AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Postpericardiotomy syndrome (PPS) is a febrile illness in patients who have undergone surgery that involves opening the pericardium. The syndrome is also characterized by pericardial or pleuritic pain, friction rubs, pericardial effusions, pleural effusions, pneumonitis, and abnormal ECG and radiography findings (see Workup).

PPS has also been described following myocardial infarction (Dressler syndrome) and as an unusual complication after percutaneous procedures such as coronary stent implantation,¹ after implantation of epicardial pacemaker leads² and transvenous pacemaker leads,^{3, 4, 5, 6, 7} following blunt trauma,⁸ stab wounds,⁹ and heart puncture.¹⁰

Pathophysiology

PPS is characterized by a febrile illness with an inflammatory reaction that typically involves the pleura and pericardium. Effusions often accompany the syndrome and may develop into early or late postoperative cardiac tamponade¹¹ and even recurrent cardiac tamponade.¹²

PPS is often associated with the development of antiheart antibodies.¹³ Various viral agents, including coxsackie B, adenovirus, and cytomegalovirus, have been present in approximately two thirds of patients with PPS, suggesting an autoimmune response associated with a viral infection. However, a prospective study found no evidence to support a viral etiology for PPS.¹⁴ This study suggested that the use of viral titers in the setting of cardiopulmonary bypass and recent blood transfusions is unreliable.

Frequency

United States

Estimated frequencies vary from 2-30% of patients undergoing surgery that involves opening of the pericardium.

Mortality/Morbidity

• PPS usually manifests as a mild, self-limited inflammatory illness.
• Life-threatening pericardial tamponade can develop due to a progressively increasing pericardial effusion.
• Tamponade occurs in fewer than 1% of patients with PPS. Elevation of cardiac filling pressures, progressive limitation of ventricular diastolic filling, and reduction of stroke volume and cardiac output characterize cardiac tamponade.

Age

PPS is uncommon in infants, but frequency increases in children and adults to as high as 30%.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

Symptoms usually develop within 1-6 weeks after surgery involving pericardiotomy. Temperature after the first postoperative week usually reaches 38-39°C orally but may spike as high as 40°C. Despite a high temperature, the patient may not appear ill. The fever usually subsides within 2-3 weeks. Malaise, chest pain, irritability, and decreased appetite are typical presenting symptoms. Patients may also report dyspnea and arthralgias. Children may report chest pain that worsens with inspiration and when in the supine position. Emesis has also been reported as the main symptom in 2 children with impending cardiac tamponade secondary to PPS.¹⁵

Physical

Patients often demonstrate tachycardia and a pericardial friction rub. The pericardial rub disappears either with improvement or with further accumulation of pericardial fluid. Systemic fluid retention and hepatomegaly can also occur. Pleural friction rubs are common. Signs of pneumonitis, including cough, fever, and decreased oxygen saturation, may also be present.

Causes

The precise etiology of postpericardiotomy syndrome (PPS) is not known. PPS is postulated to be an autoimmune response, perhaps triggered by a viral infection with viruses such as coxsackie B, adenovirus, or cytomegalovirus. However, more recent prospective evaluations have not found evidence to support a viral etiology.

A 4-fold or greater rise in antiheart antibodies is frequently found in PPS. The presence of antiheart antibodies in a high titer is a confirmatory diagnostic test.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Chylous pericardial effusion
Chylous pleural effusion

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• The expected CBC count findings include leukocytosis with a leftward shift.
• As with other patients with suspected inflammatory versus infectious conditions, obtain blood cultures early in the workup. The results of the blood cultures should be negative.
• Acute phase reactants, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, are elevated.
• Antiheart antibodies are usually present in high titers.
• Cardiac enzyme testing is not usually helpful because the results vary. In addition, studies have reported no difference in enzyme levels compared with patients who underwent cardiopulmonary bypass that do not have clinical signs of postpericardiotomy syndrome (PPS).
• If a pericardial drain is placed, fluid should be obtained for cell count, differential, cytology, culture, gram stain, triglyceride level, and total protein level.

Imaging Studies

• Chest radiography may be helpful in diagnosing PPS.
• • Chest radiography usually reveals blunting of the costophrenic angles due to a pleural effusion. A pericardial effusion enlarges the cardiac silhouette.
  • The cardiac silhouette enlarges in proportion to the amount of fluid contained in the pericardial sac.
• Echocardiography is the diagnostic standard. It is a much more sensitive imaging study than plain radiography.
• • In the early stages of PPS, a small amount of fluid may be detected posterior to the left ventricle during systole.
  • With increasing fluid accumulation, detection using echocardiography becomes easier.
  • Echocardiography assists in differentiating suspected PPS from congestive heart failure; cardiac output is reduced in both conditions. In PPS with a large effusion one or more cardiac chambers may be compressed by the pericardial fluid.
  • Echocardiography is particularly helpful in evaluating ventricular contractility.
• Cardiac MRI has been used more frequently to evaluate cardiac dynamics and pericardial abnormalities. Cardiac MRI may be more helpful in identifying posterior pericardial fluid collections that may have become loculated and are not easily viewed with trans-thoracic echocardiography.

Other Tests

ECG findings are abnormal in PPS and may include the following:

• Initial findings may simulate pericarditis, with global ST segment elevation and T-wave inversion.
• Subepicardial injury, resulting from myocardial inflammation, causes ST segment elevation.
• The ECG may also reveal low QRS amplitude, especially with a large pericardial effusion.

