Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.
BACKGROUND
Congenital absence of the pericardium (CAP) is a rare clinical entity.


METHODS
We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI).


RESULTS
Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertional (9), and heart murmur with an abnormal CXR (1). Three patients had partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagnosis; a "tongue" of lung tissue interposing between the main pulmonary artery and aorta was the most consistent diagnostic feature. Four patients underwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial appendage herniation, followed by improvement or resolution of symptoms. At a mean of 10.5 years from presentation all patients were alive. No complications were seen in the nonsurgical group.


CONCLUSIONS
Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease. CXR and MRI are required for definitive diagnosis. Symptomatic patients with the complete form may benefit from pericardioplasty.
Gatzoulis
M A
MA
Department of Medicine, Toronto Hospital, Ontario, Canada. m.gatzoulis@rbh.nthames.nhs.uk
Munk
M D
MD
Merchant
N
N
Van Arsdell
G S
GS
McCrindle
B W
BW
Webb
G D
GD
D000293:Adolescent; D000328:Adult; D002637:Chest Pain / Q000209:etiology; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010496:Pericardium / Q000002:abnormalities*; D011138:Polytetrafluoroethylene; D019736:Prostheses and Implants; D019651:Plastic Surgery Procedures
D011138:Polytetrafluoroethylene