Congenital absence of the pericardium presenting as acute myocardial necrosis.

Congenital absence of the pericardium is a rare defect of which recognition is critical because it can be associated with catastrophic outcomes. While some carriers of this condition may present with a lethal complication, most are asymptomatic, and the defect is found incidentally. The case of a 49-year-old woman is described who presented with acute myocardial necrosis and absence of obstructive coronary artery disease, and in whom a complete left pericardial defect was found. An investigation was undertaken to determine the role of the defect in this patient's presentation and to recommend the best possible therapy. Finally, a review of literature focusing on congenital absence of the pericardium, as well as a discussion of clinical presentation, imaging techniques and therapeutic options, is also presented.