Congenital absence of the pericardium and its mimics

Congenital absence of the pericardium is a rare entity, with less than 400 cases reported in the literature. Pericardial absence is typically left sided, which results in herniation of the great vessels or portions of the heart. Patients may be asymptomatic, typical for complete defects, or can present with various degrees of chest pain in the setting of partial absence and strangulation. The finding may be isolated or associated with complex heart disease. We present a number of cases of pericardial absence that show isolated right- and left-sided defects, as well as entities in the differential diagnosis. Early recognition may decrease morbidity and mortality, resulting from delay in proper treatment.