Acute Pericarditis

Practice Essentials

Acute pericarditis is an inflammation of the pericardium characterized by chest pain, pericardial friction rub, and serial ECG changes.

Signs and symptoms

Chest pain is the cardinal symptom of pericarditis, usually precordial or retrosternal with referral to the trapezius ridge, neck, left shoulder, or arm. Common associated signs and symptoms include low-grade intermittent fever, dyspnea/tachypnea (a frequent complaint and may be severe, with myocarditis, pericarditis, and cardiac tamponade), cough, and dysphagia. In tuberculous pericarditis, fever, night sweats, and weight loss are commonly noted (80%).

Specific causes of pericarditis include the following:

Idiopathic causesInfectious conditions, such as viral, bacterial, and tuberculous infectionsInflammatory disorders, such as RA, SLE, scleroderma, and rheumatic feverMetabolic disorders, such as renal failure, hypothyroidism, and hypercholesterolemiaCardiovascular disorders, such as acute MI, Dressler syndrome, and aortic dissectionMiscellaneous causes, such as iatrogenic, neoplasms, drugs, irradiation, cardiovascular procedures, and trauma

See Clinical Presentation for more detail.

Diagnosis

Initial evaluation includes a clinical history and physical examination, ECG, echocardiography, chest radiography, and lab studies.

ECG can be diagnostic in acute pericarditis and typically shows ST elevation in all leads. The ratio of the amplitude of ST segment to the amplitude of the T wave in leads I, V4, V5, and V6 on electrocardiogram can be used to differentiate acute pericarditis (AP) from early repolarization (ER) and early repolarization of left ventricular hypertrophy (ERLVH), according to a recent study. When ST elevation was present in lead I, the ST/T ratio had the best predictive value for discriminating between AP, ER and ERLVH. The study involved 25 patients with AP, 27 with ER, and 28 with ERLVH.[1]

Echocardiography is particularly helpful if pericardial effusion is suspected on clinical or radiographic grounds, the illness lasts longer than 1 week, or myocarditis or purulent pericarditis is suspected.

A chest radiograph is only helpful for diagnosis in patients with effusions >250mL. Patients with small effusions (less than a few hundred milliliters) may present with a normal cardiac silhouette.

Lab tests may include CBC; serum electrolyte, blood urea nitrogen (BUN), and creatinine levels; erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels; and cardiac biomarker measurements, lactate dehydrogenase (LDH), and serum glutamic-oxaloacetic transaminase (SGOT; AST) levels.

See Workup for more detail.

Management

Treatment for specific causes of pericarditis is directed according to the underlying cause. For patients with idiopathic or viral pericarditis, therapy is directed at symptom relief.

Pharmacologic treatment

Nonsteroidal anti-inflammatory drugs (NSAIDs) are the mainstay of therapy. These agents have a similar efficacy, with relief of chest pain in about 85-90% of patients within days of treatment. A full-dose NSAID should be used, and treatment should last 7-14 days.

Colchicine, alone or in combination with an NSAID, can be considered for patients with recurrent or continued symptoms beyond 14 days.[2]

Corticosteroids should not be used for initial treatment of pericarditis unless it is indicated for the underlying disease, the patient’s condition has no response to NSAIDs or colchicine, or both agents are contraindicated.

Surgical treatment

Surgical procedures for pericarditis include pericardiectomy, pericardiocentesis, pericardial window placement, and pericardiotomy.

Pericardiectomy is the most effective surgical procedure for managing large effusions, because it has the lowest associated risk of recurrent effusions. This procedure is used for constrictive pericarditis, effusive pericarditis, or recurrent pericarditis with multiple attacks, steroid dependence, and/or intolerance to other medical management.

Patients with effusions larger than 250 mL, effusions in which size increases despite intensive dialysis for 10-14 days, or effusions with evidence of tamponade are candidates for pericardiocentesis.

Pericardial window placement is used for effusive pericarditis therapy. In critically ill patients, a balloon catheter may be used to create a pericardial window, in which only 9 cm2 or less of pericardium is resected.

Consider subxiphoid pericardiotomy for large effusions that do not resolve. This procedure may be performed under local anesthesia and has a lower risk of complications than pericardiectomy.

See Treatment and Medication for more detail.

Image libraryThis 12-lead electrocardiogram is representative of pericarditis. NextBackground

Acute pericarditis is an inflammation of the pericardium characterized by chest pain, pericardial friction rub, and serial electrocardiographic (ECG) changes (see an example of such an ECG below). Pericarditis and cardiac tamponade involve the potential space surrounding the heart or pericardium; pericarditis is one cause of fluid accumulation in this potential space, and cardiac tamponade is the hemodynamic result of fluid accumulation.

This 12-lead electrocardiogram is representative of pericarditis.

For more information, see the the Medscape Reference articles Constrictive Pericarditis, Constrictive-Effusive Pericarditis, Pediatric Infective Pericarditis, and Imaging in Constrictive Pericarditis.

For patient education information, see the Cholesterol Center and Heart Center, as well as Pericarditis, Heart Attack, and Chest Pain.

PreviousNextAnatomy

The pericardium (pericardial complex) serves as a protective barrier from the spread of infection or inflammation from adjacent structures. It is composed of the parietal pericardium (an outer fibrous layer) and the visceral pericardium (an inner serous membrane made of a single layer of mesothelial cells). The fibrous pericardium is a flask-shaped, tough outer sac with attachments to the diaphragm, sternum, and costal cartilage. The visceral pericardium is thin, adjacent to the surface of the heart, and attached to the epicardial fat; it reflects back on itself to form the parietal pericardium.

The pericardium normally contains as much as 20-50 mL of an ultrafiltrate of plasma. Approximately 90-120 mL of additional pericardial fluid can accumulate in the pericardium without an increase in pressure. The capacity of the atria and ventricles to fill is mechanically compromised with further fluid accumulation, which can result in marked increases in pericardial pressure, eliciting reduced stroke volume, decreased cardiac output, and hypotension (cardiac tamponade physiology). The rapidity of fluid accumulation influences the hemodynamic effect. Drainage occurs via the thoracic duct and the right lymphatic duct into the right pleural space.

PreviousNextPathophysiology

Pericardial physiology includes 3 main functions. First, through its mechanical function, the pericardium promotes cardiac efficiency by limiting acute dilation, maintaining ventricular compliance with preservation of the Starling curve, and distributing hydrostatic forces. The pericardium also creates a closed chamber with subatmospheric pressure that aids atrial filling and lowers transmural cardiac pressures. Second, through its membranous function, the pericardium shields the heart by reducing external friction and acting as a barrier against extension of infection and malignancy. Third, through its ligamentous function, the pericardium anatomically fixes the heart.

In most cases of acute pericarditis, the pericardium is acutely inflamed and has an infiltration of polymorphonuclear (PMN) leukocytes and pericardial vascularization. Often, the pericardium manifests a fibrinous reaction with exudates and adhesions. The pericardium may develop a serous or hemorrhagic effusion. A granulomatous pericarditis occurs with tuberculosis, fungal infections, rheumatoid arthritis (RA), and sarcoidosis.

Uremic pericarditis is thought to result from inflammation of the visceral and parietal layers of the pericardium by metabolic toxins that accumulate in the body owing to kidney failure. Other factors may be involved, however, because pericarditis also may occur in patients with chronic renal failure who are already receiving dialysis therapy.

