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DISEASES OF THE PERICARDIUM

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1. ACUTE PERICARDITIS

Definition

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Etiology (Causes)

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Classification

• Acute: Less than 6 weeks
• Subacute: 6 weeks to 6 months
• Chronic: More than 6 months

1. Fibrinous Pericarditis:
   • Fibrinous exudate is deposited on the pericardial surface
   • May heal completely OR form varying degrees of adhesions (fibrous bands between layers)
   • This is the type seen in acute pericarditis, uremia, post-MI
2. Serous Pericarditis:
   • Produces a large effusion of turbid, straw-colored fluid with high protein content
   • Seen in viral, autoimmune, and TB pericarditis
3. Hemorrhagic Pericarditis:
   • Contains blood-stained fluid
   • Usually due to malignant disease (carcinoma of breast, bronchus, lymphoma) or tuberculosis
4. Purulent (Suppurative) Pericarditis:
   • Contains pus (neutrophils + bacteria)
   • Develops as a complication of septicemia, direct spread from an intrathoracic infection (lung abscess, pneumonia), or a penetrating injury

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Clinical Features

A. Pericardial Pain

• Character: Sharp, retrosternal (central) chest pain
• Radiation: To the shoulders, neck, and trapezius muscle ridges (due to phrenic nerve irritation - this is a hallmark feature that favors pericardial pain over cardiac pain)
• Relieved by: Sitting upright and leaning forward
• Aggravated by: Deep breathing, movement, lying down, coughing, exercise, and swallowing
• Pain may also be steady and constrictive in quality

B. Nonspecific Symptoms

• Low-grade fever
• Malaise
• Myocarditis may coexist - can produce dyspnea (Ewart's sign: dullness at lower left sternal area when large pericardial effusion compresses adjacent bronchi and lung)

C. Pericardial Friction Rub (MOST IMPORTANT SIGN)

• A high-pitched superficial scratching or crunching sound
• Produced by movement of the inflamed pericardium
• Pathognomonic (diagnostic) of pericarditis
• Best heard with: Diaphragm of stethoscope, firm pressure, at lower left sternal edge, at end of expiration, with patient sitting upright and leaning forward
• Quality: "To-and-fro," leathery quality; transitory (disappears and reappears)
• Three phases (classic): Corresponding to three movements of the cardiac cycle:
  1. Atrial systole
  2. Ventricular systole
  3. Ventricular diastole (rapid ventricular filling - early diastole)
• May be heard as monophasic (only ventricular systole) or biphasic

D. Pulsus Paradoxus

• Characterized by weakness/disappearance of arterial pulse during inspiration
• Also seen with cardiac tamponade

E. Other Features

• Cardiac tamponade: Characterized by weakness/disappearance of arterial pulse during inspiration and loss of elasticity of the pericardial sac
• Constrictive pericarditis (chronic): Pericardium thickens and impairs cardiac filling; characterized by constrictive physiology, sometimes with calcification
• Effusive-constrictive pericarditis: Results from scarring and shows chronic inflammation

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Investigations

1. Blood Tests

• Leukocytosis - in bacterial pericarditis
• Lymphocytosis - in viral pericarditis
• Raised ESR (erythrocyte sedimentation rate)
• Cardiac enzymes (CPK-MB, Troponin T): Normal unless associated with myocarditis

2. Electrocardiogram (ECG) - VERY IMPORTANT

• Stage 1 (Early): Widespread concave upward (saddle-shaped/smiling face) ST elevation in multiple leads (I, II, aVL, aVF, and V2-V6); PR segment depression (characteristic feature)
• Stage 2: ST elevation resolves; T waves flatten
• Stage 3: T wave inversion
• Stage 4: Normalization

3. Chest X-ray

• May be normal in pericarditis without effusion
• Stenciled borders of heart (if effusion present)
• Rapid increase in cardiac shadow = globular enlargement ("money bag/water-bottle appearance")
• Oreo cookie sign / Epicardial fat pad sign on lateral chest X-ray

4. Echocardiography

• Used to confirm pericardial effusion
• Useful when there is thickening (>4 mm) or inflammation of the pericardium

5. CT and Cardiac MRI

• May be useful in certain cases

6. Pericardiocentesis (Paracentesis)

• Performed when there is pericardial effusion

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Differential Diagnosis (from Acute MI)

