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.
Brulotte
Steeve
S
Quebec Heart Institute at Laval Hospital.
Roy
Louis
L
Larose
Eric
E
D000208:Acute Disease; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis*; D006801:Humans; D033162:Incidental Findings; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D009336:Necrosis / Q000175:diagnosis*; D016584:Panic Disorder / Q000150:complications; D010496:Pericardium / Q000002:abnormalities*; D013575:Syncope