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Congenital pericardium anomaly

Orpha number ORPHA2846
Synonym(s) -
Prevalence Unknown
Inheritance Not applicable
Age of onset All ages
ICD-10
  • Q24.8
ICD-O -
OMIM -
UMLS
  • C0685699
MeSH -
MedDRA -
SNOMED CT
  • 93018000

Summary

Congenital absence and pericardial cysts are the 2 major congenital abnormalities of the pericardium. Absence of pericardium can be total or partial. The complete form generally remains non symptomatic and is diagnosed incidentally. The incomplete form mainly concerns the left heart (80%) and may cause herniation and probably strangulation of parts of the left heart. Symptomatic patients complain of syncope, chest pain or arrhythmias. Death may be caused by torsion of the great arteries, constriction of a coronary artery or herniation and/or incarceration of the left atrial appendage. Chest x-ray demonstrates leftward displacement and posterior bulging of the heart in the complete form. Herniation of the left atrial appendage resembles enlargement of the pulmonary artery. On echocardiography excessive cardiac motion and enlargement of the left atrial appendage may be seen. Only magnetic resonance imaging and computed tomography can reveal the pericardial defect. The true frequency of pericardial defects is unknown. Treatment consists in enlargement of the defect or patch closure depending on the size of the defect. Surgical therapy in total absence of the pericardium is rarely needed.
Congenital non-malignant cysts include pericardial teratoma, cystic lymphangioma, bronchogenic and celomic cysts. All are most often located at the base of the heart causing compression of the great vessels. Echocardiography may demonstrate the cystic lesion. Computed tomography or magnetic resonance imaging may well show the extent and location of the cysts. In symptomatic patients excision may be necessary and will be curative.

Expert reviewer(s)

  • Dr Hans-Peter GILDEIN

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