The putative toxins suggested to precipitate uremic pericarditis when they accumulate are poorly characterized, but they may include urea, creatinine, methylguanidine, guanidinoacetate, parathyroid hormone, beta2-microglobulin, uric acid, and others. More than one toxin apparently may be involved, although considerable controversy surrounds this point.

The precise pathogenetic changes induced by these toxins when causing uremic pericarditis have not been elucidated, although a rough correlation with the degree and the duration of azotemia exists; the blood urea nitrogen (BUN) level is usually greater than 60 mg/dL (22 mmol/L). Uremic pericarditis may be associated with hemorrhagic or serous effusion, although considerable overlap exists. Hemorrhagic effusions are more common and result in part from uremia-induced platelet dysfunction.

Some authors distinguish between 2 types of pericarditis in patients with renal failure. One type is uremic pericarditis, which occurs in patients with uremia who have never received dialysis. The other type is dialysis-associated pericarditis, which occurs in patients who are already receiving dialysis. In the latter case, inadequate dialysis may usually be implicated, because aggressive dialysis often leads to resolution. Other causes of dialysis-associated pericarditis may include volume overload and bacterial or viral infections.

In an observational study that employed data from 88 maintenance hemodialysis patients, investigators found that intensive dialysis is the most effective treatment for dialysis-associated pericarditis in patients on dialysis who have diabetes and those who do not.[3] Following the intensification of hemodialysis, pericarditis improved in 85.1% of patients with diabetes and in 82.9% of those without diabetes. Among patients with diabetes, 85.1% survived without recurrence of pericarditis, 4.3% survived but did suffer recurrence, and 10.6% died, with similar outcomes recorded in the group without diabetes (87.8%, 4.9%, and 7.3%, respectively).[3]

PreviousNextEtiology

This section will first briefly discuss acute pericarditis, chronic pericarditis, and cardiac tamponade; then, several specific entities that cause pericarditis will be briefly reviewed.

Acute pericarditis

Serous pericarditis is usually caused by noninfectious inflammation such as occurs in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Fibrous adhesions rarely occur.

Fibrous and serofibrinous pericarditis represent the same basic process and are the most frequent type of pericarditis. Common causes include acute myocardial infarction (MI), postinfarction (including Dressler syndrome), uremia, radiation, RA, SLE, and trauma. Severe infections may also cause a fibrinous reaction, as does routine cardiac surgery.

Purulent or suppurative pericarditis due to causative organisms may arise from direct extension, hematogenous seeding, or lymphatic extension, or by direct introduction during cardiotomy. Immunosuppression facilitates this condition. Clinical features include fever, chills, and spiking temperatures. Constrictive pericarditis is a serious potential complication.

Hemorrhagic pericarditis involves blood mixed with a fibrinous or suppurative effusion, and it is most commonly caused by tuberculosis or direct neoplastic invasion. This condition can also occur in severe bacterial infections or in patients with a bleeding diathesis. Hemorrhagic pericarditis is common after cardiac surgery and may cause tamponade. The clinical significance is similar to suppurative pericarditis.

Until proven otherwise, caseation within the pericardial sac is tuberculous in origin. Untreated, caseous pericarditis is the most common antecedent to chronic constrictive pericarditis of a fibrocalcific nature.

Chronic pericarditis

Adhesive mediastinopericarditis is a reaction that usually follows suppurative or caseous pericarditis, cardiac surgery, or irradiation. This condition is rarely caused by a simple fibrinous exudate. The pericardial potential space is obliterated, and adhesion of the external surface of the parietal layer to surrounding structures occurs. Clinically, systolic contraction of the ribcage and diaphragm and pulsus paradoxus may be observed. The increased workload may cause massive cardiac hypertrophy and dilatation, which can mimic an idiopathic cardiomyopathy.

Constrictive pericarditis is usually caused by suppurative, caseous, or hemorrhagic pericarditis. The heart may become encased in a 0.5-cm–thick to 1-cm–thick layer of scar or calcification (concretio cordis), resembling a plaster mold. Contrary to clinical findings in adhesive mediastinopericarditis, the heart cannot become hypertrophic or dilate because of insufficient space.

Imazio et al suggest that constrictive pericarditis is a rare complication of viral or idiopathic acute pericarditis ([4]

Cardiac tamponade

Tamponade is more common in patients with malignant pericarditis. Effusions caused by tumors often progress to tamponade, eliciting bleeding in the pericardium. Blood accumulates more rapidly than a transudate or exudate and more commonly causes tamponade.

Identification of any pericardial fluid in the setting of penetrating injury to the thorax or upper abdomen requires aggressive resuscitation; penetrating cardiac injuries may occur, with hemopericardium as the most common feature. In acute massive hemopericardium, the time is insufficient for defibrination to occur. The hemopericardium organizes and may partially clot, resulting in a pericardial hematoma. The hematoma may appear echogenic instead of echo free.

Potential sources of iatrogenic cardiac perforation include central line placement, pacemaker insertion, cardiac catheterization, sternal bone marrow biopsies, and pericardiocentesis. The right atrium is the most common site of perforation from catheter placement. Perforation, as well as direct catheter infusion of fluids, can cause tamponade. In fact, a tamponade delay of hours to days has occurred secondary to catheter misplacement.

In one case report, tamponade was described as the first manifestation of dermatopolymyositis.[5]

Specific causes of pericarditis include the following and are briefly reviewed below:

Idiopathic causesInfectious conditions, such as viral, bacterial, and tuberculous infectionsInflammatory disorders, such as RA, SLE, scleroderma, and rheumatic feverMetabolic disorders, such as renal failure, hypothyroidism, and hypercholesterolemiaCardiovascular disorders, such as acute MI, Dressler syndrome, and aortic dissectionMiscellaneous causes, such as iatrogenic, neoplasms, drugs, irradiation, cardiovascular procedures, and traumaIdiopathic causes

Between 26% and 86% of cases of acute pericarditis are idiopathic in nature.[6] No clinical features distinguish idiopathic cases from viral pericarditis. It is likely that most idiopathic cases are undiagnosed viral infections. Seasonal peaks occur in spring and fall.

Chronic idiopathic pericarditis is defined as a pericardial effusion that persists more than 3 months without any apparent etiology. Pericardiocentesis alone results in resolution of large effusions; however, recurrence is common.

Viral infection

Viral infection is the most common cause of acute pericarditis and accounts for 1-10% of cases. The disease is usually a short self-limited disease that lasts 1-3 weeks and can occur as seasonal epidemics, especially coxsackievirus B and influenza.

Causative viruses include coxsackievirus B,[7] echovirus, adenoviruses, influenza A and B viruses, enterovirus, mumps virus, Epstein-Barr virus, human immunodeficiency virus (HIV), herpes simplex virus (HSV) type 1, varicella-zoster virus (VZV), measles virus, parainfluenza virus (PIV) type 2, and respiratory syncytial virus (RSV), cytomegalovirus (CMV), and hepatitis viruses A, B, and C (HAV, HBV, HCV, respectively).

Patients may have associated myocarditis. Pericardial involvement is frequent in persons with HIV, but is usually an asymptomatic pericardial effusion of small volume. Individuals with advanced HIV infection develop pericardial involvement more frequently, with one study noting right atrial diastolic compression in 5% of cases involving advanced HIV infection.[8] Symptomatic pericarditis occurs in less than 1% of cases involving HIV, and its etiology can include the usual causes, opportunistic infection, Kaposi sarcoma, and HIV.