• In MI: ST elevation is convex upward (reciprocal changes present); troponin elevated; Q waves develop; ST-to-T wave amplitude ratio >0.24 in V6 suggests pericarditis
• Echocardiography: In MI, regional wall motion abnormalities are present; pericarditis has normal wall motion
• Most reliable feature: ST segment shape + PR depression favors pericarditis

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Treatment

1. Treat the underlying cause (e.g., antitubercular drugs for TB pericarditis, dialysis for uremia)
2. Bed rest and avoid physical activity
3. Analgesics / NSAIDs - cornerstone of treatment:
   • Aspirin 600 mg 4-hourly - drug of choice when recent MI present; also used generally; can be given till pain and effusion disappear (usually 7-10 days)
   • Ibuprofen 300-800 mg 3 times daily - may also be used; monitor ESR/CRP
   • Indomethacin 25 mg 3 times daily - more potent anti-inflammatory; avoid in elderly (decreases coronary blood flow)
   • Colchicine 0.5 mg twice daily - also effective in combination with NSAIDs
4. Corticosteroids (10-30 mg/day for 2-4 weeks): Reserved for patients with immune cause or those who do not respond to NSAIDs; associated with increased recurrence
5. Anakinra or azathioprine - in resistant cases
6. Purulent pericarditis: Antimicrobial therapy + pericardiocentesis + surgical drainage
7. Indications for hospitalization: Fever >38°C, leukocytosis, large pericardial effusion, lack of response to NSAIDs after 1 week of therapy, acute trauma, cardiac tamponade, immunosuppressed state, recurrent pericarditis

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2. PERICARDIAL EFFUSION

Definition

Key Points About Accumulation

• Slowly developing effusions may be asymptomatic (the pericardial sac gradually distends)
• If it develops over a short period and volume is large, it leads to compromise of ventricular filling and embarrassment of circulation - this is called Cardiac Tamponade

Types of Fluid

• Transudate - hydropericardium (e.g., heart failure, hypothyroidism)
• Exudate - pyopericardium (infection, inflammation)
• Hemopericardium - blood (trauma, aortic dissection)

Etiology

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Clinical Signs

Symptoms

• Reflect the underlying pericarditis
• Sometimes a sensation of retrosternal oppression may be present

Signs

• Cardiovascular examination: Normal except if effusion is large - then apex beat/impulse is not palpable (medial to the left border of cardiac dullness)
• Increase in cardiac dullness on percussion
• Heart sounds: Faint, soft, distant, or muffled (as fluid accumulates)
• Pericardial friction rub: May be audible in early stages; becomes quieter as fluid accumulates (separates the pericardial layers)
• Ewart's sign: Rarely, large effusion compresses the base of the left lung; produces an area of dullness to percussion in the left axilla or left base (below the angle of the left scapula) - this is Ewart's sign

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Radiological and Other Investigations

1. Chest X-ray

• Shows increased size of the cardiac silhouette
• Large, globular or pear-shaped heart ("water-bottle appearance")
• Sharp outlines with lucent pericardial fat lines
• Pulmonary veins are not distended (unlike CCF where they are congested)
• Oreo cookie sign / Epicardial fat pad sign: On lateral X-ray, two layers of fat (epicardial fat inside and pericardial fat outside) separated by fluid create this appearance

2. ECG

• Low-voltage QRS complexes (<0.5 mV in limb leads) - in large effusions with sinus tachycardia
• Electrical alternans: Alternation of QRS amplitude or axis between beats - due to to-and-fro motion of the heart within the fluid-filled pericardial sac; may be observed

3. Echocardiography - MOST USEFUL INVESTIGATION

• Most useful investigation for demonstrating the pericardial effusion
• Also useful to monitor the size of the effusion and its effect on cardiac function

4. Cardiac CT or MRI

• Advisable if loculated pericardial effusions are suspected (e.g., post-cardiac surgery)

5. Pericardial Aspiration (Pericardiocentesis)

• Aspiration of pericardial fluid with aseptic technique under echocardiographic guidance
• Needle inserted medial to the cardiac apex or below the xiphoid process, directed upward toward the left shoulder
• Indications:
  • Diagnostic (e.g., tuberculous, malignant, or purulent effusion)
  • Treatment of cardiac tamponade
• Complications: Arrhythmias, damage to myocardium and coronary vessels, air embolism, pneumothorax
• Fluid sent for:
  • Cell counts, protein, glucose, LDH (to differentiate exudate from transudate), Gram's stain, AFB stain
  • Cytology for malignant cells
  • Mycobacterium culture

