Constrictive Pericarditis

Definitions and Pathophysiology¹

Definitions

• Constrictive pericarditis is a clinical syndrome characterized by an inelastic, thickened pericardium that restricts cardiac filling.
  • This may be transient/subacute or chronic, with chronic meaning constriction persisting for more than 3 to 6 months.
• Effusive-constrictive pericarditis is defined as constrictive pericarditis and coexisting pericardial effusion.

Pericardial Physiology

• The pericardium consists of a visceral layer on the epicardial surface of the heart and a parietal layer that forms a sac surrounding the heart, which contains a thin layer of pericardial fluid.
• Under normal physiologic conditions, the pericardium stretches to accommodate changes in cardiac volume.

Pathophysiology

• Due to thickened, fibrotic, or possibly calcified pericardium, cardiac filling is impaired, resulting in an inability to accommodate changes in cardiac volumes.
• Key pathophysiologic features of constrictive pericarditis include the following:
  • Elevated venous pressure
  • Limited late diastolic filling
  • Dissociation of intracardiac and intrathoracic pressures
  • Augmented ventricular interdependence
  • Decreased ventricular and stroke volumes as progressive disease

Causes and Clinical Presentation^1,2

Causes

• The causes of constrictive pericarditis include the following:
  • Any pericardial disease
  • Infectious/Postinfectious
    • Viral
    • Tuberculosis
    • Whipple disease
    • Other bacterial infections
  • Postcardiac surgery
  • Postradiation therapy
  • Autoimmune inflammatory disease
  • Malignancy
  • Trauma
  • Drug induced
  • Asbestosis
  • Sarcoidosis
  • Uremia
  • Idiopathic

Clinical Presentation

• The clinical presentation of constrictive pericarditis may include the following signs and symptoms:
  • Volume overload
    • Peripheral edema
    • Anasarca
    • Ascites
    • Pleural effusion
    • Elevated jugular venous pressure (JVP)
  • Decreased cardiac output
    • Fatigue
    • Dyspnea on exertion
    • Reduced exercise capability
  • Chest pain
  • Atrial arrhythmia
  • Kussmaul sign (paradoxical rise in JVP during inspiration due to impaired right ventricular (RV) filling)
  • Pericardial knock (high-pitched early diastolic heart sound due to sudden cessation of RV filling from stiff pericardium)
  • Cachexia
  • Pulsatile hepatomegaly
• Cardiac tamponade may be present in patients with effusive-constrictive pericarditis. In patients with effusive-constrictive pericarditis, elevated JVP will persist despite treatment of cardiac tamponade with pericardiocentesis.

Differential Diagnosis

• The differential diagnosis for constrictive pericarditis includes:
  • Cardiac tamponade
  • Restrictive cardiomyopathy
  • Chronic liver disease and cirrhosis
  • Nephrotic syndrome or other causes of edema

Evaluation and Management^2-4

Evaluation

• Patients with suspected constrictive pericarditis should undergo the following evaluation:
  • History and physical examination
  • 12-lead electrocardiogram
    • Nonspecific ST- and T-wave changes, tachycardia, low voltages, and atrial arrhythmias may be present.
  • Chest radiograph
    • Pericardial calcification may be visible, although the absence of this does not rule out constrictive pericarditis.
  • TTE
    • TTE can confirm the diagnosis of constrictive pericarditis in many patients.
    • Ejection fraction is typically normal.
    • Findings suggestive of constrictive physiology include:
  • IVC dilation
  • Atrial dilation and increased left atrial pressure (LA volume > RA volume)
  • Exaggerated respirophasic interventricular septal shift
  • Septal bounce or shudder
  • Premature opening of the pulmonic valve due to a rapid rise in RV filling pressure during diastole
  • Pericardial thickness (more than 4mm)
  • Doppler findings include the following:
    • Increased early diastolic left ventricular (LV) and RV filling
    • Increased respiratory variation in mitral and tricuspid inflow (mitral inflow velocity > 100 cm/s)
    • Lateral MV e’ velocity > 12 cm/s
    • Lateral e’ velocity < septal e’ velocity (annular reversus)
    • Hepatic venous flow reversal during spontaneous expiration
• Inflammatory markers such as C-reactive protein and erythrocyte sedimentation rate are often elevated and may be helpful, especially if subacute or transient constrictive pericarditis is suspected.
• Additional testing may include cardiac computed tomography (CT) or magnetic resonance imaging, positron emission tomography/CT imaging, or cardiac catheterization.
• Further discussion of imaging modalities and findings in constrictive pericarditis can be found here.

Management

• The management of constrictive pericarditis depends on the chronicity of the presentation.
  • For patients with early (subacute) disease who are hemodynamically stable, initiate medical therapy aimed at treating the underlying cause and inflammation.
    • Anti-inflammatory therapy includes nonsteroidal anti-inflammatory drugs (NSAIDs) plus colchicine for 2-3 months, followed by tapering of the NSAID and discontinuation of colchicine.
    • Glucocorticoid therapy may be used if there are contraindications to NSAIDs or for patients with refractory symptoms despite NSAIDs.
    • Interleukin 1 (IL-1) inhibitors may also be used.
    • Patients who progress to chronic disease should be evaluated by cardiothoracic surgery for pericardiectomy.
  • Pericardiectomy is indicated for patients with late (chronic) disease. Medical therapy should be initiated to relieve symptoms of heart failure and congestion while awaiting surgery or for patients who are poor surgical candidates.
  • Effusive-constrictive pericarditis should be treated with pericardiocentesis initially, followed by medical treatment of the suspected underlying cause and inflammation as described above.
• The recommended treatment approach, adapted from the American College of Cardiology Consensus Statement on the Diagnosis and Management of Pericarditis and the UpToDate article on the management of constrictive pericarditis, is shown in Figure 1.^3,4

• Constrictive pericarditis can be prevented by the prompt treatment of acute and recurrent pericarditis, pericardiocentesis, and prophylactic colchicine therapy in patients undergoing pericardiectomy.