Basics
Description
Inflammation of the pericardium, usually resulting in the accumulation of fluid in the pericardial space between the visceral (serosal tissue intimately related to the myocardium) and parietal (fibrosal layer composed of elastic fibers and collagen) pericardium. Pericarditis may be serous, fibrinous, purulent, hemorrhagic, or chylous.
Epidemiology
- Infectious pericarditis is more frequently seen in children younger than 13 years of age, with predominance in children younger than 2 years of age.
- Postpericardiotomy syndrome occurs in ¢ ¼5 " 10% of children following uncomplicated cardiac surgery, particularly when the atrium has been entered.
Pathophysiology
- Fine deposits of fibrin develop next to the great vessels, leading to altered function of the membranes of the pericardium, including changes in oncotic and hydrostatic pressure with subsequent accumulation of fluid in the pericardial space.
- Effusion is defined as excessive pericardial contents secondary to inflammation, hemorrhage, exudates, air, or pus.
- In postpericardiotomy syndrome, there appears to be a nonspecific hypersensitivity reaction to the direct surgical entrance into the pericardial space.
Etiology
- Infectious
- Viral: coxsackievirus, echovirus, mumps, varicella, Epstein-Barr, adenovirus, influenza, human immunodeficiency virus
- Bacterial: Streptococcus, pneumococcus, Staphylococcus, meningococcus, Mycoplasma, tularemia, Haemophilus influenzae type B, Pseudomonas aeruginosa, Listeria monocytogenes, Pasteurella multocida, Escherichia coli
- Tuberculosis, atypical mycobacterium
- Fungal: candidiasis, histoplasmosis, actinomycosis
- Parasitic: toxoplasmosis, echinococcus, Entamoeba histolytica, Rickettsia
- Rheumatologic/inflammatory
- Acute rheumatic fever
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Systemic sclerosis
- Sarcoidosis
- Dermatomyositis
- Kawasaki disease
- Familial Mediterranean fever
- Inflammatory bowel disease
- Metabolic/endocrine
- Hypothyroidism
- Uremia (chemical irritation)
- Gout
- Scurvy
- Neoplastic disease
- Lymphoma
- Lymphosarcoma
- Leukemia
- Sarcoma
- Metastatic disease to the pericardium
- Radiation therapy induced
- Postoperative
- Postpericardiotomy syndrome (after cardiac surgery)
- Chylopericardium
- Other
- Trauma
- Drug-induced (hydralazine, isoniazid, procainamide)
- Aortic dissection
- Postmyocardial infarction
- Idiopathic
Diagnosis
History
- Dependent on etiology
- Signs and symptoms
- Most common signs and symptoms:
- Precordial chest pain
- Fever
- Cough
- Shoulder pain aggravated by changes in position
- Rapid accumulation of fluid may lead to the following:
- Respiratory distress/dyspnea
- Signs of hypotension
- Change in mental status/loss of consciousness
- Pain: often relieved if the child sits leaning forward
- Slow, chronic accumulation may be associated with no symptoms at all until tamponade develops.
- Other symptoms depend on the etiology of the pericarditis.
- Recent upper respiratory infection or gastroenteritis (viral pericarditis)
- Sepsis or other source of bacterial infection
- Symptoms of rheumatic disease
- Known thoracic neoplasm
Physical Exam
- Pericardial friction rub is the pathognomonic finding (typically heard if only a small amount of fluid is in the pericardial space).
- Quiet precordium, tachycardia, hypotension, and muffled heart sounds may be heard when there is a large amount of fluid and/or tamponade.
- Evidence of right-sided heart failure:
- Peripheral edema, jugular venous distention, and hepatomegaly
- Pulmonary edema: rare because the heart is underfilled, and left atrial pressure, although elevated, does not exceed right atrial pressure
- Pulsus paradoxus: an exaggerated decrease in systolic BP with inspiration
- Kussmaul sign: paradoxical rise in jugular venous pressure during inspiration, often considered diagnostic of tamponade
Diagnostic Tests & Interpretation
Lab
Electrocardiogram
- Nonspecific but generally demonstrates low-voltage QRS complexes secondary to dampening of the signal transmitted through the pericardial fluid
- One can also see diffuse ST segment elevation with or without T-wave inversion.
- These findings may be secondary to inflammation of the myocardium.
- Electrical alternans can be seen with large effusions.
