Guideline sources The following summarized guidelines for the evaluation and management of constrictive pericarditis are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2021) and the European Society of Cardiology (ESC/EACTS 2015). 1 2 3 3 3 3 Definition Constrictive pericarditis is a chronic disease characterized by symptoms of fluid overload, progressive reduction in cardiac output, and congestive HF. 3 Epidemiology Constrictive pericarditis is caused by fibrous thickening of the pericardium due to idiopathic reasons, prior cardiac surgery, postradiotherapy, postinfection (tuberculosis and non-tuberculous bacterial infection), connective tissue disease, malignancy, uremia, and sarcoidosis. 3 Disease course Chronic fibrous thickening of the pericardium due to various infectious and non-infectious causes result in constrictive pericarditis, which causes clinical manifestations of dyspnea, peripheral edema, chest pain, palpitation, abdominal distension, fever, fatigue, weight loss, jugular venous distension, pulsus paradoxus, hepatosplenomegaly, ascites, Kussmaul's sign, and pericardial shock. 3 https://web.pathway.md/diseases/recxOitjAGfrr6Mcv 1/3 6/23/23, 3:35 AM Constrictive pericarditis Pathway Prognosis and risk of recurrence In-hospital mortality associated with constrictive pericarditis is 2.1%. 3 Guidelines 1. Diagnostic investigations Initial evaluation: evaluate all patients with suspected pericardial disease using auscultation, ECG, TTE, CXR and routine blood tests, including markers of inflammation such as CRP and/or ESR, WBC count with differential, renal function, liver tests, and cardiac biomarkers. B Transthoracic echocardiography: obtain TTE in all patients with suspected constrictive pericarditis. B Chest X-ray: obtain lateral and frontal CXRs in all patients with suspected constrictive pericarditis. B Cardiac MRI/CT: Obtain cardiac CT and/or cardiac MRI to assess calcifications (assessed on CT), pericardial thickness, degree and extension of pericardial involvement. B Perform CT and/or cardiac MRI as second-line imaging techniques to assess for calcifications, pericardial thickness, and degree and extension of pericardial involvement. B 2. Diagnostic procedures Diagnostic pericardiocentesis: perform diagnostic pericardiocentesis with cytological analyses of pericardial fluid to confirm malignant pericardial disease. B Cardiac catheterization: As per ESC 2021 guidelines, consider performing right heart catheterization in patients where HF is thought to be due to constrictive pericarditis, restrictive cardiomyopathy, congenital heart disease, and high output states. C As per ESC 2015 guidelines, perform cardiac catheterization when noninvasive diagnostic methods fail to confirm a definite diagnosis of constriction. B 3. Medical management Empiric antibacterial therapy: initiate intravenous antibiotic therapy in patients with purulent pericarditis. B Empiric anti-inflammatory therapy: consider empiric anti-inflammatory therapy in patients with transient or new diagnosis of constrictive pericarditis in whom there is concomitant evidence of pericardial inflammation, such as an elevated CRP or pericardial enhancement on cardiac CT or cardiac MRI. C https://web.pathway.md/diseases/recxOitjAGfrr6Mcv 2/3 6/23/23, 3:35 AM Constrictive pericarditis Pathway Empiric anti-tuberculous therapy: Initiate empiric antituberculosis therapy in patients with exudative pericardial effusion living in endemic areas, after excluding other causes. B Avoid antituberculosis treatment prior to diagnostic confirmation in patients living in non-endemic areas. D 4. Therapeutic procedures Intrapericardial urokinase: consider administering intrapericardial urokinase to reduce the risk of constriction in tuberculous effusive pericarditis. C 5. Surgical interventions Indications for pericardiectomy: refer patients with chronic, permanent constriction for pericardiectomy when clinically appropriate. B 6. Specific circumstances Patients with tuberculous pericarditis: administer standard antituberculosis drugs for 6 months for the prevention of tuberculous pericardial constriction. B Show 3 more Patients with purulent pericarditis: initiate intravenous antibiotic therapy in patients with purulent pericarditis. B Show 2 more Patients with radiation-induced constrictive pericarditis: consider performing pericardiectomy in patients with radiation-induced constrictive pericarditis, acknowledging that outcomes may be worse than when performed for constrictive pericarditis of other causes. C References 1. Adler Y, Charron P, Imazio M et al. 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 . Eur Heart J. 2015 Nov 7;36 42 2921 2964. Open 2. Theresa A McDonagh, Marco Metra, Marianna Adamo et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021 Sep 21;42 36 3599 3726. Open 3. Murat Bi er, B lent zdemir, ris Kan et al. Long-term outcomes of pericardiectomy for constrictive pericarditis. J Cardiothorac Surg. 2015; 10 177. Open https://web.pathway.md/diseases/recxOitjAGfrr6Mcv 3/3