Bacterial infection

Bacterial infections accounts for 1-8% of pericarditis cases and result from direct pulmonary extension, hematogenous spread, myocardial abscess or endocarditis, penetrating injury to chest wall from either trauma or surgery, or a subdiaphragmatic suppurative lesion. Purulent pericarditis may result from previous aseptic pericarditis, and a high percentage of patients develop constrictive pericarditis.

Organisms that have been isolated include gram-positive species such as Streptococcus pneumoniae and other Streptococcus species and Staphylococcus.[9] Isolated gram-negative species include Proteus, Escherichia coli, Pseudomonas, Klebsiella, Salmonella, Shigella, Neisseria meningitidis, and Haemophilus influenzae.

Less common organisms include Legionella, Nocardia, Actinobacillus, Rickettsia, Borrelia burgdorferi (Lyme borreliosis), Listeria, Leptospira, Chlamydophila psittaci, and Treponema pallidum (syphilis).

Anaerobes have also been isolated in 40% of patients in reviews of the pediatric population.

Previously, Pneumococcus was the predominant organism. However, in the antibiotic era, staphylococcal and gram-negative species have become more common. Most cases are now associated with thoracic surgery, renal disease, and immunosuppression.

Tuberculous infection

Tuberculosis accounts for 4% of cases and should be considered in all instances of pericarditis without a rapid course, especially in high-risk groups, such as elderly patients in nursing homes and those with acquired immunodeficiency syndrome (AIDS).[10] Approximately 50% of affected patients develop constrictive pericarditis.

Fungal and parasitic infection

Fungal organisms that may cause acute pericarditis include Histoplasma, Blastomyces, Coccidioides, Aspergillus, and Candida. Parasitic organisms include Entamoeba, Echinococcus, and Toxoplasma.

Rheumatoid arthritis

Pericarditis occurs predominantly in males with severely destructive and nodular RA. The pericardial involvement is usually clinically silent, with the diagnosis made in only 2% of adults and 6% of juveniles with RA. Rarely, pericarditis precedes the onset of RA. Autopsy studies show a pericarditis prevalence of 11-50%.

Systemic lupus erythematosus, scleroderma, sarcoidosis

Clinically evident pericarditis has been reported in 25% of patients with SLE and usually occurs in lupus flare-ups, but it may be the presenting manifestation. Autopsy series reveal pericardial involvement in 62% of lupus patients.

Pericarditis is recognized in 5-10% of patients with scleroderma, with a 70% autopsy prevalence. Pericardial effusions occur in 40% of patients with scleroderma and can be due to scleroderma, myocardial failure (restrictive cardiomyopathy), and renal failure. Restrictive cardiomyopathy and pericardial constriction can coexist. Usually, pulmonary hypertension, right heart failure, and systolic dysfunction occur.

Sarcoidosis may result in pericarditis, but this condition rarely causes cardiac tamponade or constrictive pericarditis

Rheumatic fever

Pericarditis in those with rheumatic fever occurs more commonly in lower socioeconomic groups and in children, often accompanying endocarditis and myocarditis, with a worse prognosis. Consider rheumatic fever as an etiology in any child with pericarditis. However, this disease is not a demonstrated cause of constrictive pericarditis.

In adults, pericarditis may not occur with myocardial or valvular involvement, and it is associated with a better prognosis. The pericarditis usually appears 7-10 days after the onset of fever and arthritis. Often, stage 1 electrocardiographic (ECG) findings are absent (see Electrocardiography).

Other inflammatory conditions

The following conditions may also cause acute pericarditis:

Sjögren syndromeMixed connective-tissue diseaseReiter syndromeAnkylosing spondylitisInflammatory bowel diseaseWegener granulomatosisVasculitis (eg, giant cell arteritis, polyarteritis)PolymyositisBehçet syndromeWhipple diseaseFamilial Mediterranean feverSerum sicknessRenal failure

Richard Bright described uremic pericarditis in 1836. Since that classic description, this common complication of chronic renal failure has evolved from an ominous event heralding the terminal stages of disease to an event that, with early management, is likely to have a good outcome. Furthermore, advances in dialysis technology with early and timely management of chronic renal failure have dramatically reduced the prevalence of uremic pericarditis. Uremic pericarditis has a prevalence of 6-10% in patients with acute or chronic renal failure, and it continues to be associated with significant morbidity and occasional mortality.

Renal failure accounts for approximately 12% of cases of pericarditis. In the predialysis era, pericarditis developed in 35-50% of patients with uremia who had chronic renal failure and less commonly in those with acute renal failure. Death often followed in several weeks. With dialysis, the pericarditis incidence rate is less than 10%; however, this condition occurs after the onset of dialysis in 8-12% of cases.

Asymptomatic pericardial effusions can occur in 36-62% of patients with uremia who require dialysis; these effusions are often small to moderate in size and can occur secondary to volume overload. Pericardial effusions can lead to significant hemodynamic complications during routine dialysis. Moreover, the presence of a large pericardial effusion that persists for longer than 10 days after intensive dialysis has a high likelihood of causing tamponade.

Hypothyroidism

Hypothyroidism accounts for as many as 4% of pericarditis cases. In fact, myocardial involvement is common, and pericardial involvement usually occurs with severe hypothyroidism. Patients may develop large pericardial effusions, but they rarely develop tamponade.

Cholesterol pericarditis

Cholesterol pericarditis, also called gold-paint pericarditis, is a complication of a chronic pericardial effusion exacerbated by cholesterol crystals. It usually presents with large effusions that are not hemodynamically important, and development of constriction is rare. Granulomatous pericarditis has been implicated in some cases.

Myocardial infarction

After a transmural infarction, a fibrinous pericardial exudate appears within 24 hours, begins to organize at 4-8 days, and completes organization at 4 weeks.[11, 12] Pericardial pain occurs less frequently than the friction rub, which is often detected on the second or third day after an acute MI but may be heard within 24 hours and as late as 10 days.

Before thrombolytic therapy, infarct-associated pericarditis ranges from 7% to 23% of cases. At autopsy in one study, almost all patients were noted to have localized fibrinous pericarditis overlying the area of infarction. With thrombolytic therapy and direct infarct angioplasty, the incidence of post–MI-associated pericarditis has decreased to 5-8%.

Overall, pericardial involvement indicates a larger infarction, greater incidence of left ventricular dysfunction, and greater mortality. The pericarditis usually heals without consequence; effusions may occur, but they rarely lead to tamponade.

Dressler syndrome

Dressler syndrome is now considered rare. When pericarditis associated with Dressler syndrome does occur, it is usually observed 2-3 weeks after a myocardial infarction. Initially, the syndrome was described in as many as 4% of patients following and acute MI. Later studies suggested a much lower incidence. Dressler syndrome is rarely described with pulmonary embolism.

This syndrome may be a unique autoimmune-mediated phenomenon to myocardial antigens, or it may merely be an unrecognized post–MI pericarditis. Patients may develop pulmonary infiltrates and large pericardial effusions.

Because of the risk of hemorrhagic pericarditis, anticoagulant therapy should be stopped in patients with Dressler syndrome.

Aortic dissection and Takotsubo cardiomyopathy

Aortic dissection accounts for 1% of cases of acute pericarditis, especially for cases with hemorrhage into the pericardium.

Takotsubo cardiomyopathy is a transient cardiac syndrome that involves left ventricular apical akinesis and mimics acute coronary syndrome.

