Pericardial Disease Center

The pericardium is a protective, fluid-filled sac that surrounds the heart. It supports proper heart function and protects the heart from infection and trauma. Pericarditis is inflammation of the pericardium. Pericarditis is characterized by chest pain with associated electrocardiographic changes and usually pericardial effusion (fluid in the pericardium).

Pericarditis can result from various factors such as infections, post-cardiac injury syndrome, autoimmune disease, cancer and cancer treatments. In most cases, there is no cause identified and these cases are considered idiopathic.

• Idiopathic: For some cases of pericarditis there are no known causes. This makes up for about 55% of patients diagnosed with pericarditis.
• Post-cardiac injury: Pericarditis can be caused by post-cardiac injuries from various cardiac testing and interventions. For example, pericarditis can be caused as a result of cardiac surgery, pacemaker insertion, radiofrequency ablation, transcatheter aortic valve implantation, and, rarely, percutaneous coronary intervention. Post-cardiac injury related pericarditis makes up for about 20% of patients diagnosed with pericarditis.
• Infection: Pericarditis can originate from various bacterial, viral, and fungal infections. These include myobacterium tuberculosis, Borrelia burgdorferi, Coxsackievirus, Echoviruses, Adenoviruses, Herpesviruses (EBV, CMV), HIV, Streptococcus pneumoniae, Staphylococcus aureus (including MRSA), Candida, and Histoplasma capsulatum. Infection related pericarditis makes up for about 14% of patients diagnosed with pericarditis.
• Cancer and cancer therapies: Conditions characterized by rapidly dividing cells, commonly referred to as cancer, can lead to pericarditis when the heart becomes a target of inflammatory immune cells. This inflammation can result directly from the cancer itself or from cancer treatments, such as immune checkpoint inhibitors (ICIs) and radiation therapy. Cancer-related pericarditis accounts for approximately 5% of all pericarditis cases.
• Other diseases: Autoimmune disorders and hypothyroidism are additional conditions that can lead to pericarditis, as they involve the immune system attacking the body, with the heart being a specific target. Disease related pericarditis accounts for approximately 5% of all pericarditis cases.

Types of Pericarditis

Pericarditis is classified into four types based on timeline of symptoms: acute, incessant, chronic, and recurrent. Acute pericarditis may recur in 20% to 30% of cases, and half of these patients may experience multiple recurrences. Incessant pericarditis lasts for more than 4 to 6 weeks without a symptom-free period. Recurrent pericarditis involves new symptoms appearing after a break of 4 to 6 weeks. Chronic pericarditis persists for over three months.

Acute Pericarditis

Acute pericarditis is the most common pericardial disease. There are four diagnostic criteria for pericarditis: chest pain that is typical for pericarditis (positional, pleuritic), a distinctive sound from the heart (pericardial rub), new widespread changes in the heart's electrical activity on an electrocardiogram (ST-segment elevation or PR depression), and new or worsening fluid around the heart (pericardial effusion). Additional supportive signs include high levels of inflammation markers in the blood and evidence of pericardial inflammation on imaging tests like CT or cardiac MRI.

 

 

Treatment for acute pericarditis typically involves taking high-dose aspirin or NSAIDs for about 2 weeks, with medications to protect the stomach. Colchicine (another anti-inflammatory medication) is also recommended to help reduce symptoms and recurrence. Imaging tests that may be used to monitor pericardial disease include chest x-ray, echocardiography, CT, cardiac MRI, and PET scans.

Low-risk patients can usually be treated at home, while those with one or more risk factors may need to be hospitalized (such as patients with a large pericardial effusion or with evidence of heart inflammation). Patients are considered high risk if they have a fever over 38°C, large fluid buildup around the heart, heart compression (cardiac tamponade), or no improvement after one week of anti-inflammatory treatment.

Recurrent Pericarditis

Acute pericarditis can recur in 20% to 30% of cases, and among those who experience a recurrence, up to 50% may have additional episodes. Recurrent pericarditis is thought to be an immune-mediated phenomenon after incomplete treatment of the initial pericarditis. The clinical presentation of recurrent pericarditis is similar to acute pericarditis; diagnosis requires a symptom-free interval of 4 to 6 weeks and evidence of new pericardial inflammation. Patients are often treated with colchicine, and corticosteroids or other immunosuppression such as IL-1 blockade (example: rilonacept).

Pericarditis can lead to complications such as cardiac tamponade, where fluid buildup compresses the heart, and constrictive pericarditis, where the pericardium becomes stiff. The risk of developing constrictive pericarditis increases with chronic inflammation. Constrictive pericarditis can develop with or without fluid buildup (effusion). Diagnosis typically involves echocardiography in patients with a history and physical findings suggesting high clinical suspicion. Symptoms may include fatigue, exercise intolerance, dyspnea, anorexia, and heart failure.

Surgical options for pericardial diseases include creating a pericardial window to drain fluid into the pleural space and to prevent tamponade, and pericardiectomy (surgical removal of the pericardium) for treating constrictive pericarditis.

Ongoing Research

UCSF is actively engaged in various research initiatives to better understand the mechanisms of pericarditis and to identify biomarkers of pericardial disease progression. One key area of research involves identifying the underlying causes of pericarditis, with the goal of developing novel diagnostics and treatments. The INFLAME Biorepository is one of our biobanks in which we gather biospecimens from individuals suspected or confirmed to have pericarditis, aiming to enhance our understanding of the biological attributes of inflammatory heart conditions, including pericarditis and myocarditis.

Resources:
Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC). Eur Heart J. 2015;36(42):2921-2964. doi:10.1093/eurheartj/ehv318.

Altan M, Toki MI, Gettinger SN, et al. Immune checkpoint inhibitor-associated pericarditis. J Thorac Oncol. 2019;14(6):1102-1108. doi:10.1016/j.jtho.2019.03.015.

Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol. 2004;43(8):1445-1452. doi:10.1016/j.jacc.2003.11.039.

Chiabrando J, Bonaventura A, Vecchié A, et al. Management of Acute and Recurrent Pericarditis: JACC State-of-the-Art Review. JACC. 2020;75(1):76-92. doi:10.1016/j.jacc.2019.11.021.

Gouriet F, Levy PY, Casalta JP, et al. Etiology of pericarditis in a prospective cohort of 1162 cases. Am J Med. 2015;128(7):784 e1-8. doi:10.1016/j.amjmed.2015.02.009.

Imazio M, Brucato A, Cemin R, et al. A randomized trial of colchicine for acute pericarditis. N Engl J Med. 2013;369(16):1522-1528. doi:10.1056/NEJMoa1208536.

Maisch B, Rupp H, Ristic A, Pankuweit S. Pericardioscopy and epi- and pericardial biopsy - a new window to the heart improving etiological diagnoses and permitting targeted intrapericardial therapy. Heart Fail Rev. 2013;18:317-328. doi:10.1007/s10741-012-9334-4.

Yan KL, Lee YJ, and Baik AH. Acute radiation-induced pericarditis complicated by polymicrobial infectious pericarditis in a patient with mediastinal sarcoma: a case report. European Heart Journal: Case Reports. 2024; 8(2) 

Zayas R, Anguita M, Torres F, et al. Incidence of specific etiology and role of methods for specific etiologic diagnosis of primary acute pericarditis. Am J Cardiol. 1995;75(5):378-382. doi:10.1016/s0002-9149(99)80558-x.