Pericardial Disease

What the pericardium does

The pericardium has a tough outer fibrous layer and a thin inner layer lining the heart surface. Between them is a small amount of lubricating fluid, normally about 15 to 50 ml. The pericardium:

• lets the heart move smoothly with each beat
• anchors the heart in the chest
• limits sudden overstretching of the heart
• acts as a barrier against infection spreading from the lungs

Because the outer layer cannot stretch quickly, even a modest amount of fluid that builds up fast can compress the heart.

Types of pericardial disease

Myocarditis, inflammation of the heart muscle, often occurs together with pericarditis (myopericarditis).

Causes

• Viral infections: the most common cause of acute pericarditis in high-income countries, including coxsackievirus, echovirus, adenovirus and COVID-19. Many cases labeled "idiopathic" (no known cause) are probably viral.
• Bacterial infections: rare, but serious; tuberculosis is the leading cause in many low-income countries and a major cause of constriction.
• After heart injury: after a heart attack, heart surgery or catheter procedures, including late inflammation called Dressler syndrome or post-pericardiotomy syndrome.
• Autoimmune and inflammatory diseases: lupus, rheumatoid arthritis, scleroderma, familial Mediterranean fever.
• Cancer: spread from lung or breast cancer, lymphoma or leukemia.
• Kidney failure: uremic pericarditis.
• Radiation therapy to the chest, sometimes years later.
• Medicines: rarely, such as hydralazine, procainamide, isoniazid and some cancer drugs.
• Chest trauma and aortic dissection, which can cause sudden bleeding into the sac.
• Hypothyroidism: can cause a slowly accumulating effusion.

Symptoms

• Pericarditis chest pain: sharp or stabbing, in the center or left of the chest, worse with deep breaths, coughing or lying flat, better sitting up and leaning forward. It can spread to the shoulder ridge.
• Fever and feeling unwell.
• Breathlessness, especially with a large effusion or tamponade.
• Palpitations.
• Swelling of the legs and belly, fatigue and weight gain in constrictive pericarditis.

How pericardial disease is diagnosed

1. Listening to the heart: a scratchy "pericardial friction rub" is typical of pericarditis.
2. ECG: widespread ST elevation and PR depression in acute pericarditis.
3. Echocardiogram: the key test for effusion and tamponade, showing how much fluid there is and whether it compresses the heart.
4. Blood tests: CRP and ESR (inflammation), troponin (heart muscle involvement), blood count, kidney function, and tests for TB, autoimmune disease or thyroid disease when relevant.
5. Chest X-ray: a large, "water bottle" shaped heart in big effusions.
6. Cardiac MRI or CT: shows inflammation, thickening and calcification, and helps diagnose constriction.
7. Pericardiocentesis: draining and testing the fluid for infection or cancer cells.
8. Cardiac catheterization: sometimes needed to confirm constriction.

Treatment

Pericarditis

• NSAIDs (such as ibuprofen) or aspirin at anti-inflammatory doses prescribed by a doctor, tapered over weeks
• Colchicine for about 3 months for a first episode, which halves the risk of recurrence
• Rest from strenuous exercise until symptoms and CRP settle, at least 3 months for competitive athletes
• Steroids only when other options are unsuitable, as they increase recurrence risk
• Anti-IL-1 drugs (anakinra, rilonacept) for repeated recurrences despite colchicine

Pericardial effusion and tamponade

• Treat the cause, such as infection, thyroid disease or kidney failure
• Pericardiocentesis: a needle guided by ultrasound drains fluid, which is lifesaving in tamponade
• Pericardial window: surgery creating a drainage opening for effusions that keep returning, often in cancer

Constrictive pericarditis

• Anti-inflammatory treatment if the constriction is recent and inflammatory (sometimes reversible)
• Diuretics to reduce swelling
• Pericardiectomy: surgical removal of the stiff pericardium for chronic constriction
• Anti-tuberculosis treatment when TB is the cause

Outlook

Most acute viral pericarditis resolves within 1 to 2 weeks with treatment, and long-term outlook is excellent. About 15 to 30% of people have a recurrence without colchicine. Tamponade is fatal if not drained but recovery is good with prompt treatment. Constrictive pericarditis is rare, and surgery relieves symptoms in many people, though results depend on the cause, with radiation-related constriction doing least well.

Frequently asked questions

What are the types of pericardial disease?

The main types are acute and recurrent pericarditis, pericardial effusion, cardiac tamponade and constrictive pericarditis. Less common types include pericardial cysts, tumors and congenital absence of the pericardium. Several can occur together, such as pericarditis with an effusion.

What is the most common cause of pericardial disease?

In the US and Europe, viral infection, or an unknown cause presumed viral, is the most common cause of acute pericarditis. Other causes include heart surgery, heart attack, autoimmune diseases, cancer, kidney failure and radiotherapy. Worldwide, tuberculosis is a major cause.

Is pericardial disease serious?

It ranges from mild to life-threatening. Most acute pericarditis is short-lived and responds to anti-inflammatory medicines. Cardiac tamponade is an emergency that needs immediate drainage. Constrictive pericarditis is a chronic condition that may need surgery.

How do you know if chest pain is from the pericardium?

Pericardial pain is typically sharp, worse when lying flat, breathing deeply or coughing, and better when sitting up and leaning forward. A heart attack usually causes pressure-like pain not affected by position. Chest pain should always be checked urgently, because the two can be hard to tell apart.

Can you exercise with pericardial disease?

Not during active pericarditis. Doctors advise avoiding strenuous exercise until symptoms resolve and inflammation markers normalize, and competitive athletes usually wait at least 3 months. Gentle walking is generally fine. Ask your cardiologist before returning to sport.