Neoplasm

Malignancy account for 5-17% of pericarditis cases; in patients presenting with acute pericarditis or pericardial effusion, 4-7% have an unsuspected malignancy. Primary neoplasm of the heart and pericardium is rare; most cases of neoplasm-related pericarditis are a result of metastatic disease. Autopsy studies have noted that approximately 10% of patients with cancer develop cardiac involvement, and it is often clinically silent. The neoplastic cells reach the pericardium through the bloodstream, through the lymphatic system, or via local growth.

Neoplastic disease, particularly advanced disease, is the most frequent cause of tamponade in the hospital. Occasionally, the tumor encases the heart and causes constrictive pericarditis rather than tamponade.

Pericardial mesothelioma and angiosarcoma are lethal malignancies with aggressive local spread that respond poorly to treatment. Infants and children can present with a teratoma in the pericardial space. These can often be successfully removed.

Lung cancer, including adenocarcinoma and squamous and small cell carcinoma, accounts for approximately 33% of cases; breast cancer accounts for 25%; leukemia and lymphoma, including Hodgkin and non-Hodgkin, account for 15% of cases; and malignant melanoma represents another 5%. Almost all other malignancies, except primary brain, comprise the rest of the cases. Kaposi sarcoma has also become a more prominent cause of neoplastic disease with the AIDS epidemic.

Drugs

Some medications, including penicillin and cromolyn sodium, induce pericarditis through a hypersensitivity reaction. The anthracycline antineoplastic agents, such as doxorubicin and cyclophosphamide, have direct cardiac toxicity and can cause acute pericarditis and myocarditis.

Pericarditis can also develop from a drug-induced lupus syndrome caused by medications including procainamide, hydralazine, methyldopa, isoniazid, mesalazine, and reserpine. Methysergide causes constrictive pericarditis through mediastinal fibrosis. Dantrolene, phenytoin, and minoxidil produce pericarditis through an unknown mechanism.

Smallpox vaccination infrequently leads to myocarditis. In a review of a large vaccination program in the US military, approximately 12 per 100,000 vaccinated troops developed myopericarditis within 14 days of vaccination.[13, 14] Whether this was due to a direct viral cytopathic effect or an immune-mediated phenomenon is unclear.

Irradiation

Pericardial disease is the most common cardiac toxicity from radiation therapy. Others are coronary artery disease, conduction disturbance, and myocardial and valvular disease.[15] A high incidence of such toxicity occurs with high doses, especially those greater than 4000 rad.

Radiation pericarditis can present as acute pericarditis, with or without effusion; chronic constrictive pericarditis; or effusive-constrictive pericarditis.

Invasive cardiac procedures

Electrophysiologic studies, radiofrequency ablation, pacemaker implantation, and percutaneous coronary intervention are among several invasive cardiac procedures that can cause pericarditis.

Postpericardiotomy syndrome is similar to Dressler syndrome, except that postpericardiotomy syndrome occurs after cardiac surgery. Several series note an incidence rate of 10-40%; approximately 1% of patients with postpericardiotomy syndrome develop tamponade.

Pericardial effusions can occur in the absence of typical features of postpericardiotomy syndrome. In one study, 56% developed pericardial effusions early after cardiac surgery, without correlation to pericarditis or tamponade. The effusions were more common after heavy postoperative bleeding.

Trauma

Approximately 1% of cases of acute pericarditis are caused by trauma, such as penetrating and nonpenetrating cardiac trauma. Also consider esophageal rupture or perforation and pancreatitis.

PreviousNextEpidemiology

Epidemiologic data on the incidence of acute pericarditis are lacking, likely because this condition is frequently inapparent clinically, despite its presence in numerous disorders. Lorell noted a diagnosis of acute pericarditis in approximately 1 per 1000 hospital admissions.[16] In addition, acute pericarditis comprises 1% of emergency room visits in patients with ST-segment elevation.[17] In fact, the reported incidence of acute pericardial tamponade is approximately 2% of penetrating trauma; however, this condition is rarely seen in blunt chest trauma.

Uremic pericarditis may occur in 6-10% of patients with advanced renal failure before initiation of dialysis. When patients with large effusions are studied, uremia may account for up to 20% of cases in some series. The widespread availability of dialysis has reduced the incidence of uremic pericarditis.

Malignant disease is the most common cause of pericardial effusion with tamponade in developed countries; However, tuberculosis should be considered in endemic areas.

Acute pericarditis is more common in men than in women. However, although this condition is more common in adults than in children, adolescents are more commonly affected than young adults. Nonetheless, Merce et al found no difference in etiology, clinical course, and prognosis between elderly and younger patients with moderate and large pericardial effusions.[18]

PreviousNextPrognosis

The prognosis in individuals with pericarditis depends on the etiology of this condition, as well as the presence of a pericardial effusion and/or tamponade. Idiopathic and viral etiologies usually have a self-limited course, without any risk of evolution toward constrictive pericarditis.[19, 20] Most post–MI cases have a benign course; however, pericarditis is associated with larger infarcts, and therefore, overall long-term mortality may be increased.

Patients with scleroderma or children with rheumatic fever and pericarditis have a poor prognosis, and purulent, tuberculous, and neoplastic pericardial involvement have more complicated courses with worse outcomes. Purulent pericarditis is associated with a mortality rate nearing 100% for untreated persons and a mortality rate of 12-40% for treated patients. The mortality rate in tuberculous pericarditis approaches 50%.

Uremic pericarditis continues to be associated with significant morbidity and occasional mortality. Of patients with uremic pericarditis, 3-5% may develop hemorrhagic pericarditis.

For penetrating injuries, the prognosis depends heavily on the rapid identification of tamponade. Mortality may occur in 3-5% of cases resulting from cardiac tamponade or arrhythmias. Favorable factors include minor perforations, isolated right ventricular wounds, systolic blood pressure more than 50 mm Hg, and the presence of tamponade.

PreviousProceed to Clinical Presentation , Acute Pericarditis

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Constrictive-Effusive Pericarditis

Overview

Effusive-constrictive pericarditis is a rare clinical syndrome characterized by concurrent pericardial effusion and pericardial constriction, with constrictive hemodynamics being persistent after the pericardial effusion is removed. The mechanism of effusive-constrictive pericarditis is thought to be visceral pericardial constriction. Pericardial effusions vary in size and age and may be transudative, exudative, sanguineous, or chylous. An effusion persisting for months to years may evolve into effusive-constrictive pericarditis.[1, 2, 3, 4, 5, 6, 7, 8, 9, 10]

Patients with effusive-constrictive pericarditis may present with symptoms caused by a limitation of intercardiac end-diastolic volume. These findings are secondary not only to the pericardial effusion but also to the pericardial constriction. Symptoms, as well as history and physical findings, vary, and a moderate to large pericardial effusion may occur.

The effusive-constrictive variant of pericarditis was first described in the 1960s. Hancock popularized this definition of a constrictive physiology with a coexisting pericardial effusion.[3]

In 2004, Sagrista-Sauldea et al reported on 15 subjects from Barcelona, Spain who were identified as having effusive-constrictive pericarditis.[11] These individuals were among 190 consecutive subjects with clinical tamponade who underwent pericardiocentesis and concurrent catheterization. The etiologies of the effusive-constrictive pericarditis were infectious causes, irradiation, cardiac surgery, and idiopathic. Consistent with Hancock's data, Sagrista-Sauldea reported that most cases were due to idiopathic factors.

