9th May 2025

Pericardial Disease

Background  

Pericardial disease is a relatively common cause of chest pain seen in clinical practice, and can be seen  either as an isolated disease, or as a secondary cause of another systemic pathology. The pericardium  consists of a fibrous conical shaped sac (fibrous pericardium), and a layer of serosa that lines the fibrous  pericardium, as well as the great vessels and heart (serous pericardium). These are separated by a potential  space (estimated to be around 15 – 50ml) of plasma in healthy individuals. The pericardium keeps the  heart in a stable location attached to the mediastinum, as well as providing a lubricated surface for the  heart to beat in, equalise hydrostatic, gravitational, and inertial forces to maintain the correct geometry of  the cardiac chambers (most notably the left ventricle), and is a barrier against infection. This chapter will  concentrate on the main pathologies involving the pericardium. all of which are interconnected, with one  pericardial disease possibly resulting in another.

Epidemiology  

There are several pathologies that fall under the umbrella of pericardial disease, all of which are  interconnected, with one pericardial disease possibly resulting in another. The spectrum of clinical  presentation includes:  

1) Pericarditis/Acute pericarditis – inflammation of the pericardium. One of the most common  pericardial diseases and makes up around 5% of presentations with chest pain of non ischaemic  origin in the ED, as well as in 0.1% in all patients admitted to hospital. Acute pericarditis is a  sudden onset of pericarditis without the presence of effusion.  

2) Pericardial effusion – Excess of fluid in the pericardial space, which can be transudative,  exudative or sanguineous. Viral pericarditis being the leading cause of effusion forming, with  bacterial organisms such as Mycobacterium tuberculosum (TB) the most common cause in the  developing world.  

3) Cardiac tamponade – Fluid in the pericardial space leading to limited heart expansion and  venous return. An estimate from the United States places it at 2 in 10,000 per year, with 2%  of them being secondary to penetrating injuries to the chest.  

4) Constrictive pericarditis – long term inflammation of the pericardium causing thickening and  scarring. Rare in adults, and very rare in children, but increased risk if undergone cardiac  surgery.  

The aetiology of acute pericardial disease is varied and complex, however there are certain associated risk  factors:  

• Infection – viral, bacterial including Mycoplasma tuberculosum  
• Malignancy  
• Post cardiac injury eg previous MI  
• Drugs and toxins  
• Radiation 
• Systemic illnesses/autoimmune disease
• End stage renal failure, uraemia 
• Penetrating injuries to the chest wall including trauma or surgery
• HIV/AIDS
• Male sex (in acute pericarditis)

Pathophysiology and Aetiology 

Table 1: Causes of pericarditis – RCEM learning

Pericarditis 

Pericarditis is caused by inflammation of the pericardial  layers. The causes of pericarditis are widespread and can be  separated into two different groups; non-infectious and  infectious. However, around 80-90% are ‘idiopathic’ in  nature.  

Pericarditis can be split into 3 different categories:  

1. Acute pericarditis – no effusion seen in the acute phase  
2. Subacute pericarditis – no effusion, but transient  constriction days to weeks after initial presentation  
3. Chronic pericarditis – >3 months in duration. Effusion may be present.  

Acute and subacute pericarditis usually have the same  aetiology (listed in Table 1). Chronic pericarditis can occur  following an acute episode of pericarditis, or have its own  aetiology, most commonly metastatic disease (lung cancer  being the most common), but also rheumatological  conditions (such as SLE, RA, and Behçet’s disease).

Pericardial effusion 

These conditions (chronic or acute) if left untreated can lead  to formation of a pericardial effusion. Normal volumes of  fluid range from 15ml – 50ml. However, in pericarditis, this  amount of fluid can increase. The fluid may be transudative,  exudative or serosanguinous depending on the aetiology: 

• Transudative – Heart failure, hypoalbuminaemia, cirrhosis, chest wall irradiation, renal insufficiency
• Exudative – viral/bacterial infection, malignancy, inflammatory/autoimmune  
• Serosanguinous – blunt trauma, post cardiac surgery, uraemia, also malignancy

Cardiac Tamponade

Fluid accumulation from pericardial effusion causes increased pressure in the pericardial sac, causing  impaired diastolic filling of the left and right heart. Reduced filling of the right heart can cause venous  congestion, and reduced filling of the left heart can cause a drop in stroke volume. Increased pressure in  the pericardial sac may cause compression of the heart, especially the right heart, due to a thinner wall.  Impaired diastolic filling of the right heart causes venous congestion. Reduction in the diastolic filling of  the left ventricle results in decreased cardiac output and eventually shock. This is a medical emergency  called cardiac tamponade. Cardiac tamponade can occur with small rapid increases in volume of the  pericardial fluid (around 100-150ml), with a classical example being from a traumatic cardiac injury  (blunt or penetrating trauma). However, in chronic settings where there is gradual build-up such as in autoimmune disease, neoplasms or chronic pericarditis, the pericardial effusion may become 1L – 2L in  size before it causes cardiac tamponade.  

