Congenital Abnormalities of the Pericardium

Congenital abnormalities of the pericardium are a rare group of disorders that include congenital absence of the pericardium, pericardial cysts, and diverticula. These congenital defects result from alterations in the embryologic formation and structure of the pericardium. Although many cases are incidentally found, they can present as symptomatic, life-threatening disease. Owing to their rarity, many cases are inappropriately diagnosed. Alterations in the embryologic formation and structure may result in the formation of these congenital abnormalities. We review the presentation, diagnosis, and management of congenital absence of the pericardium, pericardial cysts, and diverticula. A summary of multimodality imaging features is provided.