Pericardial effusion

The pericardium consists of 2 layers, a parietal layer and a visceral layer. The visceral pericardium is composed of 1 or 2 cell layers of mesothelial cells and adheres closely with the epicardium. The parietal pericardium is separated from the visceral pericardium by a small amount of fluid that serves as a lubricant. Any supraphysiologic accumulation of this fluid is identified as a pericardial effusion.[1, 2, 12, 13, 14] In general, a pericardial effusion should be evaluated to determine its etiology and hemodynamic significance.

Hemodynamics

Jugular venous and arterial pressures may be within the reference range, with or without signs of cardiac tamponade. Effusive-constrictive pericarditis is believed to evolve as part of a clinical continuum initiated by pericarditis or a pericardial effusion; thus, its etiologies mirror those of pericarditis, pericardial tamponade, and chronic constrictive pericarditis.

The hemodynamic definition of this syndrome is the continued elevation of right atrial, end-diastolic right ventricular, and left ventricular diastolic pressures after the removal of pericardial fluid returns the pericardial pressure to zero (or near zero).[1, 3, 11]

Treatment considerations

Recognition of effusive-constrictive pericarditis is clinically important because treatment with pericardiocentesis or a pericardial window may be inadequate; this is because neither treatment would address the visceral pericardium. Rather, a visceral pericardiectomy may be indicated for optimal therapy since it is the visceral pericardium that is constricting.

Importantly, however, not all cases of effusive-constrictive pericarditis progress to chronic constrictive pericarditis. In some clinical situations, relief from the effusion can be obtained by means of pericardiocentesis or a pericardial window, with medical treatment being used to manage the underlying condition. The constriction may be transitory and surgical pericardiectomy may be avoided. These situations usually occur in the first months of a chronic effusion and close monitoring is required.

Patient education

Although the symptoms of effusive-constriction are nonspecific, patients should be counseled to report any new or worsened dyspnea, ascites, weight loss or gain, peripheral edema, fever, or chest pain or pressure.

NextPathophysiology

Constrictive pericarditis and cardiac tamponade both restrict filling of the cardiac chambers, thereby increasing systemic and pulmonary filling pressures. In tamponade, single forward flow occurs during systole (prominent x descent in atrial pressure tracings), whereas in constriction, a biphasic pressure tracing is greater during diastole (prominent y descent).

Patients with effusive-constrictive pericarditis may have tamponade-like pressure tracings, which change to constrictive-like tracings after pericardiocentesis. This is because the visceral, rather than the parietal, pericardium is constrictive.

In rare cases, a loculated effusion may lead to constriction with regional tamponade of 1 or more cardiac chambers. Almost any form of chronic pericardial effusion has the potential to organize into an effusive-constrictive state even though the absolute number of cases is relatively low.[4]

Effusive-constrictive pericarditis may be part of a clinical continuum. Stages of infective pericarditis have been observed that range from acute pericarditis and tamponade with effusion to constrictive pericarditis without effusion. Effusive-constrictive pericarditis is likely a middle phase in this evolution. Therefore, suspicion for this entity should be high in cases of indolent, subacute pericarditis, as well in cases of chronic pericardial effusion.

PreviousNextEtiology

Effusive-constrictive pericarditis likely occurs at any point along a clinical continuum that ranges from the occurrence of an effusion to the development of chronic pericardial constriction. Leading causes of effusive-constrictive pericarditis include the following:

Idiopathic factorsIrradiationCardiac surgeryNeoplasm - Most commonly lung, breast, or hematologicInfectious disease - Particularly in immunocompromised states (most commonly tuberculosis and fungal disease, although Streptococcus species have been reported)[15, 16] Myocardial infiltrationConnective tissue diseaseUremia

Cases of effusive-constrictive pericarditis in the United States are most often secondary to irradiation, cardiac surgery, uremia, or malignancy or are idiopathic.[6] In developing countries, the disorder is usually secondary to infectious causes (eg, tuberculosis).[17] In a prospective study of 1184 patients with pericarditis, Sagrista-Sauldea et al reported that 6.9% of 218 patients with tamponade had confirmed effusive-constrictive pericarditis.[11]

The disorder’s etiology can often be suspected from the clinical setting in which the effusion occurs. The differential diagnosis of effusive-constrictive pericarditis requires a consideration of all of the causes for pericardial effusions and pericardial tamponade and then a determination of whether a particular patient has constrictive physiology.

PreviousNextPrognosis

Mortality associated with effusive-constrictive disease is directly related to its etiology. For example, patients with metastatic carcinoma in the pericardial space usually have a prognosis that is much poorer than that of patients with postviral or idiopathic pericardial effusion with constriction. Noncardiac metastatic effusions are often end-stage, with reported mortality rates of 47% and 80% at 3 and 6 months, respectively.

Constrictive physiology increases the risk of morbidity in patients with effusive-constrictive pericarditis, but no definitive statistics are available.

The most effective therapy for effusive-constrictive pericarditis is pericardiectomy with complete removal of the parietal and visceral membranes. However, the perioperative mortality rate for this procedure can be high. Indeed, only experienced surgeons should undertake visceral pericardiectomy.[18]

When visceral pericardiectomy is not chosen as the plan of care, the underlying disease may progress and cause recurrent and/or worsening effusive-constrictive syndrome or constrictive pericarditis.

PreviousNextPatient History

Symptoms of effusive-constrictive pericarditis can be hard to interpret but may include atypical or typical chest pain, chest heaviness, or pressure. Other symptoms include dyspnea on exertion, fatigability, or peripheral edema.

Many patients are asymptomatic until the advanced disease stages. In more severe cases, impaired mental status may be evident as a result of decreased cardiac output.

Specific etiologies of effusive-constrictive pericarditis may have characteristic antecedent histories that can suggest pericardial disease (eg, tuberculosis, renal failure, malignancy, radiation therapy, cardiovascular surgery).[2]

PreviousNextPhysical Examination

Physical findings may exist on a continuum, including findings common with cardiac tamponade.[19] Findings may include hypotension, jugular venous distension, and diminished heart sounds (classic Beck triad). (The classic description of percussible cardiac dullness at the apex may be unreliable.)

Other common findings can include the following:

Pulsus paradoxus (paradoxical pulse)Jugular venous pulse with a prominent x descent and absent y descentTachycardiaTachypneaHepatomegalyAscitesPeripheral edemaPleural effusion (in the absence of left-sided congestive signs)Renal dysfunctionLiver dysfunction and/or auscultation of a pericardial friction rub

Careful attention to all physical findings is required to find clues to theunderlying etiology of the pericardial disease.

PreviousNextDifferential Diagnosis

Because effusive-constrictive pericarditis is rare, the differential diagnosis is guided by few published series and case reports. Differentials to consider include the following:

Postradiation syndromesNeoplasias (metastatic)Hematologic neoplasiasImmunocompromised states with infectionConnective tissue diseaseUremiaBreast CancerCardiac tamponadeRestrictive cardiomyopathyHypothyroidismMyocardial infarctionPenetrating chest traumaPericardial effusionAcute pericarditisConstrictive pericarditisUremic pericarditisTuberculosisPreviousNextLab Studies

Laboratory studies for effusive-constrictive pericarditis include tests of serum complete blood count (CBC) with differential and serum chemistries, with additions depending on the suspected etiology.