6. Pericardial Biopsy

• If tuberculosis is suspected and pericardiocentesis is not diagnostic

7. Other Tests

• Blood cultures, autoantibody screen depending on underlying cause

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Treatment

1. Treat the underlying cause if possible
2. Anti-inflammatory drugs (aspirin or indomethacin) - most effusions resolve spontaneously; rapid effusion may produce cardiac tamponade
3. Therapeutic pericardiocentesis: Indicated to relieve pressure; a pigtail catheter drain may be left in temporarily to allow sufficient release of fluid
4. Pericardial fenestration: If effusions accumulate (e.g., malignancy), a window is created in the pericardium to allow slow release of fluid into surrounding tissues; may be done transcutaneously or by conventional surgical approach
5. Intrapericardial instillation of chemotherapeutic agents - may be useful in malignant effusion

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3. CARDIAC TAMPONADE

Definition

Key Facts About Volume

• Minimum amount of fluid needed depends on the speed of accumulation:
  • Rapidly developing: As little as 250 mL can cause tamponade
  • Slowly developing: May need more than 2,000 mL before tamponade occurs

Etiology

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Clinical Features

Symptoms

• Dyspnea, orthopnea
• Substernal chest discomfort radiating to neck and jaw

• Pain in the right upper quadrant (hepatic congestion)
• Pedal edema (in slowly developing - subacute tamponade)

Signs

1. Friedreich's Sign:
   • Markedly raised jugular venous pressure (JVP) with sharp rise and y descent
2. Pulsus Paradoxus (HALLMARK of Cardiac Tamponade):
   • An exaggeration in the normal variation in pulse pressure seen with inspiration
   • Drop in systolic blood pressure of ≥10 mm Hg during inspiration
   • The pulse becomes weak/disappears during inspiration
   • Pulsus paradoxus is the hallmark of cardiac tamponade
3. Kussmaul's Sign:
   • Rise in JVP / increased neck vein distension during inspiration
4. Others:
   • Reduced cardiac output, hypotension, tachycardia, oliguria
5. Beck's Triad (Classic triad of severe tamponade):
   • Hypotension (due to reduced cardiac output)
   • Muffled/distant heart sounds (fluid dampens sounds)
   • Elevated jugular venous pressure (raised JVP)
   • Beck's triad is useful diagnostic clue for severe tamponade

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Investigations

1. Chest X-ray: Discussed earlier (globular heart, water-bottle appearance)
2. Electrocardiogram:
   • Sinus tachycardia
   • Reduction in QRS voltage (low voltage complexes)
   • Nonspecific ST-T changes
   • Electrical alternans (alternation of QRS complex amplitude or axis between beats) - highly characteristic
3. Echocardiography (Most Specific Investigation):
   • Shows a lucent separation between parietal and visceral pericardium (fluid layer) for the entire cardiac cycle
   • Small effusions are first evident over the posterobasal left ventricle
   • Early diastolic collapse of the right ventricle and collapse of the right atrium (which occurs during ventricular diastole) - sensitive and specific signs
   • Right atrial collapse is considered more sensitive
   • RV collapse is more specific for tamponade

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Management

1. Emergency pericardiocentesis is necessary (definitive treatment)
2. If patient is hypotensive: As a temporary measure, expand blood volume by IV saline, blood, plasma, or dextran
3. Avoid positive pressure mechanical ventilation in acute tamponade because it reduces cardiac filling further
4. Treatment of underlying cause

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4. CHRONIC CONSTRICTIVE PERICARDITIS

Definition

• The pericardium usually becomes adherent, resulting in obliteration of the pericardial space
• In some cases, the constriction is formed by the visceral pericardium (epicardium) alone

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Pathophysiology

1. Restricted diastolic filling of the heart by fibrotic pericardium
2. Limitation of venous return to the heart
3. Filling pressures of the heart become equal in both ventricles and atria → reduced ventricular filling → inability to maintain adequate preload
4. Systolic function is rarely affected until late in the disease
5. Preservation of myocardial function in early diastole helps distinguish constrictive pericarditis from restrictive cardiomyopathy

