Abstract
Congenital absence of the pericardium is a very uncommon finding, and its diagnosis
poses a challenge because it is rarely suspected in daily clinical practice. Although
in most cases it has a benign course, this congenital defect should be identified
because of the associated risk of sudden death. We present a symptomatic case of partial
congenital absence of the left pericardium suspected as the result of an abnormal
response to exercise stress testing, and confirmed using cardiac magnetic resonance
imaging. We review the current diagnostic tools and therapeutic indications of this
rare anomaly.
Résumé
L’absence congénitale du péricarde est découverte très peu fréquemment et puisqu’elle
est rarement soupçonnée dans la pratique clinique quotidienne, son diagnostic pose
un défi. Bien que dans la plupart des cas son évolution soit bénigne, cette anomalie
congénitale devrait être décelée en raison du risque de mort subite associé. Nous
présentons un cas symptomatique d’absence congénitale partielle du péricarde du côté
gauche soupçonnée être le résultat d’une réponse anormale à l’épreuve d’effort et
confirmée par l’imagerie cardiaque par résonance magnétique. Nous passons en revue
les outils actuels de diagnostic et les indications thérapeutiques de cette anomalie
rare.
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References
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- Congenital absence of the pericardium: case presentation and review of literature.Int J Cardiol. 2005; 98: 21-25
- Functional MRI of congenital absence of the pericardium.Am J Roentgenol. 2007; 189: W312-W314
- Diagnosis of congenital pericardial defects, including a pathognomonic sign for dangerous apical ventricular herniation, on magnetic resonance imaging.Br Heart J. 1995; 74: 60-66
- Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215
Article info
Publication history
Published online: May 16, 2015
Accepted:
May 10,
2015
Received:
March 29,
2015
Footnotes
See page 1303.e6 for disclosure information.
Identification
Copyright
© 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