The most important laboratory studies are those performed on pericardial fluid (always under the assumption that pericardiocentesis is clinically indicated). The following tests should always be sent on an initial pericardiocentesis[20] :

Hematocrit and cell count with differentialCulture - Including tuberculosisGlucoseTotal proteinEnzymes - Lactate dehydrogenase, adenosine deaminaseGram stainingCytology

The need for other, more specific laboratory tests, including the following, is determined by the priorities of the differential diagnosis:

Suspected tuberculous pericarditis - Purified protein derivative (PPD) of tuberculin, appropriate staining of pericardial fluid Suspected infectious pericarditis - Serum aerobic and anaerobic blood cultures, viral titers, or polymerase chain reaction (PCR) assay of pericardial fluid Suspected malignancy - Pericardial fluid for tumor markers or carbohydrate antigens (CAs; eg, CA-125)Suspected human immunodeficiency virus (HIV) pericarditis - Serum HIV testingSuspected hypothyroid-related pericarditis - Serum thyroid function testingSuspected connective tissue disease - Serum connective tissue serologiesPreviousNextChest Radiography

The chest radiograph may consistently show an enlarged cardiac silhouette when the pericardial effusion is greater than 250mL. The cardiac silhouette may be flask shaped and the lung fields may show no evidence of congestion, consistent with the absence of a congestive cardiomyopathy.[21]

These findings must be interpreted with caution, as they may also be observed in severe aortic insufficiency, congestive heart failure with severe tricuspid insufficiency, severe volume overload, and mitral regurgitation. The distinguishing characteristic is pulmonary vascular congestion, which may be present with any of these conditions and is usually absent in pericardial disease.

A small effusion may have a normal cardiac silhouette, but this does not eliminate the diagnosis of effusive-constrictive pericarditis.

PreviousNextEchocardiography

Echocardiography is the most efficient way to detect an effusion because it has excellent sensitivity and specificity.[17, 22] Pericardial fluid is easily observed as an echolucent region (echo-free space) between the visceral pericardium (epicardium) and the parietal pericardium.

The size of the effusion may be estimated, even if the effusion is localized. For example, small effusions usually must be observed in 2 views, particularly behind the left ventricle. Moderate effusions are visualized circumferentially, and large effusions exceed 1cm in thickness on all views.

Doppler investigation may demonstrate increased respiratory variation of mitral and tricuspid inflow, consistent with constrictive pericarditis. Other echocardiographic findings consistent with constrictive pericarditis include abnormal septal and posterior wall motion, noted in the M-mode by using a parasternal short-axis view; a normal velocity of propagation (V p) in color M-mode; and a normal or supranormal early relaxation (Ea) on tissue Doppler imaging.

Echocardiography can also be used to distinguish a pericardial effusion from a pleural effusion, with pericardial effusions being anterior to the descending aorta.

In addition, evidence for cardiac tamponade may be inferred from an echocardiogram. For example, early diastolic collapse of the right ventricular free wall and/or late diastolic collapse of the right atrium may be observed.

PreviousNextCT Scanning, PET Scanning, and MRI

The diagnosis of effusive-constrictive pericarditis cannot be made primarily on the basis of computed tomography (CT) or magnetic resonance imaging (MRI) scan findings. However, CT scanning and MRI may provide excellent images of the pericardium and associated mediastinal structures.

CT scanning and MRI can be used to effectively image and confirm a thickened pericardium or detect a pericardial effusion if visualization with echocardiography is suboptimal. However, some patients with effusive-constrictive pericarditis have normal pericardial thickness; in such cases, the disease must be diagnosed hemodynamically.

The use of18 F-2-deoxyglucose (FDG) positron emission tomography (PET) scanning has been reported for the assessment of pericardial inflammation. The clinical use of PET imaging in effusive-constrictive pericarditis remains untested.[23]

PreviousNextElectrocardiography

An electrocardiogram (ECG) may not show any specific findings for effusive-constrictive pericarditis. However, the ECG may show changes in the ST segment, T wave, or PR segment and/or demonstrate low QRS voltage associated with pericarditis and/or effusion. Nonspecific ST- and T-wave abnormalities may be present.

With a large effusion, a cardiac rocking motion may be observed on the ECG as electrical alternans.

PreviousNextPericardiocentesis and Pericardial BiopsyPericardiocentesis

Pericardiocentesis as a diagnostic test may have a low yield, yet as a therapeutic procedure its diagnostic benefit is much improved. The risks and benefits of any invasive procedure must be considered before the start of testing.

Pericardial biopsy

Clinical circumstances determine when a biopsy is performed since procedural risk is increased. Factors include how symptomatic the patient is and how likely a finding would change clinical management.

Pericardioscopy is a developing technique that allows direct viewing of the epicardium with the possibility for biopsy. This is currently an experimental technique.[24]

Histologic findings

Pericardial biopsy samples may be examined for malignancy and inflammation by traditional and immunohistologic means. In advanced laboratories, PCR assay or in situ hybridization may be used to analyze for microbial deoxyribonucleic acid (DNA) or ribonucleic acid (RNA). Combined examination of pericardial fluid and biopsy results provides the greatest yield.

PreviousNextHemodynamic Assessment

The diagnosis of effusive-constrictive pericarditis may be suspected clinically, but it is definitively established by recording right heart and intrapericardial pressures before and after pericardiocentesis.[1]

Before pericardial fluid is removed, cardiac tamponade (or near tamponade) hemodynamic physiology must be present to make the diagnosis of effusive-constrictive pericarditis. Hemodynamic pressure recordings will indicate that intrapericardial pressures, right atrial pressure, and end-diastolic right and left ventricular pressures are elevated and equal (or nearly equal). There is usually an inspiratory decrease in right-heart filling pressures. A prominent x descent and an absent y descent may also be noted.

Pericardiocentesis should decrease intrapericardial pressure to zero but may fail to restore cardiac hemodynamics to normal. This is because the visceral constrictive component of the syndrome causes a persistent elevation and equalization of intracardiac diastolic pressures. This constrictive physiology unveils a biphasic pressure tracing in the right atrium, now with a prominent y descent and dip-and-plateau right ventricular pressure tracings, with absent or minimal respiratory variation.

Put another way, persistent constriction after pericardiocentesis suggests a constrictive visceral pericardium and, therefore, the diagnosis of effusive-constrictive pericarditis.

Diagnostic consideration

Because effusive-constrictive pericarditis is rare, intrapericardial pressures are not routinely measured during pericardiocentesis in clinical practice. This protocol may result in failure to recognize intrapericardial pressure as near zero. The consequences of this oversight include missing the diagnosis of effusive-constrictive pericarditis.

PreviousNextPharmacologic Therapy

Although potentially curative therapy for hemodynamically compromising effusive-constrictive pericarditis requires surgical intervention, medical management directed at the underlying etiology may be effective, as dictated by clinical circumstances. However, no randomized, blinded clinical trials have been completed to guide medical therapy, which is primarily supportive.

Depending on putative etiology, steroids, nonsteroidal anti-inflammatory agents, or antibiotics may be needed.[25]

Intravascular volume status must not be decreased excessively in the presence of tamponade physiology; diuretics must not be applied indiscriminately. On the other hand, after pericardial drainage, diuretics may be useful with constrictive physiology and evidence of volume overload.

PreviousNextDrainage, Thoracotomy, and Pericardial Window

Pericardiocentesis or surgical drainage of the effusion is performed as dictated by the patient's clinical situation. These procedures are undertaken in circumstances of tamponade or hemodynamic compromise or when a purulent effusion is suspected, as well as in cases in which there is a large, persistent effusion or diagnostic uncertainty exists.[26]

The most effective therapy for effusive-constrictive pericarditis is pericardiectomy, with complete removal of the parietal and visceral membranes. The perioperative mortality rate for this procedure can be high. Surgery can be risky and requires considerable thought before it may be recommended. Difficulties include the length of the procedure, infection potential, morbidity secondary to the wide exposure required, other medical problems that are often present in these patients, and the technical expertise required to perform the surgery.

