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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • MRI, CT, or echocardiographic imaging showing a thickened pericardium

  • Right heart failure signs and symptoms due to impaired diastolic filling

  • Kussmaul sign and pericardial knock on exam

  • Echocardiogram shows septal bounce, respiratory variation of the mitral peak E velocity

  • Cardiac catheterization shows “dip and plateau” or “square-root” sign

GENERAL CONSIDERATIONS

  • Results from scarring as the aftermath of almost any pericardial injury or inflammation

  • In developed countries, the most common etiologies are idiopathic or viral, followed by post–cardiac surgery, post–radiation therapy, and connective tissue disorders

  • In developing countries, tuberculosis remains a major cause

  • Pericardium becomes thickened, fibrotic, and sometimes calcified (especially with tuberculosis)

  • The noncompliant pericardium encases the heart and results in a fixed cardiac volume

  • Diastolic filling is abruptly halted in early diastole when the volume limit of the noncompliant pericardium is attained

  • It is sometimes difficult to distinguish from restrictive cardiomyopathy but is important to do so because constriction may be cured with pericardiectomy

  • Some patients have normal pericardial thickness

  • Some patients undergo spontaneous resolution or respond to medical therapy

  • Although most patients have relief of symptoms after pericardiectomy, some do not

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Fatigue

  • Dyspnea

  • Increased abdominal girth

  • Edema

  • Malabsorptive diarrhea

  • Chest pain

  • Dizziness

PHYSICAL EXAM FINDINGS

  • Markedly elevated jugular venous pressure (JVP) with x and y troughs more prominent than the a and v peaks

  • Pulsus paradoxus in some

  • Kussmaul sign (lack of inspiratory decline in JVP)

  • Pericardial knock (early diastolic sound) on auscultation

  • Abdominal distention with fluid wave from ascites

  • Pulsatile hepatomegaly

  • Profound cachexia

  • Edema or anasarca

DIFFERENTIAL DIAGNOSIS

  • Cardiac tamponade

  • Restrictive cardiomyopathy

  • Right heart failure

  • Cirrhosis with ascites

  • Malabsorption syndrome

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Antinuclear antibody titer

  • Rheumatoid factor

  • Tuberculin skin test

ELECTROCARDIOGRAPHY

  • No specific ECG findings with constriction

  • Nonspecific ST- and T-wave changes common

  • Low voltage sometimes present

  • Atrial fibrillation common in advanced cases

  • P mitrale common in less severe and less chronic cases

IMAGING STUDIES

  • Chest x-ray: pericardial calcification is specific but uncommon

  • MRI is the procedure of choice to image pericardial thickness

  • CT is also extremely useful

  • Echocardiography is an essential adjunctive test

    • – Abrupt rapid movement of the interventricular septum during inspiration, termed “septal bounce,” and moderate biatrial enlargement

    • – Doppler mitral E velocity is typically increased, and exaggerated respirophasic variation in mitral inflow (mitral E velocity decreases by at least 25% with inspiration) is characteristic. The inferior vena cava is dilated and does not collapse with inspiration. Hepatic vein flow does not increase with inspiration

DIAGNOSTIC PROCEDURES

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