Spectrum of pericardial disease: part II

Pericardial disease is a common disorder seen in varying clinical settings, and may be the first manifestation of an underlying systemic disease. In part I, we focused on the current knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis. In part II, we will focus on the knowledge and management of pericardial involvement in chylous pericardial effusion cholesterol pericarditis, radiation pericarditis, pericardial involvement in systemic inflammatory diseases, autoreactive pericarditis, pericarditis in renal failure, pericardial constriction and effusive constrictive pericarditis.     

Hypothyroidism presenting as acute cardiac tamponade with viral pericarditis

This report describes the case of a young woman who presented to an emergency department with severe abdominal pain and shock. The patient was found to have pericardial tamponade due to a massive pericardial effusion. On further evaluation, the etiology of this effusion was considered to be secondary to hypothyroidism with concominant acute viral pericarditis leading to a fulminant tamponade. The presentation, differential diagnosis, and management of pericardial effusion and tamponade secondary to hypothyroidism and viral pericarditis are discussed. The diagnosis of hypothyroidism in conjunction with acute viral pericarditis should be considered in patients presenting with unexplained pericardial effusion and tamponade.     

MR imaging findings in 76 consecutive surgically proven cases of pericardial disease with CT and pathologic correlation

To describe findings of patients with surgically confirmed pericardial disease on state of the art MR sequences. Retrospective review was performed for patients who underwent pericardiectomy and preoperative MR over a 5 year period ending in 2009. Patients' records were reviewed to confirm the diagnosis of chronic recurrent pericarditis, constrictive pericarditis, or pericardial tumor. MR imaging findings of pericardial thickness, IVC diameter, presence or absence of pericardial or pleural effusion, pericardial edema, pericardial enhancement, and septal "bounce" were recorded. Patients with constriction had a larger IVC diameter (3.1 ± 0.4 cm) than patients with recurrent pain and no constriction (2.0 ± 0.4 cm). Mean pericardial thickness for the 16 patients with chronic recurrent pericarditis but no evidence of constriction was 4.8 ± 2.9 mm. Mean pericardial thickness for patients with constriction was 9.2 ± 7.0 cm with calcification, and 4.6 ± 2.1 cm without calcification. 94% of patients with chronic recurrent pericarditis had gadolinium enhancement of the pericardium, while 76% of patients with constriction had pericardial enhancement. Septal "bounce" was present in 19% of chronic recurrent pericarditis cases and 86% of constriction cases. 5 patients had a pericardial neoplasm, 1 of which was not identified preoperatively. State of the art MR techniques can identify significant and distinct findings in patients with chronic recurrent pericarditis, constrictive pericarditis, and pericardial tumors.     

Pericardial Disease: Diagnosis and Management

Pericardial diseases can present clinically as acute pericarditis, pericardial effusion, cardiac tamponade, and constrictive pericarditis. Patients can subsequently develop chronic or recurrent pericarditis. Structural abnormalities including congenitally absent pericardium and pericardial cysts are usually asymptomatic and are uncommon. Clinicians are often faced with several diagnostic and management questions relating to the various pericardial syndromes: What are the diagnostic criteria for the vast array of pericardial diseases? Which diagnostic tools should be used? Who requires hospitalization and who can be treated as an outpatient? Which medical management strategies have the best evidence base? When should corticosteroids be used? When should surgical pericardiectomy be considered? To identify relevant literature, we searched PubMed and MEDLINE using the keywords diagnosis, treatment, management, acute pericarditis, relapsing or recurrent pericarditis, pericardial effusion, cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy. Studies were selected on the basis of clinical relevance and the impact on clinical practice. This review represents the currently available evidence and the experiences from the pericardial clinic at our institution to help guide the clinician in answering difficult diagnostic and management questions on pericardial diseases.CMR = cardiac magnetic resonance imaging; CT = computed tomography; CYP = cytochrome P450; ECG = electrocardiographic; ESC = European Society of Cardiology; IVC = inferior vena cava; LV = left ventricular; NSAID = nonsteroidal anti-inflammatory drug; RA = right atrium; RV = right ventricleThe pericardium is a thin covering that separates the heart from the remaining mediastinal structures and provides structural support while also having a substantial hemodynamic impact on the heart. The pericardium is not essential—normal cardiac function can be maintained in its absence—however, diseased pericardium presenting clinically as acute or chronic recurrent pericarditis, pericardial effusion, cardiac tamponade, and pericardial constriction can be challenging to manage and life-threatening in some cases. The etiology of pericardial disease is often difficult to determine or remains idiopathic. However, microorganisms, including viruses and bacteria; systemic illnesses, including neoplasia, autoimmune disease, and connective tissue disease; renal failure; previous cardiac surgery; previous myocardial infarction; trauma; aortic dissection; radiation; and, rarely, drugs have been associated with pericardial diseases.The diagnosis and management of pericardial diseases remain challenging because of the vast spectrum of manifestations and the lack of clinical data on which to base guidelines by the American College of Cardiology and the American Heart Association. However, the European Society of Cardiology (ESC) published guidelines on pericardial disease in 2004.¹ This review aims to describe the methods of diagnosing and managing major pericardial syndromes on the basis of the literature and the clinical experience of our pericardial clinic. Searches were performed on PubMed and MEDLINE using the keywords diagnosis, treatment, management, acute pericarditis, relapsing or recurrent pericarditis, pericardial effusion, cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy. No date limitations were set. Studies were selected on the basis of clinical relevance and the impact on clinical practice.     

