What is pericardial heart disease?

What is Pericardial Heart Disease?

Pericardial heart disease is an umbrella term encompassing all pathological conditions affecting the pericardium—the double-walled sac surrounding the heart—including pericarditis (acute, subacute, chronic, and recurrent), pericardial effusion, cardiac tamponade, constrictive pericarditis, and pericardial masses 1.

Core Pathophysiology

The pericardium consists of two layers: a serous visceral layer (epicardium) and a fibrous parietal layer, which together enclose the pericardial cavity containing pericardial fluid 1. When disease occurs, the pericardial response typically involves:

• Inflammation (fibrinous, noneffusive, or exudative processes)
• Fluid accumulation (pericardial effusion)
• Adhesive or fibrocalcific responses (constriction) 2

Main Clinical Syndromes

Pericardial disease manifests as five distinct clinical scenarios 1:

1. Pericarditis

• Acute, subacute, chronic, or recurrent inflammatory process
• Can be isolated or part of systemic disease
• Presents with sharp, pleuritic chest pain worsening when supine (~90% of cases) 3
• ECG shows widespread ST-segment elevation and PR depression (25-50% of cases) 3
• Pericardial friction rub (<30% of cases) 3

2. Pericardial Effusion

• Fluid accumulation in the pericardial space
• Clinical presentation depends on speed of accumulation 1
• Rapid accumulation (trauma, iatrogenic perforation) → immediate tamponade even with small volumes
• Slow accumulation → large effusions over days to weeks before symptoms develop
• Symptoms include dyspnoea, chest fullness, orthopnoea, and compression symptoms (nausea, dysphagia, hoarseness, hiccups) 1

3. Cardiac Tamponade

• Pan-cyclic compression of cardiac chambers by increased pericardial fluid
• Cardiac chambers compete for fixed intrapericardial volume 2
• Classic signs: elevated jugular venous pressure, pulsus paradoxus, diminished heart sounds 1
• Results in diastolic dysfunction and reduced cardiac output 2

4. Constrictive Pericarditis

• Pericardium limits diastolic filling
• Causes dissociation of intracardiac and intrathoracic pressures 2
• Heightened ventricular interdependence
• Elevated and equal venous and ventricular diastolic pressures 2

5. Pericardial Masses

• Less common manifestation requiring imaging evaluation 1

Etiological Framework

Pericardial disease can be primary (isolated to the pericardium) or secondary (part of systemic disease) 1:

Common causes in North America/Western Europe:

• Idiopathic or viral (most common) 3
• Post-cardiac procedures or operations 3
• Post-cardiac injury syndromes (post-MI, post-pericardiotomy, post-traumatic) 1

Systemic disease associations 1:

• Renal failure (uraemic pericarditis, dialysis pericarditis)
• Autoimmune diseases (SLE, rheumatoid arthritis, scleroderma, Sjögren's syndrome)
• Malignancy (higher risk with tamponade without inflammatory signs) 1
• Tuberculosis (most common cause in endemic areas) 3

Diagnostic Approach

Transthoracic echocardiography is the primary diagnostic tool (Class I recommendation) 1, providing:

• Detection and semiquantitative assessment of effusion size
• Evaluation of hemodynamic effects
• Portability and widespread availability

Additional imaging modalities 1:

• CT and CMR should be considered for loculated effusions, pericardial thickening, masses, and associated chest abnormalities (Class IIa)
• Chest X-ray recommended for suspected pleuropulmonary involvement (Class I)
• CRP assessment recommended to evaluate inflammatory markers (Class I)

Clinical Significance

Pericardial disease is relatively common, accounting for up to 5% of emergency department visits for nonischemic chest pain 3. While often considered benign, complications include:

• Recurrence in 15-30% of patients despite treatment 3
• Constrictive pericarditis (<0.5% of acute cases) 3
• Cardiac tamponade (<3% of acute cases) 3
• Chronic disease with impaired quality of life in substantial proportion of patients 4

Critical pitfall: Pericardial diseases can imitate more common cardiac diseases, making diagnosis challenging 5. The absence of ECG abnormalities (particularly in renal failure patients) does not exclude pericarditis 1.