Constrictive pericarditis

Constrictive pericarditis

I. What every physician needs to know.

Constrictive pericarditis represents the end stage of an inflammatory process involving the pericardium, resulting in thickening, dense fibrosis, and adhesions of the pericardium. The scarring forms a non-compliant shell around the heart and prevents it from increasing output in response to peripheral demand. The scarring impairs diastolic filling of the heart, leading to systemic venous congestion, fluid overload, and low cardiac output. The overall clinical features mimic a restrictive cardiomyopathy, and establishing the diagnosis often is challenging and requires extensive testing. If the diagnosis is made early, treatment is effective and consists of surgical removal of the shell of constricting fibrous tissue (pericardiectomy).

II. Diagnostic Confirmation: Are you sure your patient has constrictive pericarditis?

The overall clinical features mimic a restrictive cardiomyopathy, and establishing the diagnosis often is challenging and requires extensive testing.

A. History Part I: Pattern Recognition:

Symptoms onset is often insidious. Patients present with symptoms relating to elevated right-sided heart failure and fluid overload (peripheral edema and hepatomegaly with ascites). Symptoms of gut edema (nausea, vomiting, and pain) are also often present. Patients with more progressive disease have symptoms of low cardiac output (dyspnea and exercise intolerance).

B. History Part 2: Prevalence:

Constrictive pericarditis is a rare disease and accurate data regarding frequency and prevalence is lacking. The frequency of the disease depends on the incidence of the specific causes. A prior history of pericarditis may or may not be present. In the developing world, tuberculosis is an important cause of constrictive pericarditis. In developed countries, pericarditis, cardiac surgeries, and radiation therapy are the most common etiologies. Other causes include malignancy, chronic uremia, trauma, and inflammatory diseases such as systemic lupus erythematosus or sarcoidosis. The majority of cases, however, are idiopathic. Based on some case series, there is suggestion of a male predominance.

C. History Part 3: Competing diagnoses that can mimic constrictive pericarditis

Given the insidious onset and similar presentation, patients with constrictive pericarditis are often misdiagnosed with ascites due to liver cirrhosis. Similar symptoms and findings can also be found in patients with other cardiomyopathies, cardiac tamponade, right ventricular infarction, and pulmonary hypertension. Relatively normal brain natriuretic peptide (BNP) and clear lungs may cause physicians to prematurely exclude a cardiac etiology of volume overload. Echocardiography reliably distinguishes constrictive pericarditis from the above differential diagnoses, except for restrictive cardiomyopathy, which often requires more invasive testing.

D. Physical Examination Findings.

Patients present with findings of right-sided heart failure, including peripheral edema, jugular venous distension, hepatojugular reflux, hepatomegaly, and ascites. Accurate assessment of jugular venous pressure (JVP) is crucial as JVP is elevated in nearly all patients. The contour of the JVP often shows a rapid Y-descent due to rapid but brief early diastolic ventricular filling. Patients may also have Kussmaul’s sign (paradoxical rise in JVP with inspiration), pericardial knock (extra heart sound occurring slightly earlier than an S3), and pulsus paradoxus (exaggerated drop in systemic blood pressure during inspiration).

E. What diagnostic tests should be performed?

A mix of laboratory tests and imaging studies are required (as described in the sections below).

1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

There is no single diagnostic laboratory test. Passive congestion of the liver can cause abnormal liver function tests. Low cardiac output leading to cardiorenal syndrome can cause impaired renal function. Hyponatremia is seen with fluid overload. Hypoalbuminemia is seen with protein-losing enteropathy from chronic hepatic congestion. BNP is typically only slightly elevated because the constrictive pericardial sac prevents myocardial stretch. A normal BNP level may mislead the clinician early in the course of the disease. Antinuclear antibody, rheumatoid factor, or purified protein derivative (PPD) skin tests may be obtained depending on the level of suspicion for underlying disease.

Once the diagnosis is made, erythrocyte sedimentation rate (ESR) and C-reactive protein may be sent to help identify patients with ongoing pericardial inflammation. Nutritional status markers, such as albumin, prealbumin, and C-reactive protein should be obtained in anticipation of surgery.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Echocardiogram with simultaneous recording of respiration is the test of choice, though no single finding is pathognomonic for constrictive pericarditis. Typical echocardiographic findings include normal systolic function, pericardial thickening, abnormal ventricular septal motion (septal bounce), distended inferior vena cava, hepatic vein expiratory diastolic reversal, and exaggerated transvalvular flow with respiratory cycle. Any concern for constrictive pericarditis should be noted in the imaging requisition so that the cardiac sonographer may perform specific imaging sequences to help confirm or refute the diagnosis.

