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 Pathology of Pericardial Disease

Dr Sampurna Roy MD

 

 
Cardiac Pathology Online

 

http://www. histopathology-india.net/ HeartIndex. htm

The pericardium provides an enclosed lubricated space for the heart and functions to fix the heart in the chest cavity relative to adjacent organs.

Pericardial pathophysiology is often manifested in a spectrum of distinct cardiac and systemic disease states.

The pericardial response to injury typically involves a spectrum of inflammation with both acute and chronic features and/or fluid accumulation.

Recent advances in imaging methods have refined the diagnosis and therapy of pericardial disease.

 

Futher reading:

Pericardial diseases.

Pericardial disease--anatomy and function.

Pericardial disease: value of CT and MR imaging.

Spectrum of pericardial disease: part II.

 

The diseases of the pericardium include inflammatory conditions and effusions. Important features of these lesions have been briefly discussed:

I- Pericarditis

Pericarditis is usually secondary to disorders involving the heart, or adjacent mediastinal structures/

(Example:  Myocardial infarct , trauma, radiation, tumour or infections).

Rarely, it may be due to systemic abnormalities (uremia, diabetes mellitus, chronic nephritis, severe anemia , autoimmune disease).

Histopathology reporting of Pericardial Specimen

Classification of Pericarditis on the basis of etiological factors:

1. Acute nonspecific (idiopathic)

2. Infective : (a) Bacterial  (b) Viral  (c) Other infections

3. Immunologic : (a) Rheumatic fever (b) Other connective tissue disorder

4. Neoplastic

5. Metabolic : (a) Uremic (b) Myxedema (c) Gout.

6. Traumatic (including after cardiac surgery)

7. Associated with myocardial infarction.

Acute pericarditis is most often of viral origin.

Chronic pericarditis is seen in tuberculosis and fungal infections, which on healing may lead to damaging adhesion. Terminal pericarditis is seen in chronic debilitating diseases e.g. Uremia,

Acute Pericarditis : 

It may occur in several forms:

Serous Pericarditis:

This form usually consists of 50 to 200 ml of slowly accumulating exudates characteristically produced by nonbacterial involvement, including rheumatic fever, systemic lupus erythematosus, tumours, uremia and primary viral infection (Example: Coxsackie).

Microscopically, there is scant epicardial or pericardial acute and chronic inflammatory infiltrates.

Fibrinous and Serofibrinous Pericarditis:  

This is the most common clinical form, seen in myocardial infarct with a pericardial friction rub.

Exudate may be completely resolved or be organized causing adhesive pericarditis.  

Purulent (suppurative) Pericarditis:  

This is due to bacteria, fungus or parasitic infection.

Infection reaches by direct extension, by hematogenous or lymphatic route from the neighbouring areas of infection.

Example: Pneumonia, empyema, lung abscess, subphrenic abscess, liver abscess etc or during cardiotomy.

Most common causative organisms are Staphylococci, Streptococci, and Pneumococci. 

Purulent pericarditis is composed of 400 to 500 ml of a thin to creamy pus with erythematous, granular serous surfaces. 

The patient presents with fever, rigor and a friction rub.

It usually organizes and may produce mediastinopericarditis or constrictive pericarditis.

Hemorrhagic Pericarditis:

This is composed of an exudates of blood admixed with fibrinous to suppurative effusion.

Most commonly it follows cardiac surgery or is associated with tuberculosis or malignancy.

It usually organizes with or without calcification.

Caseous Pericarditis:

This form is due to tuberculosis (by direct extension from neighbouring lymphnodes) or less commonly, mycotic infection.

This type most frequently, causes fibrocalcific constrictive pericarditis.

Chronic Pericarditis:

Acute pericarditis may heal by resolution or by pericardial fibrosis ranging from a thick, pearly, nonadherent epicardial plaque, to thin delicate adhesion to massive adhesions. 

In some cases the cause is unknown.

Accordingly, chronic pericarditis may be of the following types:

1. Adhesive pericarditis :

Chronic pericarditis with adhesion between parietal and visceral pericardium is called adhesive pericarditis. These are mostly seen in rheumatic disease. Less commonly, it may be due to infection by pyogenic bacteria, tuberculosis etc. In some cases, the cause is unknown.

2. Adhesive Mediastinopericarditis :

Here, the pericardial sac is obliterated due to adhesion between two layers of pericardium as well as between parietal pericardium and surrounding mediastinal structures, chest wall & diaphragm.

The heart thus contracts against all the surrounding attached structures leading to hypertrophy and dilation.

3. Constrictive Pericarditis :  

There is marked thickening of the parietal pericardium with less involvement of visceral pericardium causing constriction of great vessels entering and leaving heart.

The pericardial space is obliterated by a dense fibrous tissue, which is often calcified.                      

Cardiac hypertrophy and dilation cannot occur because of the dense enclosing scar and the heart becomes smaller. Tuberculosis is the most common cause.  

Occasionally,  it may be due to pyogenic infection and in some, the cause is unknown but never rheumatic.

The patients of pericarditis may develop ascites and due to long standing ascites, liver & spleen are coated with fibrin.

Later, there is fibrosis of the liver (cardiac cirrhosis). 

Pleura may be involved similarly.

This polyserositis is known as Pick’s disease.

 
                                   

Further reading:

Pericardial syndromes: an update after the ESC guidelines 2004.

Transient constrictive pericarditis: an elusive diagnosis.

Recurrent pericarditis: autoimmune or autoinflammatory?

[Recurrent pericarditis--diagnostic and therapeutic implications].

Clostridium sordellii as a cause of constrictive pericarditis with pyopericardium and tamponade.

Bacterial pericarditis caused by Lactobacillus iners in an infant.

Bacterial pericarditis caused by infected trichilemmal cyst.

A Clearer View of Effusive–Constrictive Pericarditis.

Effusive–Constrictive Pericarditis.

Tuberculous pericarditis: a diagnostic quandary.

Constrictive pericarditis presenting as a large mediastinal mass causing functional tricuspid and pulmonary stenosis.

Mixed constrictive pericarditis and restrictive cardiomyopathy in a 36-year-old female.

Clinical case of the month. Purulent pericarditis in a patient with pulmonary sarcoidosis].

Occult malignancy presenting as constrictive pericarditis.

Constrictive pericarditis with extensive calcification.

Constrictive pericarditis presenting with an outpouching of the right ventricle free wall simulating an aneurysmal dilatation.

Familial Mediteranean fever with protein-losing enteropathy due to constrictive pericarditis.

Melioidosis pericarditis mimicking tuberculous pericarditis.

Cardiogenic shock following cesarean delivery due to undiagnosed tuberculous constrictive pericarditis.

Pericarditis and pleuritis associated with human parvovirus B19 infection in a systemic lupus erythematosus patient.

Diagnostic issues in the clinical management of pericarditis.

Constrictive pericarditis and rheumatoid nodules with severe aortic incompetence.

Purulent pericarditis caused by Haemophilus parainfluenzae.

A child with purulent pericarditis and Streptococcus intermedius in the presence of a pericardial teratoma: an unusual presentation.

Acute pericardial disease: approach to the aetiologic diagnosis.

II- Pericardial Effusion:

Visit related post -Pathology of Pericardial  Effusion

June 2014

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

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