Spectrum of pericardial disease: part I

Pericardial disease is a common disorder seen in varying clinical settings and may be the first manifestation of an underlying systemic disease. It may be due to multiple causes. Epidemiologic studies are lacking, and the exact incidence and prevalence are unknown. New diagnostic techniques have improved diagnosis, allowing early diagnosis and management. There are few randomized data to guide physicians in the management of pericardial diseases. Part I of our review focuses on the current state of knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis.     

Pericarditis, pericardial effusion, cardiac tamponade, and constriction

Critical care aspects of pericardial disease are covered, including diagnosis and differential diagnosis of acute pericarditis, pericardial effusion with and without cardiac tamponade, constrictive pericarditis and effusive-constrictive pericarditis. Emphasis is placed on clinical signs and the important invasive and noninvasive diagnostic procedures, particularly various imaging methods (emphasis on echocardiography), electrocardiography, and cardiac catheterization. Medical and surgical therapies are reviewed, and the technique of pericardiocentesis is presented.     

Purulent pericarditis misdiagnosed as septic shock

BACKGROUND: Septic shock is common, with approximately 200,000 cases recognized annually. This syndrome is so well characterized that when a patient is febrile and in shock, septic shock may be diagnosed without regard to alternative possibilities. Purulent pericarditis is a relatively rare disorder in which fever and hypotension are common. Classic signs and symptoms, such as chest pain, pericardial friction rub, pulsus paradoxus, and elevation of jugular venous pressure, are seen in only 50%. METHODS: In this report, we describe four patients in whom purulent pericarditis and pericardial tamponade was initially misdiagnosed as septic shock. During a 3-month period, three men and one woman (mean age, 44.5 years) came to Kern Medical Center with purulent pericarditis and pericardial tamponade. These cases represented 13% of patients admitted with a diagnosis of septic shock. RESULTS: All patients were bacteremic, and the classic findings of pericardial tamponade were absent or relatively subtle. Hemodynamic findings of elevated systemic vascular resistance, low cardiac output, and normal pulmonary artery occlusion pressure were critical to the diagnosis. CONCLUSIONS: Consideration of purulent pericarditis is important in cases diagnosed as septic shock. Clinicians should be aware that patients with purulent pericarditis may not exhibit classic signs and symptoms, and a high index of suspicion is necessary for appropriate management.     

MR imaging of the pericardium

Imaging of the pericardium requires understanding of anatomy and the normal and abnormal physiology of the pericardium. MR imaging is well-suited for answering clinical questions regarding suspected pericardial disease. Pericardial diseases that may be effectively imaged with MR imaging include pericarditis, pericardial effusion, cardiac-pericardial tamponade, constrictive pericarditis, pericardial cysts, absence of the pericardium, and pericardial masses. Although benign and malignant primary tumors of the pericardium may be occasionally encountered, the most common etiology of a pericardial mass is metastatic disease.     

