Constrictive pericarditis in the twenty-first century: An old disease revisited

Abstract

Constrictive pericarditis (CP) has been recognized for centuries and is an uncommon cause of heart failure with preserved ejection fraction. In developing countries, tuberculosis is the primary cause of CP, whereas in developed countries, most cases are idiopathic. In CP, the pericardium becomes thickened, fibrotic, and sometimes calcified, effectively “choking” the heart by impairing diastolic relaxation while preserving systolic contraction. As a result, right-sided heart failure develops and progressively worsens. Clinical diagnosis is often delayed because the disease shares features with more common conditions, including other causes of heart failure and non-cardiac disorders such as chronic liver disease. The triad of elevated jugular venous pressure, a diastolic apical impulse, and a pericardial knock should raise suspicion for CP. Chest radiography typically demonstrates a normal-sized cardiac silhouette and, particularly in advanced cases, curvilinear “egg-shell” pericardial calcification. In recent decades, non-invasive multimodality imaging has largely replaced invasive cardiac catheterization as the gold standard for the diagnosis of CP. In clinical practice, transthoracic echocardiography is the preferred initial imaging modality. The Mayo Clinic echocardiographic criteria help differentiate CP from restrictive cardiomyopathy. Cardiac magnetic resonance provides high-resolution anatomical imaging together with advanced tissue characterization and dynamic hemodynamic assessment. Cardiac computed tomography is the preferred modality for detecting and characterizing pericardial calcification. For clinical management, CP can be classified into inflammatory and non-inflammatory phenotypes based on inflammatory marker levels (e.g., C-reactive protein), the presence of pericardial edema and late gadolinium enhancement on cardiac magnetic resonance imaging, and evidence of pericardial inflammation on 18F-fluoro-2-deoxy-2-D-glucose positron emission tomography. Since the beginning of the 21^st century, the management of CP has shifted from a “one-size-fits-all” approach toward a more personalized, evidence-based strategy. Anti-inflammatory therapy, including colchicine and corticosteroids as first-line agents and biologic therapies such as anakinra and rilonacept as second-line agents, may be beneficial for patients with inflammatory CP. For refractory cases, patients with the non-inflammatory phenotype, and those with extensive calcification, radical pericardiectomy remains the only potentially curative treatment. It is hoped that future advances in diagnostic modalities and personalized therapeutic strategies will further improve or replace current approaches.

Keywords: Constrictive pericarditis; Diastolic heart failure; Echocardiography; Magnetic resonance imaging; Colchicine; Pericardiectomy

Core Tip: constrictive pericarditis (CP) is characterized by pericardial thickening and fibrosis, resulting in impaired diastolic relaxation and progressive right-sided heart failure. Diagnosis is often delayed because of overlapping features with other causes of heart failure and chronic liver disease. Advances in noninvasive multimodality imaging, particularly transthoracic echocardiography, cardiac magnetic resonance, and cardiac computed tomography, have improved diagnostic accuracy. Management has evolved toward a phenotype-based approach, with anti-inflammatory therapy for inflammatory CP and radial pericardiectomy remaining the only potentially curative treatment for non-inflammatory, refractory, or calcified disease.