Pericarditis

Pericarditis is an inflammation of the pericardium, often with fluid accumulation.  It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma.  Acute, subacute, and chronic forms exist.  Acute pericarditis is usually idiopathic and manifests as fever, pleuritic chest pain, and an audible pericardial rub by auscultation. Diffuse upwardly concave ST-segment elevations in the initial ECG and pericardial effusion on echocardiography confirm the diagnosis. Acute pericarditis is usually self-limiting (2–6 weeks); therefore, management is conservative. If cardiac tamponade or constrictive pericarditis develops, cardiac output can be severely reduced. Treatment depends on the cause, but general measures include analgesics, anti-inflammatory drugs, colchicine, and rarely surgery.

 

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Definition and Classification

Definition

Pericarditis is an inflammation of the pericardium, the double-layered sac surrounding the heart.

Clinical classification is based on duration.

  • Acute (< 6 weeks) 
    • Fibrinous
    • Effusive (serous or serosanguinous)
  • Subacute (6 weeks to 6 months)
    • Effusive-constrictive
    • Constrictive
  • Chronic (> 6 months)
    • Constrictive
    • Adhesive (non-constrictive)

Epidemiology and Etiology

Epidemiology

  • Reported in 0.1%–0.2% of hospitalized patients
  • Found in 5% of patients admitted to the emergency department (ED) for nonischemic chest pain

Etiology

Idiopathic (most common)After exclusion of other causes
Viral infection
  • Coxsackievirus B
  • Influenza
  • HIV
  • Echovirus
Bacterial infection
  • Tuberculosis (most common cause worldwide)
  • Streptococcus species (rheumatic fever)
  • Lyme disease
  • Pseudomonas
  • Staphylococcus species
  • Mycoplasma
Fungal infection (very rare)
  • Histoplasma
  • Blastomyces
  • Coccidioides
  • Aspergillus
Autoimmune disease
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Sarcoidosis
  • Vasculitides (Behcet’s, Takayasu’s arteritis)
Metabolic
  • Uremia
  • Hypothyroidism
Cardiovascular
  • Takotsubo cardiomyopathy
  • Dressler syndrome
    • Myocardial infarction
    • Cardiac injury (e.g., following operation)
  • Aortic dissection
  • Chronic heart failure
Cancer
  • Lung cancer
  • Breast cancer
  • Leukemia
  • Lymphoma
  • Radiation therapy
Drugs
  • Procainamide
  • Hydralazine
  • Penicillin
  • Isoniazid
  • Chemotherapy

Pathophysiology

  • Inflammatory cascade stimulates the release of fluid in the pericardial space, causing effusion.
  • Rapid accumulation of large amounts of fluid in the pericardial space can cause cardiac tamponade, compromising cardiac output and potentially resulting in obstructive shock.
  • Constrictive pericarditis is characterized by a thickened and scarred pericardial sac that lies around the heart and prevents proper diastolic filling.

Clinical Presentation and Diagnosis

Typical clinical presentation

  • Patient presents with central chest pain that worsens during inspiration or when lying flat. Leaning forward while sitting may relieve the pain. 
  • Fever can be present. 
  • In 50% of cases, a pericardial friction rub (scratching sound during systole and diastole) can be heard upon auscultation.
Important clinical and diagnostic features of acute, chronic, and constrictive pericarditis
FeaturesAcuteChronicConstrictive
Pain
  • Severe/sharp
  • Frequently: pleuritic1
  • Sometimes: steady (may get confused with acute myocardial infarction [MI])
  • Radiation: neck, shoulder(s), arm(s), trapezius
  • Less severe
  • May be absent if developing slowly
None
Other symptoms
  • Pain intensifies with lying supine
  • Pain improves with leaning forward
May have dyspnea
  • Weakness
  • Fatigue
  • Weight gain
  • Exertional dyspnea
Physical examination
  • May have neck vein distension
  • Pericardial friction rub2
  • May have neck vein distension
  • X-ray: Large cardiac silhouette
  • Neck vein distension
  • Pericardial knock3
  • Kussmaul’s sign4
  • Paradoxical pulse5
  • Hepatomegaly, ascites, peripheral edema
  • JVP curve: prominent x and y descents
  • X-ray: small/normal/slightly large cardiac silhouette and pericardial calcifications
ECG
  • Diffuse ST-segment elevation with upward concavity and without T-wave inversions6
  • Electrical alternans7 with a large effusion
Electrical alternans7 with a large effusion
  • Low-voltage QRS
  • Atrial fibrillation in ⅓ of cases
Echocardiography8Pleural effusionPleural effusionPericardial thickening
Tamponade9With rapid fluid accumulationUncommonNever
CommentsElevated cardiac biomarkers can occur, further simulating a diagnosis of acute MI.
  1. Pleuritic pain: pain that is exacerbated with inspiration or exhalation and reduced or eliminated by holding respirations
  2. Pericardial friction rub: a rasping, scratching, or grating sound with up to 3 components per cardiac cycle and best heard during expiration with the patient leaning forward
  3. Pericardial knock: an early third heart sound
  4. Kussmaul’s sign: absence of normal decline in jugular venous pressure with inspiration; also seen in tricuspid stenosis, right ventricular infarction, and restrictive cardiomyopathy
  5. Paradoxical pulse: a drop by > 10 mm Hg in systolic blood pressure (SBP) during inspiration (i.e., difference between the first SBP sound heard during exhalation and the first SBP sound heard audible throughout the respiratory cycle > 10 mm Hg)
  6. Subsequent ECG changes include: ST-segment elevation returns to normal after several days (stage 2), followed by T-inversions (stage 3), and complete normalization of ECG after weeks to months (stage 4)
  7. Electrical alternans: alternating QRS amplitudes
  8. Echocardiography for pericarditis: most widely used modality (CT and MRI are more accurate for pericardial disease)
  9. Tamponade: pericardial effusion, usually of rapid onset, exceeding ventricular filling pressures and causing collapse of the heart with a markedly reduced cardiac output