In patients who may have a high mortality risk with thoracotomy yet have a significant chance of effusion recurrence with needle drainage alone, a pericardial-peritoneal window is an effective treatment for recurrent pericardial effusions.[27]

PreviousNextInpatient and Outpatient CareInpatient care

If hemodynamic compromise is possible, inpatient care is required to monitor the patient. Moreover, necessary pericardial procedures usually involve hospitalization.

Transfer is required when necessary diagnostic or therapeutic modalities such as echocardiography, pericardiocentesis, or cardiothoracic surgery are not available at the treating facility.

Outpatient care

The priorities of outpatient care reflect the treatments required for specific etiologies and include monitoring patients for signs of worsening constrictive physiology or for the development of cardiac tamponade.

In general, patients are given maintenance therapy with a diuretic to maintain euvolemia. Other medications depend on the specific etiology being treated.

PreviousNextConsultations

A cardiologist can assist with echocardiographic interpretation, pericardiocentesis, and invasive hemodynamics. A cardiothoracic surgeon may help when a pericardial window or pericardiectomy is being considered.

In complicated cases, such as those involving tuberculous pericarditis or purulent uremic pericarditis, multidisciplinary involvement may be required. Specialists in infectious disease, nephrology, cardiology, and/or cardiothoracic surgery may be consulted.

PreviousNextDiet and Activity

No specific dietary changes are recommended. However, patients with effusive-constrictive pericarditis often have chronic underlying diseases for which adequate nutrition is especially important. Moreover, euvolemia is a goal, and salt restriction may be indicated.

The patient’s activities are generally limited by the underlying disease or the decreased cardiac output that may occur with effusive-constriction, but no specific activity prohibitions exist.

Previous, Constrictive-Effusive Pericarditis

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Constrictive Pericarditis

Background

Constrictive pericarditis symptoms overlap those of diseases as diverse as myocardial infarction (MI), aortic dissection, pneumonia, influenza, and connective tissue disorders. This overlap can confuse the most skilled diagnostician. An increased suspicion of constriction helps move constrictive pericarditis to the top of a lengthy differential diagnosis list and facilitates correct diagnosis and timely therapy.

Constrictive pericarditis occurs when a thickened fibrotic pericardium, of whatever cause, impedes normal diastolic filling. This usually involves the parietal pericardium, although it can involve the visceral pericardium (see Constrictive-Effusive Pericarditis). Acute and subacute forms of pericarditis (which may or may not be symptomatic) may deposit fibrin, which, in turn, can evoke a pericardial effusion. This often leads to pericardial inflammation, chronic fibrotic scarring, calcification, and restricted cardiac filling.[1]

The classic diagnostic conundrum associated with constrictive pericarditis is the difficulty distinguishing this condition from restrictive cardiomyopathy (see Restrictive Cardiomyopathy) and other syndromes associated with elevated right-sided pressure that all share similar symptoms, physical findings, and hemodynamics.

Although obtaining a careful history and performing a physical examination remain the cornerstones of evaluation, technologic advances have facilitated diagnosis, particularly with the appropriate use of Doppler echocardiography, high-resolution computed tomography (CT), magnetic resonance imaging (MRI), and invasive hemodynamic measurement.

Pericardiectomy is the predominant definitive treatment. Hemodynamic and symptomatic improvements are rapid. Medical management, such as careful observation or symptomatic treatment, has been suggested in less severe cases; however, this option is controversial. The underlying disease usually determines the prognosis. Poorer prognoses are associated with malignancy and New York Heart Association (NYHA) class III or IV heart failure symptoms.

NextPathophysiology

The normal pericardium is composed of 2 layers: the tough fibrous parietal pericardium and the smooth visceral pericardium. Usually, approximately 50 mL of fluid (plasma ultrafiltrate) is present in the intrapericardial space to minimize friction during cardiac motion.[2]

Acute and subacute forms of pericarditis (which may or may not be symptomatic) may deposit fibrin, which, in turn, can evoke a pericardial effusion. This often leads to pericardial organization, chronic fibrotic scarring, and calcification, most often involving the parietal pericardium (see Constrictive-Effusive Pericarditis for visceral pericardial disease).[3]

In constrictive pericarditis, the easily distensible, thin parietal and visceral pericardial linings become inflamed, thickened, and fused. Because of these changes, the potential space between the linings is obliterated, and the ventricle loses distensibility. Venous return to the heart becomes limited, and ventricular filling is reduced, with associated inability to maintain adequate preload. Filling pressures of the heart tend to become equal in both the ventricles and the atria.

Since the myocardium is unaffected, early ventricular filling during the first third of diastole is unimpeded. After early diastole, the stiff pericardium affects flow and hemodynamics. Accordingly, the ventricular pressure initially decreases rapidly (producing a steep y descent on right atrial pressure waveform tracings) and then increases abruptly to a level that is sustained until systole (the “dip-and-plateau waveform” or “square root sign” seen on right or left ventricular pressure waveform tracings).[4]

The preservation of myocardial function in early diastole aids in distinguishing constrictive pericarditis from restrictive cardiomyopathy. Systolic function is rarely affected until late in the course of the disease, presumably secondary to infiltrative processes that affect the myocardium, atrophy, or scarring or fibrosis of the myocardium from the overlying adjacent pericardial disease.

Experimental models indicated that a change in volume-elasticity curves (see the image below) was the fundamental pathophysiologic change associated with the disease. During development of the constriction, right and left ventricular diastolic pressure increased, and stroke volume decreased. A small increase in volume resulted in a considerable increase in end-diastolic pressure.

Left ventricular volume curve in constrictive pericarditis.

Symptoms consistent with congestive heart failure (CHF), especially right-sided heart failure, develop as a result of the inability of the heart to increase stroke volume.[5] Over time, cardiac output gradually becomes inadequate (see the Cardiac Output calculator), at first with exercise and then at rest.

The clinical symptoms and classic hemodynamic findings of constrictive pericarditis can be explained by the early rapid diastolic filling and elevation, with eventual equalization of the diastolic pressures in all of the cardiac chambers. This restricts late diastolic filling, leading to venous engorgement and decreased cardiac output, all secondary to a confining pericardium.

PreviousNextEtiology

Chronic constrictive pericarditis is a disease that has multiple possible causes and is associated with variable clinical findings, depending on its severity. It develops insidiously, and in many cases, no etiology is ever determined. In some patients (approximately 10%), an antecedent acute pericarditis is present. Other cases of constriction are postulated to have been preceded by a subclinical, or occult, form of pericarditis.

All forms of pericarditis may eventually lead to pericardial constriction. They may be broadly classified into common, less common, and rare forms. The top 3 causes of constrictive pericarditis are idiopathic (presumably viral), cardiothoracic surgery, and radiation therapy, which, according to one study, are responsible for 46%, 37%, and 9% of cases of constrictive pericarditis, respectively (in patients who underwent surgical therapy).[6]

Common forms

Idiopathic

In many cases, particularly in developed countries, no antecedent diagnosis can be found. These cases are termed idiopathic. Reports by many authors indicate that a high percentage of idiopathic cases of constrictive pericarditis may be related to previously recognized or unrecognized viral pericarditis (see below).

