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Postpericardiotomy Syndrome Last Updated: August 18, 2005 |
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| Synonyms and related keywords: postcardiac injury syndrome, PPS, cardiac tamponade, pericardium surgery
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AUTHOR INFORMATION
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| Author: Kelly Tieves, DO, MS, FAAP, Assistant Professor, Department of Pediatrics, Section of Critical Care, Medical College of Wisconsin Coauthor(s): John Graneto, DO, FACEP, FAAP, Clinical Assistant Professor of Emergency Medicine, Chicago College of Osteopathic Medicine of Midwestern University, Medical Staff, Department of Emergency Medicine, Swedish Covenant Hospital; David A Lewis, MD, FAAP, FACC †, Former Director of Medical Education, Former Pediatric Program Director, Former Associate Professor, Department of Pediatric Cardiology, Medical College of Wisconsin |
| Kelly Tieves, DO, MS, FAAP, is a member of the following medical societies:
American Academy of Pediatrics, and
Society of Critical Care Medicine |
| Editor(s): Ira H Gessner, MD, Professor, Department of Pediatrics, University of Florida College of Medicine; Robert Konop, PharmD, Director, Clinical Account Management, Ancillary Care Management, Inc;
Hugh D Allen, MD, Professor, Departments of Pediatrics and Medicine, Ohio State University College of Medicine and Public Health;
Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College;
and Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin |
Disclosure
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INTRODUCTION
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Background: Postpericardiotomy syndrome (PPS) is a febrile illness in patients who have undergone surgery that involves opening the pericardium. The syndrome was first described in 1952 after surgical repair of mitral stenosis. Pathophysiology: A febrile illness with an inflammatory reaction that typically involves the pleura and pericardium characterizes the syndrome. Effusions often accompany the syndrome.
PPS is often associated with the development of antiheart antibodies. Various viral agents, including Coxsackie B, adenovirus, and cytomegalovirus, have been present in approximately two thirds of patients with postpericardiotomy syndrome, suggesting an autoimmune response associated with a viral infection. However, a prospective study by Webber et al in 2001 found no evidence to support a viral etiology for PPS. The Webber study suggested that the use of viral titers in the setting of cardiopulmonary bypass and recent blood transfusions is unreliable. Frequency:
- In the US: PPS is uncommon in infants but occurs with increasing frequency in children and adults. Estimated frequencies vary from 2-30% of patients undergoing surgery involving opening of the pericardium.
Mortality/Morbidity:
- Postpericardiotomy syndrome 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: Postpericardiotomy syndrome is uncommon in infants, but frequency increases in children and adults to as high as 30%.
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CLINICAL
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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 worsening with inspiration and in the supine position. 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. Causes: The precise etiology of postpericardiotomy syndrome is not known. The most common hypothesis is an autoimmune response, perhaps triggered by a viral infection. Viruses implicated include Coxsackie B, adenovirus, and 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 associated. The presence of antiheart antibodies in a high titer is a confirmatory diagnostic test.
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DIFFERENTIALS
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Endocarditis, Bacterial Heart Failure, Congestive Myocarditis, Nonviral Myocarditis, Viral Pericarditis, Constrictive Pericarditis, Viral Postpericardiotomy Syndrome
Other Problems to be Considered:
Chylous pericardial effusions
Chylous pleural effusion |
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Patient Education
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Click here for patient education.
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WORKUP
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Lab Studies:
- Complete blood cell count: The expected findings are 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 an erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, show an elevation.
- Antiheart antibodies are usually present in a high titer.
- Cardiac enzyme testing is usually not helpful because the results vary; also, studies have reported no difference in enzyme levels compared with patients who underwent cardiopulmonary bypass that do not have clinical signs of PPS.
- If a pericardial drain is placed, the fluid should be sent for cell count, differential, cytology, culture, gram stain, triglycerides, and total protein.
Imaging Studies:
- Chest radiography may be helpful in diagnosing PPS.
- The chest radiograph 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 by means of echocardiography becomes easier.
- Specific echocardiographic criteria indicate impending or established cardiac tamponade.