Imaging
- Chest x-ray
- Often shows enlargement of the cardiac silhouette ( "water bottle sign " ), usually in association with normal pulmonary vascular markings. However, heart size may appear normal in acute pericarditis.
- Calcification may be seen in constrictive pericarditis.
- Computed tomography (CT)
- CT can also demonstrate calcification of the pericardium with excellent sensitivity
- Echocardiogram
- Most sensitive and specific test for pericardial thickening and fluid in the pericardial space
- In the presence of a large effusion, the heart may appear to swing within the pericardial cavity.
- In tamponade, diastolic collapse of the right atrium may be seen. Collapse of the left atrium and right ventricle occur in severe cases.
- Tamponade can be diagnosed using Doppler inflow patterns of the tricuspid and mitral valves. In tamponade, mitral inflow E-wave velocity decreases by more than 30% during inspiration, whereas the tricuspid inflow E-wave velocity increases by more than 50% during inspiration.
Diagnostic Procedures/Other
- Pericardiocentesis is performed when the etiology of the effusion is in question or tamponade has developed.
- Fluid obtained should be sent to the lab for cell count, cytology, and culture (including bacteria, viruses, Mycobacterium tuberculosis, and fungi).
- Complications include myocardial puncture, coronary artery/vein laceration, hemopericardium, and pneumothorax.
- Echocardiogram or fluoroscopic guidance is useful for this procedure but is not required if there is impending cardiovascular collapse.
- Pericardial window
- In cases of chronic pericardial effusion, removal of part or all of the pericardium may be performed (pericardial window).
Differential Diagnosis
- Acute myocarditis
- History, physical examination, and laboratory findings of acute pericarditis can be quite similar to those found in acute myocarditis.
- In addition, myocarditis can be associated with pericardial disease and vice versa.
- Echocardiogram is an excellent tool to help differentiate between these two entities.
- Restrictive cardiomyopathy
- Other causes of chest pain
- Myocardial infarction
Treatment
General Measures
- Treatment should be directed toward the etiology of the disease. However, no matter the cause, pericardiocentesis is required if there is an effusion that causes hemodynamic compromise. It may be life-saving in patients with bacterial pericarditis.
- Viral pericarditis usually resolves spontaneously in 3 " 4 weeks with bed rest and analgesics (NSAIDs).
- Bacterial pericarditis is potentially life threatening and requires immediate decompression of the pericardial space (often with open drainage and pericardial window creation), IV antibiotic therapy for at least 4 weeks, and supportive therapy (i.e., volume expansion, inotropes).
- S. aureus is the most common organism responsible for bacterial pericarditis.
- Rheumatologic causes of pericardial inflammation usually respond to corticosteroids and/or salicylates and rarely require pericardiocentesis.
- Uremic pericarditis usually responds to dialysis, but pericardiotomy (surgical removal of the pericardium) may be necessary in chronic situations.
- Neoplastic pericarditis is addressed by treating the primary disease and performing pericardiocentesis if indicated for diagnostic and/or hemodynamic reasons.
- Hemorrhagic pericarditis with effusion accumulation secondary to trauma should be drained because of the risk of subsequent development of constrictive pericarditis.
- Constrictive pericarditis is treated with complete stripping of the pericardium (pericardiectomy). Often, immediate clinical improvement is not seen because there has been myocardial damage. However, eventual full recovery is the norm.
- Postpericardiotomy syndrome occurs 1 " 4 weeks after cardiac surgery.
- Treat with anti-inflammatory drugs, bed rest, and occasionally steroids.
- Pericardiocentesis is indicated if tamponade develops.
Ongoing Care
- Most forms of pericarditis resolve on their own, or with anti-inflammatory medication, over the course of several weeks.
- Follow-up is necessary to ensure that effusions have resolved and to assess for recurrence (up to 15% relapse).
- Patients with bacterial pericarditis require long-term antibiotic therapy and close follow-up to assess for the development of constrictive pericarditis.
- Signs to watch for include the following:
- Postpericardiotomy syndrome: All cardiac surgical patients need an evaluation 2 " 4 weeks after surgery to assess for postpericardiotomy syndrome, with treatment and follow-up as necessary.
- Signs of low cardiac output and right-sided heart failure indicate impending cardiac tamponade.
- Constrictive pericarditis may present with a rapidly decreasing cardiac silhouette, calcifications on chest roentgenogram, and signs or symptoms of right-sided heart failure.
Prognosis
- Most children recover fully from pericarditis, even if it is bacterial in etiology.
