Constrictive pericarditis is a disorder caused by inflammation of the pericardium (the sac-like covering of the heart) with subsequent thickening, scarring, and contracture of the pericardium.

Constrictive pericarditis is a chronic form of pericarditis in which the pericardium is rigid, thickened, scarred, and less elastic than normal. The pericardium cannot stretch as the heart beats, which prevents the chambers of the heart from filling.

A direct consequence is a reduced cardiac output (the amount of blood pumped by the heart). The blood backs up behind the heart, resulting in symptoms of heart failure.

The inflamed pericardium may cause pain when it rubs against the heart.

The most common causes of constrictive pericarditis are conditions that induce chronic inflammation of the pericardium: tuberculosis, radiation therapy to the chest, and cardiac surgery.

Less frequently, constrictive pericarditis results from mesothelioma (a tumor) of the pericardium or from incomplete drainage of abnormal fluid accumulating in the pericardial sac, which can occur in purulent pericarditis or in post-surgery hemopericardium (bleeding within the pericardial sac). Constrictive pericarditis may also develop without apparent cause.

The condition is relatively rare in children.

• Difficulty breathing (dyspnea) that develops slowly and progressively worsens
• Fatigue, excessive tiredness
• Weakness
• Chronic swelling (edema) of the legs, ankles
• Swollen abdomen

Symptoms of acute (but not chronic) pericarditis also include:

• Chest pain (less than that seen with acute pericarditis)
  • Radiating to the neck, shoulder, back, or abdomen
  • Described as sharp, stabbing
  • Increases with breathing
• Splinting of ribs with deep breathing (requiring the need to bend over or hold chest when breathing deeply)
• Most comfortable position is upright (standing or sitting)
• Fever
• Sweating
• Chills
• Dry cough
• Anxiety

Constrictive pericarditis is notoriously difficult to diagnose and must be distinguished from restrictive cardiomyopathy and cardiac tamponade.

The examination of the neck veins may show that the jugular venous pressure is elevated. Neck veins may be prominent and may not decrease when inhaling (related to increased pressure in the veins). This is called Kussmaul's sign and is caused by lack of transmission of intrathoracic respiratory changes to the pericardial space and heart chambers.

Heart sounds may be weak or distant. There may be signs of hepatic (liver) congestion, such as enlargement of the liver and fluid in the abdomen (ascites). The spleen may be examined by touch. Pericardial thickening, scarring, or calcification (mineral deposits) can be verified by the results of the following tests:

• Chest x-ray
• Echocardiogram -- may also show abnormal movement of the interventricular septum
• Transesophageal echocardiography (TEE) with respiration correlates
• Echo-Doppler -- shows abnormal diastolic filling pattern (blood cannot return normally to the heart)
• Chest MRI
• Tagged cine MRI
• Chest CT scan
• Coronary angiography or cardiac catheterization
• ECG -- may show changes, atrial fibrillation is often present

Both echo-Doppler and cardiac catheterization may clearly show that during inhalation, the venous blood flow into the right atrium does not increase as it would normally.

The goal of treatment is to improve heart function. The cause must be identified and treated. This may include antibiotics, anti-tuberculosis medications, or other treatments.

Diuretics are commonly prescribed in small doses to gradually decrease excess fluid. Analgesics may be needed to control pain. Decreased activity may be recommended for some cases, and a low-sodium diet may be recommended.

The definitive treatment is surgical pericardiectomy -- cutting or removing the scarring and part of the pericardium.

Constrictive pericarditis may be life-threatening if untreated. The condition's surgical treatment (pericardiectomy), on the other hand, has a relatively high complication rate and is usually reserved for severely symptomatic cases.

For patients with radiation-induced constrictive pericarditis the long-term results of pericardiectomy are not as helpful as was once expected.

• Cardiac tamponade
• Heart failure
• Pulmonary edema
• Myocardial fibrosis (scarring of the heart muscle)
• Damage to the coronary arteries

Call your health care provider if symptoms indicate constrictive pericarditis may be present.

Constrictive pericarditis in some cases is not preventable, but if a condition of the pericardium that can lead to constrictive pericarditis exists, it should be adequately treated and the patient should undergo periodic check-ups.