- Echocardiography assists in differentiating suspected PPS from congestive heart failure. Echocardiography is particularly helpful in evaluating ventricular contractility.
- Cardiac magnetic resonance imaging has been used more frequently to evaluate cardiac dynamics and pericardial abnormalities.
- Cardiac magnetic resonance imaging may be more helpful in identifying posterior pericardial fluid collections that may have become loculated and not easily viewed with trans-thoracic echocardiography.
Other Tests:
- Electrocardiography (ECG) produces abnormal findings in PPS. 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 demonstrate 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 with PPS may cause pericardial adhesions resulting in a localized tamponade.
- Pericardiocentesis may be required emergently if cardiac tamponade is present.
- The standard subxiphoid approach is recommended. Because of the possible localized nature of the tamponade, using echocardiographic guidance is recommended. Echo-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.
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TREATMENT
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Medical Care: Evaluation of patients with suspected 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. Patients with symptoms indicating tamponade must be admitted to hospital for definitive care. Surgical Care: Immediate pericardiocentesis is necessary to relieve life-threatening cardiac tamponade. A surgically placed pericardial window may be necessary in patients with persistent symptoms or relapse after medical therapy. Involvement of a cardiothoracic surgeon is indicated in patients with symptoms refractory to medical management. 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.
- 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 represent near baseline studies.
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MEDICATION
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The mainstay of medical therapy is use of anti-inflammatory agents. A variety of drugs are available, all having 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 exists that pre-cardiopulmonary bypass steroids reduce the risk of developing PPS. One case has been reported of low-dose methotrexate used in PPS refractory to standard therapy but has not been further supported.
Drug Category: Anti-inflammatory agents -- These agents decrease inflammatory responses and interfere with systemic events leading to inflammation. Drug Name
| Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin) -- First-line medication for patients with PPS. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. |
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| Adult Dose | 650 mg PO q4h |
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| Pediatric Dose | 80-120 mg/kg/d PO divided q6h |
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| Contraindications | Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; because of association of aspirin with Reye syndrome, do not use in children (<16 y) with flu or varicella |
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| Interactions | Possible decreased effects with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose lowering effect of sulfonylurea drugs |
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| Pregnancy |
C - Safety for use during pregnancy has not been established.
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| Precautions | Category D in third trimester of pregnancy; may cause transient decrease in renal function and aggravate chronic kidney disease; avoid use with severe anemia, with history of blood coagulation defects, or with anticoagulants |
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Drug Name
| Indomethacin (Indocin) -- Nonsteroidal anti-inflammatory medication often used as a first-line drug in PPS. Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis. |
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| Adult Dose | 25-50 mg PO bid/tid |
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| Pediatric Dose | 1-3 mg/kg/d PO divided q6-8h; not to exceed 200 mg/d |
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| Contraindications | Documented hypersensitivity; GI bleeding or renal insufficiency |
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| Interactions | Increased risk of serious NSAID-related adverse effects when coadministered with aspirin; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
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| Pregnancy |
B - Usually safe but benefits must outweigh the risks.
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| Precautions | Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur (discontinue if there is persistent leukopenia, granulocytopenia, or thrombocytopenia) |
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Drug Name
| Prednisone (Deltasone, Orasone, Sterapred) -- May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Usually reserved for treating more severe cases or relapses. Also may be used as a first-line drug. Corticosteroid use has been shown to result in faster resolution of symptoms than other therapies. |
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| Adult Dose | 20-60 mg/d PO |
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| Pediatric Dose | Week 1: 2 mg/kg/d PO; tapered over 2-4 wk
Week 2: 1 mg/kg/d PO
Week 3: 0.5 mg/kg/d PO| Contraindications | Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy |
B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur |
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FOLLOW-UP
| Section 8 of 10  |
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Further Inpatient Care:
- Inpatient care of patients with 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 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 know preventative therapy for PPS exists.
- A randomized controlled trial evaluating the use of colchine to prevent PPS in patients undergoing cardiopulmonary bypass did not find a statistically significant difference from placebo. 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.