Constrictive Pericarditis 

During healing of an acute/chronic pericarditis or resorption of pericardial effusion, granulation tissue  forms on the pericardium. This granulation tissue gradually contracts over time and encases the heart and  may get calcified. This creates pericardial noncompliance (stiff ventricle), leading to increased diastolic  pressures and more rapid rise in ventricular pressures for a given venous return, causing reduced  ventricular filling as compared to a normal pericardium. This results in decreased end diastolic volume  and decreased stroke volume and cardiac output. Approximately 9% of patients with acute pericarditis  develop constrictive physiology. In developed nations, the leading cause of this condition is idiopathic or  post-viral infection with incidence being 40% to 60% of total cases.

Diagnosis  

The diagnosis of pericardial disease should be a deduced from a combination of:  

• Thorough clinical history (including relevant risk factors)  
• Signs and symptoms on physical examination  
• Simple tests such as an ECG, biochemistry profiles  
• Imaging modalities such as echocardiography  

Table 2:  describes the findings for each pericardial disease condition

Management

Treatment of pericardial disease is dependent on the type of pericardial disease manifestation, as well as  severity. The management involves a range of conservative watching and waiting measures, medical  management, and procedural measures.

Pericarditis  

For pericarditis, first line therapy is NSAIDs with the goal of analgesia as well as resolution of inflammation. Indomethacin, or aspirin are commonly used. Patients who respond poorly or slowly to NSAIDs  may use Colchicine. It is also effective  in preventing recurrence of pericarditis, especially in the 72-hour  period. However the current treatment regime recommends a  combined therapy of both NSAIDs  and colchicine.

Table 3: treatment of pericarditis – adapted from European society of cardiology

Pericardial effusion 

Treatment of pericardial effusion if not causing any haemodynamic compromise is mainly conservative  watchful waiting (sometimes with serial echocardiography if indicated), with identification and  management of the initial cause of the effusion. Larger effusions may also merit a diagnostic  pericardiocentesis.

Cardiac Tamponade 

The patient will most likely be shocked, and so in the first instance should be provided with oxygen,  volume expansion with crystalloid fluid, lower limb elevation. Vasopressor support may be needed. The  definitive treatment will involve removing the fluid in the pericardial space that is causing haemodynamic compromise by bedside pericardiocentesis. This is performed either using traditional landmark technique  in a sub-xiphoid window or using a point-of-care echo to guide needle placement in real-time. Patients  may also undergo a thoracotomy using the ‘clam shell’ approach, but this is reserved for traumatic arrests  with suspected tamponade.  

Constrictive Pericarditis 

Diuretics can be used to reduce oedema or elevated venous pressure for control of symptoms. Patients  with newly diagnosed constrictive pericarditis who are hemodynamically stable and do not have signs or  symptoms of a chronic constriction (complete obliteration of the pericardial cavity by granulation tissue)  may be treated with anti-inflammatory agents for up to three months with close monitoring. The  definitive management however is a surgical pericardiectomy. There is a significant risk of mortality  associated with this procedure, therefore careful consideration of the co-morbidities of the patient as well  as the severity of the pericarditis must occur before a decision is made.

Complications  

There are many possible complications of pericardial disease, and as previously said, the diseases of  pericardial disease are often interconnected (e.g A pericardial effusion could grow large enough to cause  cardiac tamponade). Other complications include:  

• Recurrent pericarditis  
• Myopericarditis  
• Impaired cardiac function  
• Pathology related to treatment – high mortality rate in surgical pericardiectomy, puncture of  large vessels during pericardiocentesis  
• Heart failure  
• Shock – from tamponade/constrictive pericarditis  
• Hepatomegaly, pulmonary hypertension, renal failure – signs of right sided heart failure caused  by constrictive pericarditis  
• Death

References  

1. https://www.ncbi.nlm.nih.gov/books/NBK482256/#:~:text=Introduction,%5D%5B2%5D%5  B3%5D  
2. https://www.uptodate.com/contents/etiology-of-pericardial-disease 
3. https://www.uptodate.com/contents/acute-pericarditis-treatment-and-prognosis 4.  https://www.rcemlearning.co.uk/reference/pericarditis/#1570789440709-8d65dbc0-ea49 5.  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume  15/Management-of-acute-pericarditis-treatment-and-follow-up  
4. https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/pericardi  al-disease/  
5. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878263/  
6. https://www.ncbi.nlm.nih.gov/books/NBK431089/  
7. https://www.ncbi.nlm.nih.gov/books/NBK431080/  
8. https://www.ncbi.nlm.nih.gov/books/NBK431090/  
9. https://www.ncbi.nlm.nih.gov/books/NBK459314/  
10. https://www.ncbi.nlm.nih.gov/books/NBK482292/#:~:text=In%20cardiac%20tamponade%2  0resulting%20in,referred%20to%20as%20ventricular%20interdependence. 
11. https://bestpractice.bmj.com/topics/en-gb/458  
12. https://bestpractice.bmj.com/topics/en-gb/3000214  
13. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203036/#:~:text=The%20x%20descent%2  C%20which%20corresponds,absent%20in%20patients%20with%20tamponade.  
14. https://litfl.com/pericarditis-ecg-library/  
15. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7539677/  
16. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3936968/