| Interrupted right aortic arch in DiGeorge syndrome. | |
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MedLine Citation:
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PMID: 3663429 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome. |
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Authors:
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P Moerman; M Dumoulin; J Lauweryns; L G Van der Hauwaert |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: British heart journal Volume: 58 ISSN: 0007-0769 ISO Abbreviation: Br Heart J Publication Date: 1987 Sep |
Date Detail:
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Created Date: 1987-12-17 Completed Date: 1987-12-17 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 0370634 Medline TA: Br Heart J Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 274-8 Citation Subset: AIM; IM |
Affiliation:
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Department of Pathology, Gasthuisberg University Hospital, Leuven, Belgium. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple* Aorta, Thoracic / abnormalities* Cardiovascular Abnormalities DiGeorge Syndrome / complications*, congenital Female Humans Immunologic Deficiency Syndromes / complications* Infant, Newborn Thymus Gland / abnormalities* |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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