- However, there is significant morbidity and mortality associated, especially in young infants, when the diagnosis is delayed and/or when S. aureus is the etiologic agent.
- Pericarditis can also recur in as many as 15% of patients.
- Prognosis varies with the cause of pericarditis but generally is related directly to the primary disease.
Complications
- Cardiac tamponade
- Intrapericardial pressure increases at a rapid rate secondary to decreased compliance of the pericardial membranes, resulting in restriction of ventricular filling and eventual decrease in stroke volume and cardiac output.
- The compliance of the pericardium is influenced by the disease process itself (i.e., the pericardium is thickened and stiff in bacterial and tuberculous pericarditis).
- During cardiac tamponade, ventricular end-diastolic, atrial, and venous pressures are all equal.
- In acute pericarditis, tamponade may occur with small amounts of fluid because of a rapid increase in the intrapericardial pressure. In contrast, large amounts of fluid may be tolerated if the accumulation is a chronic, slow process.
- Constrictive pericarditis
- Thick, fibrotic, and often calcified pericardium is seen, usually a late result of purulent or tuberculosis pericarditis; it can occur months to years after the initial infection. It can also be seen in oncology patients with direct invasion of tumor into the pericardium or after significant radiation to the chest.
- Poor compliance of the pericardium leads to diminished diastolic filling of the ventricle. Patients may complain of exercise intolerance and fatigue. Additionally, they may have signs of right heart failure.
- This entity may be difficult to distinguish from restrictive cardiomyopathy.
Additional Reading
- Demmler GJ. Infectious pericarditis in children. Pediatr Infect Dis J. 2006;25(2):165 " 166. [View Abstract]
- Tissot C, Phelps C, Younoszai AK. Restrictive cardiomyopathy and pericardial disease in Echocardiography. In: Lai WW, Mertens L, Cohen MS, et al, eds. Pediatric and Congenital Heart Disease from Adult. Oxford, United Kingdom: Wiley-Blackwell; 2009:597 " 618.
- Towbin JA. Myocarditis and pericarditis in adolescents. Adolesc Med. 2001;12(1):47 " 67. [View Abstract]
Codes
ICD09
- 423.9 Unspecified disease of pericardium
- 420.90 Acute pericarditis, unspecified
- 423.8 Other specified diseases of pericardium
- 420.91 Acute idiopathic pericarditis
- 423.2 Constrictive pericarditis
- 423.1 Adhesive pericarditis
ICD10
- I31.9 Disease of pericardium, unspecified
- I30.9 Acute pericarditis, unspecified
- I30.1 Infective pericarditis
- I31.1 Chronic constrictive pericarditis
- I31.0 Chronic adhesive pericarditis
SNOMED
- 3238004 Pericarditis (disorder)
- 15555002 Acute pericarditis (disorder)
- 41739008 infectious pericarditis (disorder)
- 23627006 Chronic pericarditis (disorder)
- 37715009 Adhesive pericarditis (disorder)
- 2554006 Acute purulent pericarditis
- 70189005 Viral pericarditis (disorder)
- 85598007 Constrictive pericarditis (disorder)
FAQ
- Q: How does cardiac tamponade present?
- A: Patients with impending tamponade appear quite ill, with tachycardia, chest pain, and signs of right heart failure including jugular venous distention, hepatomegaly, ascites, and peripheral edema. They may also have signs of poor systemic perfusion secondary to low cardiac output. Chest x-ray may or may not show an enlarged cardiac silhouette, depending on how acutely the process occurs. It takes much less fluid to cause tamponade in an acute process than in a chronic process. Echocardiography is the standard diagnostic tool, and pericardiocentesis is the treatment.
- Q: What is pulsus paradoxus and how does one measure it?
- A: Pulsus paradoxus is an exaggerated response of the systolic BP to the normal respiratory cycle. Normally with inspiration, the systolic BP drops ¢ ¼5 mm Hg secondary to the increased capacitance of the pulmonary veins from the increased systemic venous return. In tamponade, this response becomes more profound (>10 mm Hg), most likely secondary to diminished filling of the left heart. Pulsus paradoxus can also be seen in patients with severe respiratory distress associated with asthma and emphysema.
- To assess for pulsus paradoxus, measure the systolic BP first in expiration, then allow it to fall to the place where it is heard equally well in inspiration and expiration. A difference of >10 mm Hg is considered abnormal.