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MISCELLANEOUS
| Section 9 of 10  |
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Medical/Legal Pitfalls:
- Failure to diagnose or adequately treat PPS
- Failure to diagnose or treat impending or manifest cardiac tamponade
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BIBLIOGRAPHY
| Section 10 of 10 |
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Bartels C, Honig R, Burger G, et al: The significance of anticardiolipin antibodies and anti-heart muscle antibodies for the diagnosis of postpericardiotomy syndrome. Eur Heart J 1994 Nov; 15(11): 1494-9[Medline].
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Cabalka AK, Rosenblatt HM, Towbin JA, et al: Postpericardiotomy syndrome in pediatric heart transplant recipients. Immunologic characteristics. Tex Heart Inst J 1995; 22(2): 170-6[Medline].
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Cheung EW, Ho SA, Tang KK, et al: Pericardial effusion after open heart surgery for congenital heart disease. Heart 2003 Jul; 89(7): 780-3[Medline].
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De Biase L., Di Renzi, P., et al: Cardiac magnetic resonance imaging diagnosis of a loculated pericardial effusion in a patient with a postpericardiotomy syndrome. talian Heart Journal: Official Journal of the Italian Federation of Cardiology 2002; 3(7): 435-6.
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Engle MA, Zabriskie JB, Senterfit LB, et al: Viral illness and the postpericardiotomy syndrome. A prospective study in children. Circulation 1980 Dec; 62(6): 1151-8[Medline].
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Finkelstein Y, Shemesh J, et al: Colchicine for the prevention of postpericardiotomy syndrome. Herz 2002; 27 (8): 791-4.
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Hoffman JIE, Stanger P: Diseases of Pericardium. Rudolph's Pediatrics; 1996: 1530-31.
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Horneffer PJ, Miller RH, Pearson TA, et al: The effective treatment of postpericardiotomy syndrome after cardiac operations. A randomized placebo-controlled trial. J Thorac Cardiovasc Surg 1990 Aug; 100(2): 292-6[Medline].
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Kim BJ, Ma JS: Constrictive pericarditis after surgical closure of atrial septal defect in a child. J Korean Med Sci 1998 Dec; 13(6): 658-61[Medline].
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Kronick-Mest C: Postpericardiotomy syndrome: etiology, manifestations, and interventions. Heart Lung 1989 Mar; 18(2): 192-8[Medline].
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Prabhu AS, Ross RD, Heinert MR, et al: Decreased incidence of postoperative pericardial effusions after cardiac surgery for congenital heart disease. Am J Cardiol 1996 Apr 1; 77(9): 774-6[Medline].
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Prince SE, Cunha BA: Postpericardiotomy syndrome. Heart Lung 1997 Mar-Apr; 26(2): 165-8[Medline].
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Takata M, Robotham JL: Pericardial Effusion and Tamponade. In: Critical Heart Disease in Infants, Children, and Adolescents. 1995: 255-71.
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Tsang TS, El-Najdawi EK, Hagler DJ: Percutaneous echocardiographically guided pericardiocentesis in pediatric patients: evaluation of safety and efficacy. Journal of American Society of Echocardiogram 1998; 11: 1072-7.
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Tsang TSM, Barnes ME, Hayes SN: Clinical and Echocardiographic Characteristics of Significant Pericardial Effusions Following Cardiothoracic Surgery and Outcomes of Echo-Guided Pericardiocentesis for Management. Chest 1999; 116(2): 322-31[Full Text].
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Webber SA, Wilson NJ, et al: Postpericardiotomy syndrome: no evidence for a viral etiology. Cardiology in the Young 2001; 11 (1): 67-74.
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Wilson NJ, Webber SA, Patterson MW, et al: Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome. Pediatr Cardiol 1994 Mar-Apr; 15(2): 62-5[Medline].
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Zucker N, Levitas A, et al: Methotrexate in recurrent postpericardiotomy syndrome. Cardiology in the Young 2003; 13(2): 206-8.
Postpericardiotomy Syndrome excerpt |