Other imaging modalities can offer important clues, however they can often be inconclusive. Chest X-ray may show calcification of the pericardium, but is neither sensitive nor specific in diagnosing constrictive pericarditis. Volumetric and high-resolution computed tomography (CT) scans are more sensitive at detecting small amounts of calcification and may detect pericardial thickening. Testing characteristic of cardiac magnetic resonance imaging (MRI) is similar to CT, and in addition MRI may demonstrate abnormal septal motion and ongoing pericardial inflammation. However, even CT and MRI miss pericardial thickening in up to 28% of patients with surgically proven constrictive pericarditis.

Unlike acute pericarditis, no specific electrocardiogram (ECG) pattern is associated with chronic constrictive pericarditis. Nonspecific T wave flattening and inversion as well as atrial arrhythmia may be seen. Low voltage is not commonly present.

Despite extensive noninvasive workup, right heart catheterization is still considered the “gold standard” to confirm the diagnosis and differentiate constrictive pericarditis from restrictive cardiomyopathy. Both constrictive and restrictive pericarditis cause elevated and equalized diastolic pressures on cardiac catheterization. Additionally in both conditions, early ventricular filling is rapid and blunted late by the pericardial sac, leading to the characteristic square root sign on ventricular pressure tracings. Features distinguishing constrictive pericarditis are ventricular interdependence and discordant changes in left ventricular (LV) and right ventricular (RV) pressure during respiration. This is caused by increased blood flow to the right ventricle during inspiration causing the interventricular septum to compress the left ventricle.

If the diagnosis is still unclear despite right heart catheterization, direct inspection and pericardial biopsy may be required, but this is a more invasive procedure. Pathologic findings of the pericardium include fibrosis, chronic inflammation, granulomas, and calcification.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

Unfortunately, confirming a diagnosis of constrictive pericarditis is challenging and often requires extensive workup.

III. Default Management.

A. Immediate management.

Loop diuretics can be used for volume overload, however they must be used with caution as patients with constrictive pericarditis can be very dependent on preload, and dehydration can cause a marked reduction in cardiac output.

In patients with evidence of ongoing pericardial inflammation, a short course anti-inflammatory (nonsteroidal anti-inflammatory drugs, colchicine, or corticosteroids) may be considered.

B. Physical Examination Tips to Guide Management.

Weight, JVP, and degree of peripheral edema should be closely monitored. Note that early on the course, JVP may be so elevated that it can only be seen with the patient sitting at a 90° angle.

C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.

Renal function should be closely monitored for overdiuresis. Liver function test often improves significantly with resolution of hepatic congestion.

In patients receiving anti-inflammatory therapy, serial ESR and C-reactive protein may help monitor response to therapy.

D. Long-term management.

Constrictive pericarditis is a progressive disease with poor overall prognosis. Although a small number of cases may resolve with anti-inflammatory treatment (transient constrictive pericarditis), most patients will eventually require surgical pericardiectomy for definitive treatment. Removal of densely adherent pericardium is usually successful but can be extremely challenging and recovery can be prolonged. Operative mortality is as high as 20%, but if they survive patients can have dramatic relief of symptoms and good long-term prognosis. Given the limitation of medical management, early referral to cardiothoracic surgery is recommended.

E. Common Pitfalls and Side-Effects of Management

Though patients with constrictive pericarditis can be markedly volume overloaded on presentation, care must be taken to avoid overdiuresis as they are very dependent on preload. Additionally, afterload-reducing agents can result in precipitous and dangerous drops in blood pressure as patients cannot augment cardiac output. Finally, nodal-blocking agents should be avoided as these prevent the compensatory tachycardia required to maintain adequate cardiac output.

IV. Management with Co-Morbidities

Patients often present late in the course of disease. Chronic hepatic congestion can lead to cirrhosis. Chronic low-output state can lead to chronic kidney disease and anemia of chronic disease. Other sequelae include severe deconditioning and malnutrition (cardiac cachexia). The severity of these factors can make patients very high-risk operative candidates. Unfortunately, patients with advanced disease (abnormal ventricular function, severely reduced cardiac output, cachexia, or end-organ dysfunction) derive the least benefit from surgical pericardiectomy.