Infection (bacterial and viral)

Tuberculosis is the leading cause of constrictive pericarditis in developing nations but represents only a minority of causes in the United States and other developed countries.

Bacterial infections that lead to purulent pericarditis are also declining in frequency. In the past, purulent pericarditis associated with pneumococcal pneumonia was the most common presentation of a bacterial source. However, the widespread use of antibiotics has drastically changed the frequency and spectrum of purulent pericarditis such that the most common presentation now occurs following cardiac surgery.

An increasing number of gram-positive organisms, including multiple resistant strains of staphylococci, may be isolated. Group A and B streptococci and gram-negative rods (eg, Pseudomonas species, Escherichia coli, and Klebsiella species) have also been documented.

While the absolute number of tuberculous and bacterial pericarditis cases are decreasing, it must be recognized that these processes remain closely associated with constrictive pericarditis. A recent prospective analysis determined that the incidence of constrictive pericarditis was 0.76 cases per 1,000 person-years after acute idiopathic/viral pericarditis but 31.7 cases for 1,000 person-years for acute tuberculous pericarditis and 52.7 cases per 1,000 person-years for purulent pericarditis.[7]

Viral pathogens that can cause constrictive pericarditis include coxsackievirus, hepatitis, adenovirus, and echovirus.[8]

Radiation

The long-term effects of thoracic and mediastinal radiation therapy (as used in the treatment of hematologic, breast, and other malignancies) are increasingly being realized. The common features of radiation-induced cardiac complications stem from microcirculation injury with endothelial damage, capillary rupture, and platelet adhesion. This sets up an inflammatory response, which may either resolve or organize to form adhesions between the visceral pericardium and the parietal pericardium. This cascade potentially leads to constriction.

Generally, radiation-induced constrictive pericarditis presents 5-10 years after radiation therapy and is more likely to present with an associated pericardial effusion. In a study by Bertog, the median time between radiation and pericardiectomy was 11 years, with a broad range of 2-30 years. These findings were consistent with those of other previous studies.[6]

Cardiac surgery

Any operative or invasive procedure in which the pericardium is opened, manipulated, or damaged may invoke an inflammatory response, leading to constrictive pericarditis (postpericardiotomy syndrome). The most common example is constrictive pericarditis in the setting of previous coronary artery bypass grafting (CABG) when only part of the pericardium is resected.

Less common forms

Infection (fungal)

Fungal infections are an uncommon source of constrictive pericarditis in patients who are immunocompetent. Nocardia species can be causative organisms, especially in endemic areas such as the Ohio Valley. Aspergillus, Candida, and Coccidioides species are important pathogens in patients infected with HIV and in other immunocompromised hosts.

Neoplasms

Malignant involvement may also manifest as pericardial effusion (with or without tamponade) or as an encased heart with thickening of both visceral and parietal layers, resulting in constrictive physiology. Although many types of neoplasms have been reported, breast and lung carcinomas and lymphomas are the metastatic malignancies most commonly associated with constrictive pericarditis. Other malignancies that involve the pericardium with relative frequency include melanoma and mesothelioma.

Uremia

Uremia with long-term hemodialysis can lead to constrictive pericarditis and is usually associated with a pericardial effusion.

Connective tissue disorders

Autoimmune disorders that involve the pericardium are not unusual, typically manifesting as a small pericardial effusion or as an episode of acute pericarditis. Chronic pericardial involvement is less common but can occur in patients with rheumatoid arthritis, usually associated with the presence of subcutaneous nodules. Systemic lupus erythematosus (SLE) and scleroderma also may lead to constrictive pericarditis; in the latter case, the prognosis is poor.

Drugs

Procainamide and hydralazine have been reported to cause constrictive pericarditis through a drug-induced lupuslike syndrome. Methysergide therapy also has been implicated as a cause of constrictive pericarditis.

Trauma

Blunt and penetrating injuries to the chest wall have been reported to cause constrictive pericarditis, presumably through an inflammatory mechanism. Trauma-induced constrictive pericarditis is generally uncommon.

Myocardial infarction

Post-MI constrictive pericarditis has been reported. The patient typically has a history of Dressler syndrome or hemopericardium after thrombolytic therapy.

Rare forms

Constrictive pericarditis after implantation of an epicardial pacemaker or automated implantable cardiac defibrillator is a rare but reported phenomenon.

Mulibrey nanism is an autosomal recessive disorder characterized by multiple abnormalities, including dwarfism, constrictive pericarditis, abnormal fundi, and fibrous dysplasia of the long bones.

In rare instances, constrictive pericarditis may occur after sclerotherapy for esophageal varices.

Chylopericardium is a rare cause of constrictive pericarditis.

PreviousNextEpidemiology

As with many diseases that once were predominantly infectious in origin, the clinical spectrum of constrictive pericarditis has changed. Approximately 9% of patients with acute pericarditis from any cause go on to develop constrictive physiology.[8] The true frequency is dependent on the incidence of the specific causes of pericarditis, but given that acute pericarditis is clinically diagnosed in only 1 in 1000 hospital admissions, the frequency of a diagnosis of constrictive pericarditis must be less than 1 in 10,000 admissions.

In the developing world, infectious etiologies remain more prominent (tuberculosis has the highest total incidence).

Age-, sex-, and race-related demographics

Although pediatric data are lacking for epidemiologic analysis, it is clear that the condition is rare in adults and even more rare in children. In all age groups, prevalence is increased among patients who are hospitalized and among patients who have undergone cardiac surgery. Cases have been reported in persons aged 8-70 years. Predilection is likely reflective of the underlying disease. Historical studies suggest a median age of 45 years, whereas more recent studies suggest a median age of 61 years (possibly reflecting ongoing demographic changes).

There appears to be a male predominance, with some studies report a male-to-female ratio of 3:1. No racial predilection exists for this disorder.

PreviousNextPrognosis

Because the disease is rare, prognostic data are relatively scant. Constrictive pericarditis is a potentially curable disease if diagnosed early, but it is potentially fatal if overlooked. One study showed postpericardiectomy survival rates of 71% and 52% at 5 and 10 years, respectively. The long-term prognosis with medical therapy alone is poor. Life expectancy is reduced in untreated children and in patients with relatively acute onset of symptoms.

Long-term survival after pericardiectomy depends on the underlying cause. Of common causes, idiopathic constrictive pericarditis has the best prognosis (88% survival at 7 years), followed by constriction due to cardiac surgery (66% at 7 years). The worst postpericardiectomy prognosis occurs in postradiation constrictive pericarditis (27% survival at 7 years), probably as a reflection of confounding comorbidities. Occasionally, the etiology of the constriction may cause coincident myocardial dysfunction.

With surgical treatment, the long-term outcomes of patients with constrictive pericarditis have been shown to be independently less favorable with advanced age, poor renal function, abnormal left ventricular systolic function, high pulmonary artery systolic pressure, lower serum sodium level, worsening NYHA classification, and, as noted above, radiation therapy as the cause of the constrictive pericarditis.[6, 9] The degree of pericardial calcification has shown no effect on survival.

Failure of conventional medical therapy for CHF often follows an extensive diagnostic workup, leading to the final diagnosis of constrictive pericarditis. Decline in function is a result of decreased cardiac output (see the Cardiac Output calculator) with symptoms of CHF, along with morbidity stemming from chronic systemic venous congestion.

Multisystem failure can develop into an end-stage of illness when global tissue hypoxia leads to worsening metabolic acidemia.

PreviousProceed to Clinical Presentation , Constrictive Pericarditis

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