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Etiology (Box 1.54)

• Idiopathic
• Infectious: Tuberculosis (most common in developing countries), Viral (especially Coxsackie B), Bacterial, Fungal, Parasitic
• Postradiotherapy
• Postcardiac surgery
• Post-traumatic
• Neoplastic
• Connective tissue diseases (RA, SLE)
• Toxic/metabolic: Uremia, chylous pericardium, methysergide
• Postmyocardial infarction
• Familial

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Clinical Features

1. Reduced Ventricular Filling (similar to cardiac tamponade)

• Kussmaul's sign, Friedreich's sign, pulsus paradoxus

2. Systemic Venous Congestion (Right-sided heart failure picture)

• Symptoms consistent with congestive cardiac failure (CCF), especially right-sided:
  • Ascites
  • Dependent edema
  • Hepatomegaly
  • Raised JVP

3. Reduced Cardiac Output

• Inability of the heart to increase stroke volume
• Produces: Fatigue, hypotension, reflex tachycardia

4. Rapid Ventricular Filling - Pericardial Knock

• Pericardial knock occurs 0.09-0.12 second after A2 (aortic valve closure)
• Can be heard in early diastole at the lower left sternal border
• Caused by the sudden cessation of ventricular filling when the rigid pericardium is reached

5. Rarely

• Pulmonary venous congestion causing dyspnea, cough, orthopnea

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Investigations

1. ECG:
   • Rarely normal
   • Nonspecific and highly variable
   • Atrial arrhythmias are frequent; atrial fibrillation occurring late in course
   • Low voltage (<50% of cases)
   • LA enlargement (19-37%)
2. Chest X-ray:
   • Pulmonary venous congestion and pleural effusions (late in disease)
   • Calcified pericardium - highly suggestive of constrictive pericarditis (especially when constrictive/restrictive physiology is present)
3. Echocardiography:
   • Pericardial thickening better seen through transesophageal echo
   • Normal RV and LV chamber size
   • LA and RA enlargement
   • Abnormal septal and posterior wall motion
   • Paradoxical septal motion
   • Premature opening of the pulmonic valve
   • Dilated IVC without respiratory variation
4. MRI/CT Scan:
   • Can confirm pericardial thickening
   • Pericardial thickening >4 mm assists in differentiating constrictive pericarditis from restrictive cardiomyopathy
   • Thickening >6 mm - even more specific for constriction
   • NOTE: Normal pericardial thickness does NOT exclude constriction; clinical situation must always be taken into account
5. Cardiac Catheterization:
   • Ventricular pressure initially decreases rapidly ("steep y descent" on RA pressure waveform tracings)
   • Then increases abruptly to a level that is sustained until systole = "dip-and-plateau waveform" or "square root sign" on RV or LV pressure waveform tracings

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Treatment

1. Pericardiectomy (surgical resection of the pericardium) - the only definitive treatment
2. Pharmacologic therapy:
   • Steroids - for subacute constrictive pericarditis (before pericardial fibrosis/calcification occurs)
   • Diuretics - to relieve congestion and optimize clinical volume status (caution: may decrease preload excessively)
   • Treatment of the causative disease (e.g., anti-TB medication)
   • Avoid beta-blockers and calcium channel blockers (CCBs) (these reduce heart rate and the heart in constrictive pericarditis is heavily dependent on tachycardia to maintain cardiac output)

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5. COMPARISON TABLE: Constrictive Pericarditis vs. Restrictive Cardiomyopathy

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How to Remember the Key Distinctions for Exams:

• Beck's Triad (tamponade): Hypotension + Muffled heart sounds + Raised JVP
• Friction rub = Diagnostic of pericarditis
• Pulsus paradoxus = Hallmark of cardiac tamponade
• Pericardial knock = Heard in constrictive pericarditis
• Calcified pericardium on X-ray = Constrictive pericarditis
• Water-bottle heart on X-ray = Pericardial effusion
• Electrical alternans on ECG = Pericardial effusion / Tamponade
• PR depression + Concave ST elevation = Acute pericarditis
• TB = Most common cause of pericarditis in India
• Pericardiectomy = Definitive treatment of constrictive pericarditis
• Emergency pericardiocentesis = Treatment of cardiac tamponade