Recurrent pericarditis: still idiopathic? The pros and cons of a well-honoured term.

In developed countries, more than 80% of cases of acute pericarditis remain without an established diagnosis after a conventional and standard diagnostic approach. These cases are generally labelled as 'idiopathic', i.e. without a known cause. This lack of information is a matter of concern for both patients and clinicians. Some years ago, this term reflected the state of the art of scientific knowledge on the topic. Advances have changed this point of view, in light of available molecular techniques like polymerase chain reaction able to identify viral cardiotropic agents in pericardial fluid and biopsies. Furthermore, the remarkable efficacy of interleukin-1 antagonists, a therapy targeting the innate immune response, suggests clinical and pathogenic similarity between a proportion of patients with idiopathic recurrent pericarditis and classical autoinflammatory diseases. So, it seems useful to discuss the pros and cons of using the term "idiopathic" in light of the new knowledge.

The classification concept of the ESC Working Group on myocardial and pericardial diseases for dilated cardiomyopathy.

In the WHO/ISFC classification of 1996, cardiomyopathies were defined as primary myocardial disorders of unknown cause. Heart muscle disorders of known etiology or associated with systemic disorders were classified as secondary or specific heart muscle diseases. An expert panel of the American Heart Association has recently suggested a new scheme that combines genetic and clinical criteria. In this system, the term primary is used to describe cardiac diseases in which the heart is the sole or predominantly involved organ and secondary to describe diseases in which myocardial dysfunction is part of a systemic disorder. In a radical departure from convention, they also suggested that ion channelopathies and disorders of conduction should be considered cardiomyopathies as well. The ESC Working Group on Myocardial and Pericardial Diseases has taken a different approach based on the belief that a clinically oriented classification system in which heart muscle disorders are grouped according to ventricular morphology and function remains the clinically most useful method for diagnosing and managing patients and families with heart muscle disease. In the ESC position statement, cardiomyopathies are defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality. In this article, this is illustrated by examples of dilated cardiomyopathy as familial/genetic forms and nonfamilial/nongenetic forms.

Effectiveness of intrapericardial administration of streptokinase in purulent pericarditis.

BACKGROUND AND PURPOSE: Purulent pericarditis is very rare. However, among patients suffering from this disease the mortality rate is very high. The aim of this study was to evaluate the effectiveness and side effects of intrapericardial streptokinase administration in patients with confirmed purulent pericarditis. PATIENTS AND METHODS: Three patients, one 50-year-old man and two women aged 64 and 40 years, who were admitted to the intensive care unit (ICU) due to purulent pericarditis, entered the study. In all three cases a subxiphoid pericardiotomy followed by insertion of a drainage line into the pericardial space was performed. Antibiotic therapy was started immediately on admission to the hospital. Despite continued antibiotic therapy in all three patients, daily drainage from the pericardium--during several days after surgery--staggered between 50-200 ml/day. Due to considerable purulent pericardial drainage loculations and/or fibrin deposits confirmed by echocardiography, streptokinase (500,000 IU dissolved in 50 ml of normal saline) was administered into the pericardial space over 10 min, using the previously inserted drainage catheter. This regimen was repeated after 12 and 24 h. The total dose of streptokinase was 1,500,000 IU. RESULTS: The clinical effect of intrapericardial streptokinase administration was excellent. Several days after intrapericardial administration of streptokinase, drainage of purulent pericardial fluid stopped. No complications associated with intrapericardial streptokinase administration were observed. In the follow-up echocardiography (in two patients repeated 6 and 9 months after delivery of streptokinase), pericardial fluid and echocardiographic signs of pericardial constriction were not observed. CONCLUSION: Intrapericardial administration of streptokinase in purulent pericarditis is effective and safe.

The classification of pericardial disease in the age of modern medicine.

