Postpericardiotomy Syndrome – Causes, Symptoms and Treatment

Postpericardiotomy syndrome (PPS) occurs in a subgroup of patients who have undergone cardiothoracic surgery and is characterized by fever, pleuritic pain, pleural effusion, and pericardial effusion. It occurs due to immune-mediated inflammation of the pleura and pericardium.

Postpericardiotomy syndrome after is known to occur following myocardial infarction too and it is called Dressler syndrome.

Both fall in the spectrum of post-cardiac injury syndrome, a group of inflammatory pericardial syndromes that include post-myocardial infarction Dressler syndrome, postpericardiotomy syndrome and post-cardiac trauma.

Post-pericardiotomy syndrome is also reported to occur after percutaneous procedures such as coronary stenting, implantation of pacemaker leads and transvenous pacemaker leads.

It can also occur after blunt trauma, stab or puncture of heart.

Postpericardiotomy syndrome  occurs in about 15-30% of patients undergoing cardiac surgery

It can occur in children and adults both but is not common below the age of 2 years.

Common procedures known to be associated are repair of tetralogy of Fallot, repair of atrial and ventricular septal defects, and after cardiac transplantation. 

Occasional recurrence after years has been reported.

The syndrome was first described in 1953 after mitral valve surgery

Criteria of Postpericardiotomy Syndrome

Postpericardiotomy syndrome is said to occur when 2 of the following 5  are present

• Fever occurring at least one week after surgery without underlying infection
• Pleuritic pain
• Pericardial rub
• Pleural effusion
• Pericardial effusion

A modified definition has also been proposed to minimize false-positives.

Here the postpericardiotomy syndrome is diagnosed in the presence of 2 of the following

• Pericardial rubbing
• Fever >72 hours postoperative
• Pleuritic chest pain
• New significant pleural effusion on chest radiograph (above the highest level of the diaphragm)
• Significant pericardial effusion (10 mm).

Cause and Pathophysiology

The precise etiology of postpericardiotomy syndrome is not known. Postpericardiotomy syndrome is postulated to be an autoimmune response involving autoantibodies which target antigens exposed after the pericardium has been damaged. 

There is formation of antiheart antibodies which lead to an inflammatory reaction within the pericardial space.

An autoimmune reaction developing after viral infection has been proposed but no evidence of any virus has yet been found.

There could be multiple factors responsible. The inflammatory response,  bleeding during surgery and coagulation have been suggested to play a role.

Following factors have been found to be associated with higher risk are

• Preoperative low values of

• • Hemoglobin and platelets
  • Interleukin 8 [IL8]

• Postoperative high levels of complement conversion products
• Younger age
• RBC transfusions during surgery

Clinical Presentation

The patient will have fever [rarely beyond 40 degres], usually 7-42 days after the heart surgery that involved pericardiotomy.

The patient looks alright and not unwell despite the high fever. The fever subsides in 15-20 days.

Other symptoms that could be present are

• Malaise
• Chest pain
• Irritability
• Decreased appetite
• Dyspnea
• Joint pains

A chest pain that worsens with inspiration and supine position may be seen in children. This pain improves when the child sits and leans forward.

Vomiting is the main symptom in children with impending cardiac tamponade in this syndrome.

On examination, there is tachycardia and a pericardial friction rub [may disappear with further fluid accumulation].

• Hepatomegaly and fluid retention in other organs
  • Decreased cardiac output due to decreased ventricular filling.
• Pulses paradoxicus[a decrease in systemic blood pressure over 10 mmHg during inspiration and diminished pulse-wave amplitude by palpation of the radial artery] may occur.
• Pleural friction rubs
• Signs of pneumonitis
  • Cough
  • Decreased oxygen saturation.

Differential Diagnoses

• Constrictive Pericarditis
• Congestive Heart Failure
• Pediatric Infective Pericarditis
• Pediatric Nonviral Myocarditis
• Chylous Pericardial/pleural Effusion

Lab Studies

Routine Tests

• CBC
  • Leukocytosis
• Blood culture for infection
• Raised CRP and ESR

Antiheart antibodies would be positive. However, this test is often not done.