A. Renal Insufficiency.

Patients with underlying chronic kidney disease are even more prone to overdiuresis, and great care must be taken in titrating diuretic doses.

B. Liver Insufficiency.

No change in standard management.

C. Systolic and Diastolic Heart Failure

The pathophysiology of constrictive pericarditis is dysfunction of diastolic filling. Concurrent systolic dysfunction is uncommon until very late in the course of disease, at which point prognosis is uniformly dismal.

D. Coronary Artery Disease or Peripheral Vascular Disease

Prior to pericardiectomy, patients with risk factors for coronary artery disease should undergo cardiac catherization with coronary angiogram. Concomitant coronary artery disease can be addressed at the time of surgical pericardiectomy with coronary bypass grafting.

E. Diabetes or other Endocrine issues

No change in standard management.

F. Malignancy

No change in standard management.

G. Immunosuppression (HIV, chronic steroids, etc).

No change in standard management.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change in standard management.

I. Gastrointestinal or Nutrition Issues

No change in standard management.

J. Hematologic or Coagulation Issues

No change in standard management.

K. Dementia or Psychiatric Illness/Treatment

No change in standard management.

V. Transitions of Care

A. Sign-out considerations While Hospitalized.

The therapeutic window of diuresis is small, and in-and-outs must be monitored closely. If undergoing diuresis, patients may require aggressive repletion of potassium and magnesium to prevent arrhythmia. If a patient decompensates, overdiuresis should be suspected.

B. Anticipated Length of Stay.

Length of stay is often prolonged due to difficulty of making the diagnosis.

C. When is the Patient Ready for Discharge.

Patients can be discharged when they are euvolemic on a stable diuretic regimen with stable renal function.

D. Arranging for Clinic Follow-up

1. When should clinic follow up be arranged and with whom.

Close follow-up with cardiology and cardiothoracic surgery clinic should be arranged prior to discharge.

2. What tests should be conducted prior to discharge to enable best clinic first visit.


3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

Renal function should be monitored to assess adequacy of diuresis in an outpatient setting.

E. Placement Considerations.


F. Prognosis and Patient Counseling.

Prognosis depends on the stage of disease and underlying comorbidities. If the patient is deemed to be an appropriate surgical candidate, prognosis can be quite good. However, if comorbidities and poor nutrition status preclude surgery, prognosis can be dismal, and palliative care consultation should be considered.

VI. Patient Safety and Quality Measures

A. Core Indicator Standards and Documentation.

Constrictive pericarditis can be coded as congestive heart failure and would need to meet the corresponding Joint Commission core measure. Left ventricular function should be documented. Given that ejection fraction (EF) is not typically reduced, patients usually do not have to be discharged on angiotensin-converting enzyme inhibitors (ACEI). Smoking cessation counselling must be provided to all smokers. Discharge instructions must include dietary instructions, exercise level, weight monitoring, and comprehensive list of discharge medications. Pneumonia and influenza vaccine should be given if no contraindication exists.

B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.

As inpatient, deep vein thrombosis (DVT) prophylaxis should be used if there are no indications. Patients should also receive pneumococcal and influenza vaccine prior to discharge. Upon discharge, patients should be counselled extensively regarding low salt diet, fluid restriction, and weighing themselves at home.

VII. What's the evidence?

Reed, MC, Dhaliwal, G, Saint, S, Nallamothu, BK. “Clinical problem-solving. The right angle”. . vol. 364. 2011. pp. 1350-1356.

Welch, TD, Oh, JK. “Constrictive Pericarditis: Old Disease, New Approaches”. . 2015. pp. 17-20.

Syed, FF, Schaff, HV, Oh, JK. “Constrictive pericarditis–a curable diastolic heart failure”. . vol. 11. 2014. pp. 530-44.

Talreja, DR, Nishimura, RA, Oh, JK, Holmes, DR. “Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory”. . vol. 51. 2008. pp. 315-319.

Yared, K, Baggish, AL, Picard, MH, Hoffmann, U, Hung, J. “Multimodality imaging of pericardial diseases”. . vol. 3. 2010. pp. 650-660.

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