The spectrum of pericardial diseases comprises pericarditis, pericardial neoplasms, cysts, and congenital defects. Due to the insufficient diagnostic value of standard, noninvasive diagnostic techniques, many cases remained etiologically unclear, and were therefore classified as idiopathic. A major improvement in the classification of pericardial disease is its clear distinction between the two most frequent forms of idiopathic pericarditis: viral infection and autoreactive pericarditis. This classification has major therapeutic consequences. In autoreactive forms, systemic and intrapericardial corticosteroid treatment has a favorable effect; its application in viral forms is contraindicated. The new classification of pericardial diseases synthesizes the achievements of modern imaging with molecular biology and immunology. Systematic implementation of new techniques of pericardial fluid analyses, pericardioscopy and pericardial biopsy, and the application of molecular biology and immunology techniques have opened new windows to the pericardial diseases, permitting early specific diagnosis, and creating foundations for etiologic treatment in many cases.

Tratamiento de la pericarditis aguda y crónica / Treatment of acute and chronic pericarditis

Hay una gran variedad de agentes infecciosos, físicos, traumáticos, inflamatorios, metabólicos y de otra índole que pueden afectar el pericardio. La reacción del pericardio ante estas diferentes agresiones se traduce en forma de unas manifestaciones clínicas relativamente inespecíficas como son los síntomas inflamatorios propios de la pericarditis aguda, la producción de líquido en el saco pericárdico con la eventual posibilidad de taponamiento cardíaco, y la reacción fibrosa-retráctil del pericardio que puede conducir a la pericarditis constrictiva. Estas manifestaciones no son excluyentes entre sí y pueden coincidir o sucederse en la evolución clínica en un mismo paciente, pero a efectos de claridad de exposición se comentan de forma independiente.El objetivo de este artículo es comentar el tratamiento de las pericarditis agudas y crónicas de una forma sindrómica general completada con los aspectos particulares de las etiologías concretas más frecuentes.Por extensión se comenta el manejo del derrame pericárdico crónico, del taponamiento y de la pericarditis constrictiva. (AU)

A new classification for pericarditis associated with meningococcal infection.

UNLABELLED: Acute meningococcal pericarditis is a rare clinical disorder. Our review of the literature disclosed that current classifications are confusing since they fail to differentiate between two distinct criteria: time and causality. We suggest a new classification of the various states of meningococcal pericarditis on the basis of the pathophysiological process: disseminated meningococcal disease with pericarditis (purulent, culture-positive, associated with meningococcal bacteraemia); isolated meningococcal pericarditis (purulent, culture-positive but without signs of meningeal or other clinical systemic involvement); and reactive meningococcal pericarditis (immunological, late-onset, culture-negative, resembling post-viral pericarditis). It is essential that clinicians recognize the various states of the disease, since they differ in natural history, treatment and prognosis. CONCLUSION: From personal experience and a literature review it emerges that meningococcal pericarditis should be classified as: (1) Pericarditis as local manifestation of disseminated meningococcal disease; (2) isolated meningococcal pericarditis; (3) reactive (immunopathic) meningococcal pericarditis.

[Pericarditis: classification, etiology, pathogenesis]. / Zapalenie osierdzia: klasyfikacja, etiologia, patogeneza.

The most frequent pericardial diseases are inflammations which in surviving patients are recognized much less frequently comparing with autopsy material. The most frequent causes of pericarditis include various infections, inflammations associated with systemic diseases, acute myocardial infarction, traumas and iatrogenic lesions, and neoplasms. Viral etiology is most often in infectious pericarditis. A considerable percentage of idiopathic pericarditis is most probably of such etiology. Bacterial (purulent) pericarditis in contrast to viral one is characterized by high mortality and frequent progression of acute to chronic type. The latter with the symptoms of pericardial constriction occurs in at least 50% of the patients with tuberculous etiology irrespective of specific treatment. In recent years the number of iatrogenic pericarditis has increased related to hypersensitivity or autoimmunity. One should mention here post-cardiotomy syndrome, pacemaker implantation, diagnostic procedures such as biopsy, cardiac catheterization, hypersensitivity to drugs and radiation pericarditis as a complication of therapy.

[Pericarditis as one of the manifestations of periodic disease]. / Perikardit kak odno iz proiavlenii periodicheskoi bolezni.

The authors studied 34 patients with periodic disease. In its thoracal course the development of pericarditis was proved in 1/3 of the patients.