Constrictive Pericarditis

Question 1

Clinical signs of restrictive cardiomyopathy / constrictive pericarditis?

Answer 1

Extracardiac signs which may indicate a secondary restrictive cardiomyopathy.

Raised JVP (may see a brief dominant y-descent due to high RA pressures and an early rise in RV diastolic pressure due to poor pericardial compliance.

Pulsus paradoxus: >10mmHg drop in systolic BP during inspiration.

Kussmaul’s sign: paradoxical increase in JVP on inspiration (may need to sit the patient at 90 degress to observe JVP meniscus)

Pitting oedema
Ascites & hepatomegaly.

Usually present with signs and symptoms of HF in the setting of good LV systolic function.

Question 2

Causes of constrictive pericarditis?

Answer 2

Viral / bacterial pericarditis
Post-surgery (CABG - scars)
Post-TB (look for cervical lymphadenopathy, apical crackles)
Radiation (e.g., for lymphoma/lung ca - look for scars or RT tattoos)
Connective tissue disease (RA hands, SLE signs, scleroderma skin, face)

Idiopathic, post-surgical and post RT are most common causes in UK.

Question 3

Causes of restrictive cardiomyopathy?

Answer 3

Endomyocardial fibrosis
Loffler’s syndrome (eosinophilic infiltration of myocardium)
Sarcoidosis (2nd)
Scleroderma
Haemochromatosis (3rd)
Amyloidosis (most common UK cause)

Question 4

How to differentiate between restrictive cardiomyopathy and constrictive pericarditis on further imaging?

Answer 4

CXR: shows pericardial calcification in the case of constriction, may show signs of old TB

Echo: may show bright white pericardium in pericardial disease, may show ventricular independence which is pathognomonic for constrictive disease

Cardiac MRI: to look for restrictive cardiomyopathy.

Cardiac CT: to look for both restrictive / constrictive pathology.

Question 5

Why do you need to follow up with right or left heart catheterisation if any suspicion of restrictive cardiomyopathy or constrictive pericarditis?

Answer 5

Need to measure invasive haemodynamics.

The presence of ventricular interdependence: a fluctuating LV/RV pressure during respiration is highly diagnostic for constrictive disease.

Question 6

What is the difference between constrictive pericarditis and restrictive cardiomyopathy?

Answer 6

Constrictive pericarditis (CP) involves a stiff pericardium (the sac around the heart) restricting its filling, while restrictive cardiomyopathy (RCM) is caused by stiffness within the heart muscle (myocardium) itself. The primary difference is the location of the problem, leading to distinct causes, diagnostic findings, and treatment options, with CP often curable by surgery (pericardiectomy) and RCM only treatable with medications or cardiac transplantation.

Question 7

Explain interventricular independence.

Answer 7

Thickened, fibrous capsule reduces ventricular filling and insulates the heart from intrathoracic pressure changes during respiration leading to interventricular independence i.e. filling of one ventricle reduces the size and filling of another.

Question 8

How do you manage a patient with constrictive pericarditis?

Answer 8

Diuretics and fluid restriction

Pericardiectomy