Constrictive pericarditis: symptoms, diagnosis and treatment

The pericardium is a two-layered sac around the heart containing a small amount of lubricating fluid. Liverpool Heart and Chest Hospital NHS Foundation Trust explains that certain conditions can allow the sac to thicken, stiffen and stick to the heart. The heart can then not relax and fill properly, so the amount of blood it can circulate is reduced.

Patient's clinical reference describes chronic pericarditis as long-lasting, gradual inflammation of the pericardium that causes fluid to accumulate or the pericardium to thicken. It says there are two main chronic types: chronic effusive pericarditis, with fluid, and chronic constrictive pericarditis, with a thickened and fibrotic pericardium.

The British Heart Foundation's pericarditis information covers the more common acute inflammatory form. Constrictive pericarditis is a rare long-term complication that can follow it.

Because the heart cannot fill properly, symptoms resemble right-sided heart failure. The clinical reference notes that onset is typically very gradual, usually over months.

• Shortness of breath, which may be relatively slight at first.

• Fatigue.

• Swelling of the ankles and legs.

• Swelling of the abdomen from fluid, with an enlarged liver in some people.

• Distended neck veins that do not fall on breathing in, which doctors check for.

• In advanced disease, the clinical reference says people may be ill with jaundice and muscle wasting.

Liverpool Heart and Chest lists tuberculosis, viral, bacterial or fungal infections, asbestos exposure, heart attacks, autoimmune diseases and radiation exposure as causes of pericarditis. Patient's reference says constrictive pericarditis can occur after any pericardial disease process.

It lists the more common causes of constriction as idiopathic (no clear cause), viral, tuberculosis (the highest total incidence worldwide), mediastinal irradiation (5 to 10 years later) and post-surgical, including cardiac catheterisation. Less common causes include other infections, tumours, kidney failure, connective tissue disorders, drugs and trauma.

The reference says the risk is higher following bacterial forms of pericarditis, intermediate for post-pericardiotomy syndromes and systemic inflammatory diseases and low for viral and idiopathic cases.

Progressive breathlessness, leg or abdominal swelling or fatigue should be assessed by a GP, especially after previous pericarditis, heart surgery, radiotherapy to the chest or tuberculosis. The NHS advises asking for an urgent GP appointment or help from NHS 111 for sharp or stabbing chest pain that gets worse when you breathe in, swallow, cough or lie down, and calling 999 for sudden chest pain that spreads to the arms, back, neck or jaw, feels tight or heavy, or comes with breathlessness and sweating.

A history of cardiac surgery or a systemic disease that affects the pericardium makes the diagnosis more likely, according to Patient's reference, which is why that history matters when you describe your symptoms.

Patient's reference says an echocardiogram is usually diagnostic and helps distinguish constriction from restrictive cardiomyopathy, the main differential diagnosis. Calcification of the pericardium on a chest X-ray strongly suggests constrictive pericarditis in patients with heart failure.

It describes MRI estimating pericardial thickness, CT showing increased thickness and calcification, cardiac catheterisation to measure pressures and confirm diastolic dysfunction and exclude restrictive cardiomyopathy, and a pericardial biopsy if infective, malignant or granulomatous causes are suspected. The ECG may show low voltage with non-specific T-wave changes.

Because the condition is uncommon and resembles other causes of heart failure, referral to a cardiology service with experience of pericardial disease is usual.

The reference says the underlying cause should be treated where possible. Chronic constriction has a definite surgical treatment, whereas transient cases may be reversible with empirical anti-inflammatory therapy. Patients are therefore observed for improvement on medical treatment for a period, whenever possible, before proceeding to pericardiectomy.

Liverpool Heart and Chest explains that the surgery for constrictive pericarditis is called pericardiectomy (also pericardial stripping). It involves removing as much of the thickened heart sac as possible. The heart is not deliberately entered, but if the sac is very stuck, the heart can be damaged trying to remove it, and sometimes cardiopulmonary bypass is used to support the heart during the operation.

Patient's reference says early pericardiectomy with complete removal of fibrous tissue, if technically feasible, provides good symptomatic relief and is the treatment of choice before severe constriction and myocardial atrophy occur. It stresses that complete pericardiectomy is very important because symptoms may persist after partial removal.

On the NHS, diagnosis and surgery are carried out in specialist cardiology and cardiothoracic centres, with referral via the GP or local cardiology service. Because the surgery is uncommon and technically demanding, it is concentrated in experienced units.

Private cardiology can speed up imaging and consultation. For surgery, experience of the unit and surgeon with pericardiectomy matters, and it is reasonable to ask how many are performed and about outcomes, and to keep the GP informed.