74. Constrictive Pericarditis- Indications and Guidelines

Navyatha Mohan MD MPH, Kenneth K. Liao MD PhD
Baylor College of Medicine
August 2024

Abbreviations & Definitions

CP – Constrictive pericarditis
CPB – Cardiopulmonary bypass
ESC – European Society of Cardiology
RAP – Right atrial pressure

Indications & Guidelines for Management by Grade/Stage of Disease

The management of constrictive pericarditis (CP) is largely empirical because of the general lack of randomized controlled trials regarding pericardial diseases. The guidelines for management of CP described in this chapter are largely adopted from the 2015 European Society of Cardiology (ESC) Guidelines for the management of pericardial disease.1 There are no recent American College of Cardiology/American Heart Association guidelines on this topic.

CP is considered to exist along a spectrum of ‘pericardial constrictive syndromes.’2 There are three main CP syndromes, namely:

  • Transient CP
  • Effusive CP
  • Chronic CP

Transient CP

This is a reversible pattern of constriction. Some patients may go through a transient phase of cardiac constriction at the end of the effusive period of acute idiopathic pericarditis. The clinical course implies the presence of acute inflammatory pericarditis with constriction due to inflammation. Once the inflammatory process is treated, the constriction resolves.3,4 It is important to make this distinction because medical therapy (generally 2-3 months of empiric anti-inflammatory medical therapy) may resolve transient constriction.

In the absence of evidence that the condition is chronic (e.g., cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification), patients with newly diagnosed CP who are hemodynamically stable may be given a trial of conservative management for 2–3 months before recommending pericardiectomy.1,2 If transient CP is accompanied by a heart failure presentation, then there is a low threshold to escalate to triple therapy, including NSAIDs, colchicine, and steroids, for better clinical response and to avoid delays in case there is a need for pericardiectomy.1 IL-1 inhibitors (e.g., rilonacept or anakinra) can be used as a viable alternative to corticosteroids or as third-line therapy for patients.5

Effusive CP

Patients with effusive CP usually have clinical features of pericardial effusion, CP, or both. The pericardial cavity is typically obliterated in patients with CP. Thus, even the normal amount of pericardial fluid is absent. However, pericardial effusion may be present in some cases. In this setting, the scarred pericardium not only constricts the cardiac volume, but can also put pericardial fluid under increased pressure, leading to signs suggestive of cardiac tamponade. This combination is called effusive CP.1,2

Unexpected persistence of the V wave of right atrial pressure (RAP) is a clue to the possibility of effusive CP that may be present before pericardiocentesis. After pericardiocentesis, despite lowering of the pericardial pressure to near zero, persistence of elevated RAP suggests the presence of effusive CP. The diagnosis has been defined by failure of the RAP to fall by 50% or to a level <10 mmHg after pericardiocentesis.6,7

Since it is the visceral layer of the pericardium and not the parietal layer that constricts the heart, visceral pericardiectomy must be performed. However, the visceral component of the pericardiectomy is often difficult, requiring sharp dissection of many small fragments until an improvement in ventricular motion is observed. Pericardiectomy for effusive CP should be performed only at centers with experience in pericardiectomy for CP.1,2,6

Chronic CP

Pericardiectomy is the accepted standard of treatment in patients with chronic CP who have persistent and prominent symptoms, such as NYHA class III or IV heart failure. Surgical removal of the pericardium has a significant operative mortality ranging from 6 to 12%. Pericardiectomy must be as complete as is technically feasible and should be performed by experienced surgeons. Referral to a center with a special interest in pericardial disease may be warranted in centers with limited experience in this surgery.5

Patients with ‘end-stage’ CP derive little or no benefit from pericardiectomy, and the operative risk is inordinately high. Manifestations of end-stage disease include cachexia, atrial fibrillation, a low cardiac output (cardiac index <1.2 L/m2/min) at rest, hypoalbuminemia due to protein-losing enteropathy, and/or impaired hepatic function due to chronic congestion or cardiogenic cirrhosis.1

Surgical Treatment

Anterior pericardiectomy is limited to the removal of the pericardium between the left and right phrenic nerves. This improves hemodynamics in many patients, but this limited procedure may be inadequate in others.8 Accordingly, other authors have advocated radical pericardiectomy, excising the pericardium posterior to the phrenic nerves on the left and that between the inferior walls of the left and right ventricles and diaphragm to decorticate the atria and ventricles and prevent recurrent constriction.9,10,11

Median sternotomy is the preferred approach. The decortications should remove as much of the pericardium as possible with all constricting parietal and epicardial layers, taking care to preserve phrenic nerves bilaterally. After completing a “phrenic to phrenic” or anterior pericardiectomy, additional dissection is then done between the pericardium and the posterolateral left ventricular wall and along the inferior walls of the left and right ventricles. Laterally, the pericardium posterior to the left phrenic nerve down to the left-sided pulmonary veins is dissected. In addition, the diaphragmatic pericardium is dissected off the inferior left and right ventricular walls.11 When the constricting peel is adherent or calcified, it may be necessary to leave behind a few islands of pericardium, or to perform cross-hatched relaxing incisions on the pericardium to better relieve constriction.