Cardiac enzyme testing is not usually helpful because the results vary

Pericardial drain fluid biochemistry and cytology to know about exudative or transudative nature.

The culture of the drained fluid would rule out infection. out infection/inflammation

ECG

• Global ST segment elevation and T-wave inversion initially
  • [suggestive of pericarditis or subepicardial injury or myocardial inflammation.
• Low QRS amplitude
  • with a large pericardial effusion.

Chest X-ray

Blunting of costophrenic angles due to a pleural effusion may be seen.

Enlargement of cardiac silhouette the cardiac silhouette

Echocardiography

It is the diagnostic procedure of choice.

Earlies fluid detection is posterior to the left ventricle during systole. With further accumulation, detection is easier.

Echocardiography is able to distinguish suspected postpericardiotomy syndrome from congestive heart failure and is helpful in ventricular contractility evaluation.

Cardiac Magnetic Resonance Imaging

• Evaluates cardiac dynamics and pericardial abnormalities
• Can demonstrate pericardial thickening and inflammation a
• Helpful in identifying loculated posterior pericardial fluid collections not visible on echo.

Treatment

Usually, the patient is treated with non-operative treatment and includes the administration of NSAIDs such as aspirin.

Antiinflammatory drugs are given for 4-6 weeks and are tapered as the fluid decreases.

Ibuprofen or naproxen may be given to patients not responding to aspirin.

Corticosteroids can be given in refractory cases.

Most of the cases of the postpericardiotomy syndrome are managed on OPD basis.

Sever cases may require indoor care.

Patients cardiac tamponade may need drainage of the fluid by pericardiocentesis.

The patients should not carry strenouds activieis and preferably have bed rest till symptoms regress and ECG is almost normal.

Cardiac tamponade is a syndrome caused when the substantial fluid in the pericardial space results in decreased ventricular filling and subsequent hemodynamic compromise.

It is a life-threatening condition that includes pulmonary edema, shock, and death.

Pericardiocentesis is the emergency procedure required for drainage of tamponade.  Pericardiocentesis preferably under echocardiographic guidance is used for removal of the fluid.

In the case of recurrent relapses thoracotomy [open or video assisted] to create a percasrdial window may be considered.

Percutaneous balloon pericardiotomy is a less invasive procedure in which a pericardial window is created using a balloon catheter under fluoroscopic guidance. The fluid drains  into the abdominal cavity via a perforation of the diaphragm.  This could be an alternative procedure.

Complications

• Cardiac tamponade
• Constrictive pericarditis

In case of coronary artery bypass grafting in children, occlusion of the graft is reported as unusual and fatal complication.

Prognosis

Most cases of postpericardiotomy syndrome resolve within a few weeks. Rarely, symptoms may occur for more than 6 months.

Relapse may occur after tapering anti-inflammatory medications; it is estimated to occur in 10-15% of patients. Most recurrences occur within 6 months of the initial insult.

Postpericardiotomy syndrome is usually mild. Cardiac tamponade occurs in less than 1% of the patients.

Presence of Mediterranean Fever gene may reduce  the severity of the postpericardiotomy syndrome.

References

• 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.
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• Wendelin G, Fandl A, Beitzke A. High-dose intravenous immunoglobulin in recurrent postpericardiotomy syndrome. Pediatr Cardiol. 2008 Mar. 29(2):463-4
• Ziskind AA, Pearce AC, Lemmon CC, et al. Percutaneous balloon pericardiotomy for the treatment of cardiac tamponade and large pericardial effusions: description of technique and report of the first 50 cases. J Am Coll Cardiol. 1993 Jan. 21(1):1-5.
• 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
• Takata M, Robotham JL. Pericardial effusion and tamponade. Critical Heart Disease in Infants, Children, and Adolescents. 1995. 255-71.
• Tsang TS, Barnes ME, Hayes SN, et al. Clinical and echocardiographic characteristics of significant pericardial effusions following cardiothoracic surgery and outcomes of echo-guided pericardiocentesis for management: Mayo Clinic experience, 1979-1998. Chest. 1999 Aug. 116(2):322-31.
• Johnston DR. Surgical management of pericardial diseases. Prog Cardiovasc Dis. 2017 Jan – Feb. 59(4):407-16.