovirus was in the focus of attention. In the meantime, "new" cardiotropic pathogens such as parvovirus B19, Epstein-Barr virus, and human herpesvirus 6 have been detected in patients with dilated cardiomyopathy with and without inflammation. Their persistence in the myocardium correlates with a decline in pumping capability within 6 months. While the virus is still being eliminated, the second phase of the disease begins, which is characterized by autoimmune phenomena and often a cardiac inflammatory response which likewise correlates with a worsening prognosis. The transition to the third and final phase with development of dilated cardiomyopathy occurs gradually and can take years. The goal of every diagnostic and therapeutic intervention must be to eradicate the virus and eliminate the inflammatory response to prevent the disease from progressing to terminal cardiac insufficiency.

[Classification, genetic predisposition and risk factors for the development of cardiomyopathies]. / Klassifikation, genetische Prädisposition und Risikofaktoren für die Entwicklung einer Kardiomyopathie.

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.

[The heart in cases of viral, bacterial and parasitic infections]. / Das Herz bei viralen, bakteriellen und parasitären Infektionen.

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.

Recurrent pericarditis in children: elevated cardiac autoantibodies.

UNLABELLED: Recurrent pericarditis is a rare disease in childhood. Nevertheless, it may represent a challenge to the clinician due to its resistance to anti-inflammatory treatment. The initial etiology often remains unclear; specific laboratory parameters predicting the frequency or severity of the recurrences are lacking. We report on four patients with recurrent pericarditis in whom antimyolemmal antibodies (AMLAs) were detected. A prolonged persistence of IgM-type AMLAs was found in three patients: two of them presented with acute inflammation as the initial event and one with 48 recurrences during 5.5 years. The fourth patient showed a fast conversion from IgM to IgG-type AMLAs after a less acute initial presentation and showed 4 mild recurrences during the 48-month follow-up. CONCLUSION: We were able to detect AMLAs in four children with recurrent pericarditis. This finding may be attributed to an auto-immunological disease following a first, acute event. We propose the detection of AMLAs in all children with unexplained recurrent pericarditis. Pediatric patients with a persistence of IgM-type AMLAs may face frequent recurrences and should be monitored therefore more closely. In addition, medical treatment may be changed in these patients with a slower tapering of the dosage of steroidal and non-steroidal antiinflammatory drugs.

[Recurrent autoreactive pericardial effusion. Impact of an aetiological classification of pericarditis]. / Rezidivierender autoreaktiver Perikarderguss. Bedeutung einer ätiologischen Klassifikation der Perikarditis.

HISTORY: A 36 year-old man suffered from fever, fatigue, pleurodynia and precordial discomfort. His family physician suspected febrile tracheobronchitis and treated it with ampicillin for 5 days. Because symptoms persisted an ECG was done which suggested acute myocardial infarction. The patient underwent an emergency coronary angiography which excluded coronary artery disease and aortic dissection. Pericarditis was suspected and the patient put on aspirin, 500 mg/d. Because of persisting cardiac symptoms an echocardiography was performed which revealed systolic separation between epi- and pericardium, characteristic of a small pericardial effusion after acute pericarditis. The symptoms improved after one week of treatment with diclofenac and the ECG had become normal. Two months later the patient was seen at our cardiac outpatient clinic. He had night sweats, sporadic precordial pain and severe dyspnoe. INVESTIGATIONS: Further investigations revealed tachycardia (120/min), hypotension (95/70 mm Hg), pulsus paradoxus and jugular vein sustension. Echocardiography revealed a large pericardial effusion ("swinging heart"), which explained the low voltage and the electrical alternans in the ECG. TREATMENT AND COURSE: Pericardiocentesis was carried out the same day to relieve the tamponade. It was followed by pericardioscopy and epi- as well as pericardial biopsy. 485 ml of a serous effusion were drained. Cytology and histology demonstrated a lymphocytic fibrinous pericarditis. Polymerase chain reaction (PCR) on viral and bacterial RNA and DNA of potentially cardiotropic agents remained negative. The pigtail catheter was left in place and 80 mg of gentamycin were given intrapericardially on day 1 and 2, followed by 500 mg of crystalloid triamcinolone acetate after the PCR was found to be negative. Oral treatment with 0.5 mg colchicine three times a day (off-label use) was started and maintained for 6 months. After 9 months no effusion was detected and the patient was free of symptoms. CONCLUSIONS: After exclusion of bacterial and viral pericardial infection, a high single dose of intrapericardial triamcinolone combined with long-term oral colchicine has proven to be a highly efficacious treatment of autoreactive pericarditis which will avoid relapses in most cases.

Invasive techniques--from diagnosis to treatment.

Invasive diagnostic and therapeutic techniques are indispensable for the diagnosis and interventional treatment of coronary artery disease, valvular involvement and, in particular, if the specific components of the inflammatory or degenerative processes in rheumatic disease are to be identified in the different components of the heart. Although impairment of cardiac function and ischaemia can be suspected also by non-invasive techniques, coronary involvement needs the final proof by angiography. Endomyocardial or epicardial biopsy identifies the key players of autoreactivity: the infiltrating cells and the bound and circulating antibodies. Before corticoid treatment is started, a viral or microbial aetiology has to be excluded at the site of cardiac inflammation. This again can only be done by the analysis of cardiac tissue samples.