Etiology-specific features

  • Viral or acute idiopathic pericarditis:
    • Chest pain 1–2 weeks after a viral-like illness is suggestive.
    • Must exclude acute MI, postcardiac injury, collagen vascular disease, drugs, pyogenic pericarditis, etc.
    • Most frequent complication is relapsing pericarditis.
  • Postcardiac injury acute pericarditis:
    • 1–4 weeks after a cardiac operation or blunt/penetrating trauma
  • Tuberculous pericarditis:
    • Common cause of chronic pericardial effusion in developing countries
    • In a patient with tuberculosis or systemic illness and large cardiac silhouette: positive fluid culture or pericardial biopsy revealing caseating granuloma confirms the diagnosis.
  • Uremic pericarditis:
    • Seen with severe renal failure or in patients on chronic dialysis

Management

Viral or idiopathic pericarditis

  • Oxygen and analgesia
  • High-dose aspirin/nonsteroidal anti-inflammatory drugs (NSAIDs) (e.g., ibuprofen, indomethacin) plus gastric protection
  • Adjuvant therapy with colchicine enhances response and reduces the recurrence rate.
  • Short-term glucocorticoids can be used if NSAIDs/colchicine are ineffective or contraindicated.

Constrictive pericarditis

  • Complete pericardial resection (pericardiectomy) is the only definitive treatment and is best performed as early as possible
  • Diuretics to reduce symptoms leading to surgery

Specific treatments

  • Initiation or intensification of dialysis in addition to NSAIDs in uremic pericarditis
  • Antibiotics if an underlying infection is found
  • Antituberculous therapy ± pericardiectomy

Differential Diagnoses

  • Stable and unstable angina: paroxysmal chest pain or discomfort caused by myocardial ischemia. Stable angina is characterized by a short duration of the complaints that are often associated with triggers like physical activity or stress. In unstable angina, symptoms are present even at rest. Most individuals with angina have coronary heart disease.
  • MI: myocardial cell death due to ischemia of the myocardial tissue caused by a complete obstruction or drastic constriction of the coronary artery. Typical symptoms include chest pain that may radiate to the left arm, jaw, neck, and upper back, as well as nausea and vomiting. ST-elevation on ECG can be present (STEMI) or absent (NSTEMI). 
  • Aortic stenosis: a narrowing of the aortic valve aperture, characterized by a narrowed left ventricular outflow tract and obstruction of blood flow into the aorta
  • Esophagitis: an inflammation of the esophageal lining, which can be caused by gastroesophageal reflux disease (GERD), infections, drugs, and allergic reactions.
  • Pancreatitis: an inflammation of the pancreas that typically causes epigastric pain that radiates to the back 
  • Pneumonia: acute or chronic inflammation of lung tissue most commonly caused by infection with bacteria, viruses, or fungi
  • Pleuritis: also known as pleurisy, an inflammation of the pleura that lines the lungs. May be caused by a viral infection (most common), pneumonia, lung cancer, autoimmune disease, or pulmonary embolism 
  • Tuberculosis: a disease caused by Mycobacterium tuberculosis, which usually attacks the lungs but can also damage other parts of the body. Presents with fever, weight loss, night sweats, and a productive cough
  • Pneumothorax: a collection of air in the pleural space that causes the lung to collapse due to the loss of negative pressure. Presents with pleuritic chest pain, dyspnea, tachycardia, and reduced breath sounds on the ipsilateral side
  • Herpes zoster: a dermatomal rash with painful blistering, preceded by sharp burning pain, caused by the reactivation of the varicella-zoster virus. It usually affects dermatomes T3 through L3.

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