constrictive pericarditis

Etiology

• most cases are idiopathic
• post pericardiotomy syndrome following:
  • cardiac surgery
  • mediastinal irradiation
• may occur as a late complication of pericarditis
  • tuberculous pericarditis
• cardiac surgery
• connective tissue disorders
• uremia

Pathology

• non compliant pericardium
  • pericardial thickening, fibrosis, calcification
• impairment of late diastolic cardiac ventricle filling
  • early diastolic cardiac ventricle filling unimpaired
• reduced & fixed cardiac chamber volume
• diminished cardiac output
• decreased transmission of intrathoracic pressure results in enhanced ventricular interdependence
• elevation of central venous pressure
  • volume overload

Clinical manifestations

• insidious onset
  • fatigue
  • exercise intolerance
  • venous congestion
• jugular venous distension (JVD) in > 90%
  • prominent x-descent & y-descent
  • inspiratory elevation in JVD (Kussmaul's sign)
• peripheral edema
• hepatic congestion & hepatomegaly, ascites
• pleural effusion
• pericardial knock during diastole difficult to distinguish from S3
• absence of pulmonary congestion
  • > 90% have clear lung on auscultation
• diminished apical impulse
• pulsus paradoxus in < 20%

Laboratory

• serum B-type natriuretic peptide (serum BNP)
  • normal or minimally elevated; mean value 130 pg/mL (> 800 pg/mL for restrictive cardiomyopathy)
  • sensitive, but not specific test for distinguishing constrictive pericarditis from restrictive cardiomyopathy
  • does NOT substitute for cardiac catheterization with hemodynamic assessment
• erythrocyte sedimentation rate may be elevated
• complete blood count (CBC) may show leukocytosis
• adenosine deaminase in pericardial fluid to rule out tuberculosis

Diagnostic procedures

• electrocardiogram
  • low voltage
• echocardiography
  • pericardial thickening
  • respiratory variation in filling of right & left ventricles
  • reduced diastolic filling with ventricular interdependence (diastolic filling of one chamber impedes diastolic filling of the other) manifested by 'to-&-fro' diastolic motion of the interventricular septum
  • exaggerated respiratory variation in mitral & tricuspid flow velocities
  • ventricular septal shift during respiration
  • may be pericardial effusion with effusive constrictive pericarditis
    • intrapericardial pressure reduced following drainage
    • intracardiac pressures unchanged following drainage
  • right ventricular size & systolic function generally normal
  • normal left ventricular size & function, no left ventricular hypertrophy
  • myocardial thickness generally normal
  • no right atrial & right ventricular diastolic collapse
  • early mitral flow velocity increased
  • plethora (dilation) of inferior vena cava
• cardiac catheterization if echocardiography indeterminate
  • demonstration of elevated & equalized diastolic pressures in all 4 cardiac chambers
    • elevated atrial pressures
    • LV diastolic pressure within 5 mm of Hg of RV diastolic pressure
  • prominent x-descent & y-descent

Radiology

• chest X-ray may show pericardial calcification (generally absent)
• chest CT or MRI of thorax
  • may show pericardial thickening, calcification
  • more sensitive than X-ray
• radionuclide ventriculography
  • more rapid early diastolic filling in patients with constrictive pericarditis relative to restrictive cardiomyopathy

Differential diagnosis

• restrictive cardiomyopathy*
  • pericardial knock during diastole absent in restrictive cardiomyopathy, but difficult to distinguish from S3
  • restrictive cardiomyopathy more likely associated with amyloidosis (see Etiology: both disorders)
  • distinction is critical since surgery is not indicated for restrictive cardiomyopathy
  • doppler echocardiography & doppler velocity required to differentiate
• pericardial tamponade
  • early diastolic right ventricular collapse, hypotension & tachycardia consistent with pericardial tamponade
• right ventricular myocardial infarction

* see features distinguishing constrictive pericarditis

Management

• high-dose NSAID or glucocorticoid for potentially transient constrictive pericarditis (see pericarditis)
  • glucocorticoid + colchicine
• clinical improvement in 2-6 months (90%)
• definitive therapy requires complete pericardiectomy
  • perioperative mortality 5-10%
  • indicated for patients with functional NYHA class II or class III heart failure
  • 2-3 months of anti-inflammatory therapy reasonable prior to pericardiectomy
• minimally symptomatic patients
  • sodium & fluid restriction
  • diuretics
  • closely monitor for hemodynamic deterioration
• patient education
  • hemodynamic & symptomatic relief in 2-6 months (90%)
• follow-up: all patients to identify patients with extension to epicardium & myocardium

More general terms

• pericarditis

More specific terms

• effusive constrictive pericarditis

Additional terms

• features distinguishing constrictive pericarditis