Document Detail


Left ventricular non-compaction on MRI in a patient with 22q11.2 distal deletion.
MedLine Citation:
PMID:  20425839     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on a 22-year-old male carrying a presumptive clinical diagnosis of Dubowitz-like phenotype who has been followed-up by cardiology for bicuspid aortic valve with ascending aorta and aortic root dilatation. Cardiac magnetic resonance imaging (CMRI) confirmed these findings, along with an incidental finding of left ventricular non-compaction (LVNC). Genetic workup revealed the diagnosis of 22q11.2 distal deletion encompassing the BCR gene. This is the first time LVNC has been reported in a patient with 22q11.2 distal deletion.
Authors:
Shobhit Madan; Suneeta Madan-Khetarpal; Sang C Park; Urvashi Surti; Ariel L Bailey; Juliann McConnell; Sameh S Tadros
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  152A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-28     Completed Date:  2010-08-12     Revised Date:  2011-06-29    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1295-9     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Wiley-Liss, Inc.
Affiliation:
Department of Radiology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA 15224, USA.
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Chromosome Deletion*
Chromosomes, Human, Pair 22 / genetics*
Facies
Heart Defects, Congenital / genetics*
Heart Ventricles / abnormalities*
Humans
Magnetic Resonance Imaging*
Male
Oligonucleotide Array Sequence Analysis
Young Adult
Comments/Corrections
Comment In:
Am J Med Genet A. 2011 Mar;155A(3):662-3; author reply 664-5   [PMID:  21337687 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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