Diseases of the Pericardium

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Chapter 434 Diseases of the Pericardium

Robert Spicer, Stephanie Ware

The heart is enveloped in a bilayer membrane, the pericardium, which normally contains a small amount of serous fluid. The pericardium is not vital to normal function of the heart, and primary diseases of the pericardium are uncommon. However, the pericardium may be affected by a variety of conditions (Table 434-1), often as a manifestation of a systemic illness and can result in serious, even life-threatening, cardiac compromise.

Table 434-1 ETIOLOGY OF PERICARDIAL DISEASE

CONGENITAL

Absence (partial, complete)

Mulibrey nanism (TRIM 37 gene mutation)

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome (PRG4 gene mutation)

INFECTIOUS

Viral (coxsackievirus B, Epstein-Barr virus, influenza, adenovirus, parvovirus)

Bacterial (Haemophilus influenzae, streptococcus, pneumococcus, staphylococcus, meningococcus, mycoplasma, tularemia, listeria, leptospirosis)

Immune complex (meningococcus, H. influenzae)

NONINFECTIOUS

Systemic inflammatory diseases (acute rheumatic fever, juvenile rheumatoid arthritis, systemic lupus erythematosus, mixed connective tissue disorders, systemic sclerosis, Wegener granulomatosis)

Metabolic (uremia, hypothyroidism, Gaucher disease, very long chain acyl CoA dehydrogenase deficiency)

Traumatic (surgical, catheter, nonmedical)

Lymphomas, leukemia, radiation therapy

Primary pericardial tumors

434.1 Acute Pericarditis

Robert Spicer and Stephanie Ware

Pathogenesis

Inflammation of the pericardium may have only minor pathophysiologic consequences in the absence of significant fluid accumulation in the pericardial space. When the amount of fluid in the nondistensible pericardial space becomes excessive, pressure within the pericardium increases and is transmitted to the heart resulting in impaired filling. Although small to moderate amounts of pericardial effusion can be well tolerated and clinically silent, once the noncompliant pericardium has been distended maximally, any further fluid accumulation causes abrupt impairment of cardiac filling and is termed cardiac tamponade. When untreated, tamponade can lead to shock and death. Pericardial effusions may be serous/transudative, exudative/purulent, fibrinous, or hemorrhagic.

Clinical Manifestations

The most common symptom of acute pericarditis is chest pain, typically described as sharp/stabbing, positional, radiating, worse with inspiration, and relieved by sitting upright or prone. Cough, fever, dyspnea, abdominal pain, and vomiting are nonspecific symptoms associated with pericarditis. Additionally, signs and symptoms of organ system involvement may occur in the presence of generalized systemic disease.

Muffled or distant heart sounds, tachycardia, narrow pulse pressure, jugular venous distension, and a pericardial friction rub provide clues to the diagnosis of acute pericarditis. Cardiac tamponade is recognized by the excessive fall of systolic blood pressure (>10 mm Hg) with inspiration. This pulsus paradoxus can be assessed by careful auscultatory blood pressure determination (automated blood pressure cuffs are inadequate), arterial pressure line wave form, or pulse oximeter tracing inspection. Conditions other than cardiac tamponade, which may result in pulsus paradoxus include severe dyspnea, obesity, and positive pressure ventilator support.

Diagnosis

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