The ESC guidelines recommend that cardiopulmonary bypass (CPB) be used only in cases of co-existent cardiac surgical lesions to minimize bleeding with full heparinization, with CPB available for rescue in case of hemorrhagic lesions during the operation. However, other authors believe CPB alone does not increase procedural risk or negatively affect survival, but instead aids in the avoidance of accidental cardiac injury, facilitates maintenance of end-organ perfusion during the dissection of the lateral left ventricle and diaphragmatic surface, and allows for more controlled complete resection.12 Our institutional experience favors the use of CPB to achieve complete resection.

Summary Table.

Syndrome Clinical Presentation Management
Transient Temporary and reversible form of constriction with pericarditis and mild effusion Anti-inflammatory therapy First line – NSAIDS, colchicine Second line – Steroids Third line – IL-1 inhibitors (rilonacept or anakinra)
Effusive Pericardial effusion/tamponade along with pericarditis. Persistently high RAP despite normalization of intrapericardial pressure after drainage of pericardial effusion Pericardiocentesis for the effusion, followed by aggressive anti-inflammatory therapy. Pericardiectomy when refractory to medical therapy
Chronic Generally irreversible with signs and symptoms of right-sided heart failure Surgical treatment with radical pericardiectomy

Supporting Evidence for Current Indications & Guidelines

2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC).
Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS).

Ongoing Trials/Recent Publications

At this time, the authors are not aware of any ongoing trials on this topic.

Expert Commentary

We advocate performing pericardiectomy during the phase of effusive CP and early phase of chronic CP, rather than waiting for the late stage of chronic CP when cachexia, low cardiac output (cardiac index <1.2 L/m2/min) not responding to inotropes, hypoalbuminemia due to protein-losing enteropathy, and/or cardiogenic cirrhosis occur and surgical risk is exceedingly high. When performing pericardiectomy, we advocate radical pericardiectomy and apply CPB early to facilitate complete pericardium dissection and safe resection. When the constricting epicardial layer is encountered, we will arrest the heart to ensure complete and safe resection.

Sources

  1. Adler, Y., Charron, P., Imazio, M., et al. (2015). 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC). European Heart Journal, 36(42), 2921–2964.
  2. Imazio, M., Brucato, A., Mayosi, B. M., et al. (2010). Medical therapy of pericardial diseases: Part II: Noninfectious pericarditis, pericardial effusion and constrictive pericarditis. Journal of Cardiovascular Medicine, 11(11), 785–794.
  3. Sagrista-Sauleda, J., Permanyer-Miralda, G., Candell-Riera, J., et al. (1987). Transient cardiac constriction: An unrecognized pattern of evolution in effusive acute idiopathic pericarditis. American Journal of Cardiology, 59(9), 961–966.
  4. Haley, J. H., Tajik, A. J., Danielson, G. K., et al. (2004). Transient constrictive pericarditis: Causes and natural history. Journal of the American College of Cardiology, 43(2), 271–275.
  5. Kumar, S., Khubber, S., Reyaldeen, R., et al. (2022). Advances in imaging and targeted therapies for recurrent pericarditis: A review. JAMA Cardiology, 7(9), 975–985.
  6. Sagrista-Sauleda, J., Angel, J., Sánchez, A., et al. (2004). Effusive-constrictive pericarditis. New England Journal of Medicine, 350(5), 469–475.
  7. Hancock, E. W. (2004). A clearer view of effusive-constrictive pericarditis. New England Journal of Medicine, 350(5), 435–437.
  8. Hemmati, P., Greason, K. L., & Schaff, H. V. (2017). Contemporary techniques of pericardiectomy for pericardial disease. Cardiology Clinics, 35(4), 559–566.
  9. Chowdhury, U. K., Subramaniam, G. K., Kumar, A. S., et al. (2006). Pericardiectomy for constrictive pericarditis: A clinical, echocardiographic, and hemodynamic evaluation of two surgical techniques. Annals of Thoracic Surgery, 81(2), 522–529.
  10. Cho, Y. H., Schaff, H. V., Dearani, J. A., et al. (2012). Completion pericardiectomy for recurrent constrictive pericarditis: Importance of timing of recurrence on late clinical outcome of operation. Annals of Thoracic Surgery, 93(4), 1236–1241.
  11. Villavicencio, M. A., Dearani, J. A., & Sundt, T. M. (2008). Pericardiectomy for constrictive or recurrent inflammatory pericarditis. Operative Techniques in Thoracic and Cardiovascular Surgery, 13(1), 2–13.
  12. Unai, S., & Johnston, D. R. (2019). Radical pericardiectomy for pericardial diseases. Current Cardiology Reports, 21(6), 1–9.
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