CT Imaging of the Pericardium

Pericardial disease can be challenging to diagnose, and imaging can play a useful role in confirming or even suggesting the diagnosis. Computed tomography (CT) is a particularly appealing option for investigating pericardial disease in many patients because the differential diagnosis for symptoms of acute pericarditis or constrictive pericarditis often includes other diseases which are also well assessed with CT. In addition, many patients will have findings of pericardial disease manifest on CT imaging for other suspected diseases, and these findings can be missed if careful attention is not paid to the pericardium. CT also can play an important role in evaluating specific pericardial lesions, such as cysts, tumors, and abscesses. We will review findings of various pericardial diseases on CT with illustrative cases.     

Echocardiographic diagnosis of effusive-constrictive pericarditis due to staphylococcal pericarditis after cardiac surgery

Two weeks after coronary artery bypass surgery, a 43-year-old man was readmitted with fever, pneumonia, left pleural effusion, and pericarditis. Echocardiography showed a localized posterior pericardial effusion, pericardial thickening, and bulging of the ventricular septum toward the left ventricle. Right-sided catheterization indicated pericardial constriction. Effusive-constrictive pericarditis was confirmed at surgery. Cardiac imaging played an important role in diagnosis of this unusual complication of cardiac surgery.     

Acute pericarditis

Although acute pericarditis is most often associated with viral infection, it may also be caused by many diseases, drugs, invasive cardiothoracic procedures, and chest trauma. Diagnosing acute pericarditis is often a process of exclusion. A history of abrupt-onset chest pain, the presence of a pericardial friction rub, and changes on electrocardiography suggest acute pericarditis, as do PR-segment depression and upwardly concave ST-segment elevation. Although highly specific for pericarditis, the pericardial friction rub is often absent or transient. Auscultation during end expiration with the patient sitting up and leaning forward increases the likelihood of observing this physical finding. Echocardiography is recommended for most patients to confirm the diagnosis and to exclude tamponade. Outpatient management of select patients with acute pericarditis is an option. Complications may include pericardial effusion with tamponade, recurrence, and chronic constrictive pericarditis. Use of colchicine as an adjunct to conventional nonsteroidal anti-inflammatory drug therapy for acute viral pericarditis may hasten symptom resolution and reduce recurrences.     

Nocardia asteroides pericarditis: report of a case and review of the literature

In this report, we describe a patient who had purulent Nocardia asteroides pericarditis. In addition, we identified 13 previously suspected and reported cases of Nocardia pericarditis, but only 5 of these studies reported isolation of Nocardia from cultures of pericardial fluid or pericardium. Analysis of the clinical course of these five patients and our patient revealed the importance of long-term sulfonamide antibiotic therapy in combination with surgical pericardial drainage procedures. In our review, only patients who received antibiotics and underwent pericardiectomy survived. Our case substantiates the excellent penetration of sulfisoxazole into the pericardial fluid, even with oral administration of the drug, and provides evidence in support of aggressive management of Nocardia pericarditis.     

A review of pericardial diseases: clinical, ECG and hemodynamic features and management

Pericardial diseases are common, have multiple causes, and are often misdiagnosed. Physicians need to recognize the characteristic and distinguishing features of the three most important pericardial conditions: acute pericarditis, cardiac tamponade, and constrictive pericarditis. In these conditions, proper diagnosis and appropriate management can significantly reduce morbidity and mortality.     

MR diagnosis of herniation of the left ventricle through a pericardial window

The diagnosis of herniation of the left ventricle through a pericardial window was made using MRI. This is a rare type of herniation because it presented 6 years after a pericardial window was made for pericarditis. Herniation of the heart through congenital, traumatic and post surgical pericardial defects are discussed.     

Haemophilus influenzae pericarditis with tamponade as the initial presentation of systemic lupus erythematosus

Although cardiac tamponade is an important and emergent complication of systemic lupus erythematosus (SLE), purulent pericarditis is rare despite the high frequency of pericardial effusion in SLE. We describe the first SLE case of Haemophilus influenzae type-f pericarditis with cardiac tamponade with SLE as the initial presentation. The pathophysiology and therapy are discussed.     

Cardiac tamponade in systemic lupus erythematosus: an unusual initial manifestation

I have described a patient in whom cardiac tamponade occurred as the initial clinical manifestation of SLE. Although pericarditis is a common clinical entity in SLE, cardiac tamponade with this disease is rare. If suspected, the diagnosis can be made by the proper selection of tests of serum and pericardial fluid, which should include the search for pericardial LE cells. This report emphasizes the importance of screening for connective tissue disease in patients with pericarditis.     

