ArticleEuropean heart journal. Case reports2025
A very rare phenotype of immunoglobulin G4-related disease that was manifested as constrictive pericarditis: a case report.
Article in European heart journal. Case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Constrictive pericarditis (CP) can arise from various causes, including post-operative degeneration, tuberculosis, and sequelae of pericarditis. Immunoglobulin (Ig) G4-related disease is a rare but recognized cause of CP. However, the specific mechanisms underlying these aetiologies and pathologies remain unclear. Case summary: A 67-year-old man presented with a 6-month history of bilateral leg oedema, anorexia, and dyspnoea on exertion. Computed tomography (CT) revealed significant pericardial thickening without calcification, right pleural effusion, and ascites. Echocardiography demonstrated a reduced left ventricular ejection fraction and pericardial thickening. The early diastolic mitral annular tissue velocity (e') was preserved as 11.7 cm/s, despite inferior vena cava dilation. Respiratory variations in mitral inflow velocities and septal bounces were unremarkable. Cardiac catheterization further showed a 'dip and plateau' pattern with equalization of bilateral ventricular end-diastolic pressure. A preliminary diagnosis of CP was made, and pericardiectomy was performed, increasing the cardiac index from 2.0 to 3.0 L/min/m Discussion: Optimal therapy for IgG4-related CP remains elusive due to its rarity. Potential therapeutic options include pericardiectomy, pericardiotomy, and corticosteroid therapy. Further examination through the accumulation of similar cases is crucial to establish definitive treatment approaches for this condition.
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