Procedures

Tamponade is a life-threatening condition that can result from PPS. The inflammatory changes seen in PPS may cause pericardial adhesions that result in a localized collection of pericardial fluid.

Pericardiocentesis may be emergently required if cardiac tamponade is present.

The standard subxiphoid approach is recommended. Because of the possible localized nature of the tamponade, echocardiographic guidance is recommended. Echocardiography-guided pericardiocentesis with extended catheter drainage is considered the primary management for patients with clinically significant pericardial effusions. The drainage tube is usually left in place for 24-48 hours, during which anti-inflammatory treatment is initiated.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

Evaluation of patients with suspected postpericardiotomy syndrome (PPS) is usually performed in an outpatient setting. The workup and treatment may continue on an outpatient basis if the patient is not hemodynamically affected. Medical management includes the use of nonsteroidal anti-inflammatory agents and corticosteroids. Pericardial drainage is indicated in patients with symptoms consistent with tamponade. Patients with tamponade must be admitted to the hospital for definitive care.

Anecdotally, successful treatment of recurrent pericardial effusion has been described using a single high dose of intravenous immunoglobulin in one patient¹⁶ and a low weekly dose of methotrexate in one other.¹⁷

Surgical Care

Immediate pericardiocentesis is necessary to relieve life-threatening cardiac tamponade.

A surgically created pericardial window may be necessary in patients with persistent symptoms or relapse after medical therapy. This may be achieved through an open thoracotomy^{18, 19, 20} or through a video-assisted thoracoscopic technique.²¹

Percutaneous balloon pericardiotomy (PBP) may be another alternative for these patients. This is a less invasive procedure in which a pericardial window is created in the catheterization laboratory using a balloon catheter under fluoroscopic guidance.^{22, 23, 24}

Consultations

Consult a pediatric cardiologist to diagnose and treat as well as to follow care of patients with PPS.

Consult a pediatric cardiothoracic surgeon in cases of patients with persistent symptoms or relapse after medical therapy. These patients may require a pericardial window.

Diet

Patients usually have decreased appetite; however, special dietary restrictions are usually not required in patients with PPS.

Activity

Patients with suspected or confirmed PPS should avoid strenuous activity. Bed rest alone may be adequate to treat mild cases. Enforce strict bed rest until the fever has resolved and chest radiography and ECG reveal near baseline findings.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

The mainstay of medical therapy is use of anti-inflammatory agents. Various drugs are available; all have similar efficacy. Corticosteroids are often used in more severe or refractory cases. Corticosteroids have resulted in rapid improvement in clinical symptoms and decrease in antiheart antibodies.

No evidence suggests that steroids administered prior to cardiopulmonary bypass reduce the risk of developing postpericardiotomy syndrome (PPS). One case has been reported of low-dose methotrexate used in PPS refractory to standard therapy;¹⁷ however, this has not been further supported.

Drug Category: Anti-inflammatory agents

These agents decrease inflammatory responses and interfere with systemic events leading to inflammation.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• Inpatient care of patients with postpericardiotomy syndrome (PPS) is indicated in more severe cases, such as patients with symptoms and signs indicative of tamponade.
• After drainage of the pericardial effusion and improvement in the clinical symptoms, most patients can be treated on an outpatient basis.

Further Outpatient Care

• Bed rest and otherwise restricted physical activity may be indicated. Limit activity at least until resolution of the acute symptoms and findings.

In/Out Patient Meds

• Outpatient medications can include aspirin, prednisone, or indomethacin.

Transfer

• Patients refractory to medical management require transfer to a facility that has a pediatric cardiothoracic surgeon available. These patients may require a surgical pericardial window.

Deterrence/Prevention

• Currently, no known preventative therapy is available for PPS.
• A randomized controlled trial evaluating the use of colchicine to prevent PPS in patients undergoing cardiopulmonary bypass did not find a statistically significant difference from placebo.²⁵ The authors noted a trend toward significance that may be more evident with larger study.

Complications

• Cardiac tamponade is a life-threatening complication of PPS. Emergent pericardiocentesis and drainage of pericardial effusion is necessary. Tamponade occurs in approximately 1% of patients with PPS.
• Constrictive pericarditis occurs late postoperatively in fewer than 0.5% of patients but may not be related to PPS. The high prevalence of PPS and quite low prevalence of constriction suggests that a direct association is unlikely. With constriction, the pericardium becomes thickened and adherent to the heart and restricts filling of the ventricles. A pericardiectomy may be required for treatment.
• Patients with pain from the inflammatory response may demonstrate splinting during breathing. This can result in hypoxemia. Monitor oxygen saturation by pulse oximetry in patients presenting with these findings.
• Coronary artery bypass grafting is an unusual procedure in children. Occlusion of the graft is reported as a rare, but fatal, complication of PPS.

Prognosis

• Most cases resolve within a few weeks. Rarely, symptoms may occur for more than 6 months.
• Relapse may occur after tapering anti-inflammatory medications. Relapse is estimated to occur in 10-15% of patients. Most recurrences occur within 6 months of the initial surgery.

Patient Education

• Instruct caregivers of children who have undergone cardiac surgery on warning signs of PPS. They should contact their physician if fevers, chest pain, fatigue, weight loss, or shortness of breath develop in these children.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Failure to diagnose or adequately treat postpericardiotomy syndrome (PPS)
• Failure to diagnose or treat impending or manifest cardiac tamponade

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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Article Last Updated: May 29, 2008