Diagnosing tuberculous pericarditis

BACKGROUND: Definitive diagnosis of tuberculous pericarditis requires isolation of the tubercle bacillus from pericardial fluid, but isolating the organism is often difficult. AIM: To improve diagnostic efficiency for tuberculous pericarditis, using available tests. DESIGN: Prospective observational study. METHODS: Consecutive patients (n = 233) presenting with pericardial effusions underwent a predetermined diagnostic work-up. This included (i) clinical examination; (ii) pericardial fluid tests: biochemistry, microbiology, cytology, differential white blood cell (WBC) count, gamma interferon (IFN-gamma), adenosine deaminase (ADA) levels, polymerase chain reaction testing for Mycobacterium tuberculosis; (iii) HIV; (iv) sputum smear and culture; (v) blood biochemistry; and (vi) differential WBC count. A model was developed using 'classification and regression tree' analysis. The cut-off for the total diagnostic index (DI) was optimized using receiver operating characteristic (ROC) curves. RESULTS: Fever, night sweats, weight loss, serum globulin (>40 g/l) and peripheral blood leukocyte count (<10 x 10(9)/l) were independently predictive. The derived prediction model had 86% sensitivity and 84% specificity when applied to the study population. Pericardial fluid IFN-gamma >or=50 pg/ml, concentration had 92% sensitivity, 100% specificity and a positive predictive value (PPV) of 100% for the diagnosis of tuberculous pericarditis; pericardial fluid ADA >or=40 U/l had 87% sensitivity and 89% specificity. A diagnostic model including pericardial ADA, lymphocyte/neutrophil ratio, peripheral leukocyte count and HIV status had 96% sensitivity and 97% specificity; substituting pericardial IFN-gamma for ADA yielded 98% sensitivity and 100% specificity. DISCUSSION: Basic clinical and laboratory features can aid the diagnosis of tuberculous pericarditis. If available, pericardial IFN-gamma is the most useful diagnostic test. Otherwise we propose a prediction model that incorporates pericardial ADA and differential WBC counts.     

Haemophilus influenzae pericarditis successfully treated by catheter drainage

Surgical drainage together with antibiotic therapy is generally considered the treatment of choice for purulent pericarditis. A case of culture-proven Haemophilus influenzae pericarditis is described in a young, previously healthy adult. Successful management of his illness included placement of an indwelling pericardial catheter and intravenous antibiotics. Pericardial catheter drainage may be an alternative to surgical drainage in some cases of purulent pericarditis.     

Diagnostic issues in the clinical management of pericarditis

Aims: To review the current major diagnostic issues on the diagnosis of acute and recurrent pericarditis. Methods: To review the current available evidence, we performed a through search of several evidence‐based sources of information, including Cochrane Database of Systematic Reviews, Clinical Evidence, Evidence‐based guidelines from National Guidelines Clearinghouse and a comprehensive Medline search with the MeSH terms ‘pericarditis’, ‘etiology’ and ‘diagnosis’. Results: The diagnosis of pericarditis is based on clinical criteria including symptoms, presence of specific physical findings (rubs), electrocardiographical changes and pericardial effusion. Although the aetiology may be varied, most cases are idiopathic or viral, even after an extensive diagnostic evaluation. In such cases, the course is often benign following anti‐inflammatory treatment, and management would be not affected by a more precise diagnostic evaluation. A triage of pericarditis can be safely performed on the basis of the clinical and echocardiographical presentation. Specific diagnostic tests are not warranted if no specific aetiologies are suspected on the basis of the epidemiological background, history and presentation. High‐risk features associated with specific aetiologies or complications include: fever > 38 °C, subacute onset, large pericardial effusion, cardiac tamponade, lack of response to aspirin or a NSAID. Conclusions: A targeted diagnostic evaluation is warranted in acute and recurrent pericarditis, with a specific aetiological search to rule out tuberculous, purulent or neoplastic pericarditis, as well as pericarditis related to a systemic disease, in selected patients according to the epidemiological background, presentation and clinical suspicion.     

Primary pneumococcal pericarditis complicated by acute constriction

We report a case of a 31-year-old male with primary pneumococcal pericarditis. Severe acute constrictive pericarditis developed within a month of the onset of the illness in spite of adequate antibiotic therapy and pericardial drainage.