Document Detail


An unusual cardiac defect in a patient with clinical features overlapping between cardiofaciocutaneous and Noonan syndromes.
MedLine Citation:
PMID:  20136862     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
It is important to recognize the possibility of a syndromic etiology of cardiac defects when dysmorphic features and other congenital defects are present. We report a patient who presented with atrial fibrillation and was found to have an abnormal mitral valve, congenital aneurysm of the left atrial appendage, and features consistent with both Cardiofaciocutaneous syndrome and Noonan syndrome. The congenital aneurysm of the left atrial appendage was a previously unreported cardiac presentation for either syndrome. Diagnostic considerations based upon his genotype and phenotype are discussed, along with his unique cardiac presentation and treatment.
Authors:
Holly C DeSena; Dmitriy M Niyazov; P Eugene Parrino; Victor W Lucas; Sangeeta B Shah; Douglas S Moodie
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Congenital heart disease     Volume:  5     ISSN:  1747-0803     ISO Abbreviation:  Congenit Heart Dis     Publication Date:    2010 Jan-Feb
Date Detail:
Created Date:  2010-02-08     Completed Date:  2010-04-22     Revised Date:  2011-05-05    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  70-5     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Tulane University, New Orleans, LA 70112, USA. hdesena@tulane.edu
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple* / diagnosis,  genetics,  surgery
Atrial Appendage / abnormalities*,  surgery
Atrial Fibrillation / diagnosis,  genetics*,  surgery
Cardiac Surgical Procedures
DNA Mutational Analysis
Electrocardiography
Genotype
Heart Aneurysm / congenital*,  diagnosis,  genetics,  surgery
Heart Defects, Congenital / complications*,  diagnosis,  genetics,  surgery
Heart Valve Prosthesis Implantation
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Mitral Valve / abnormalities*,  surgery
Noonan Syndrome / complications*,  diagnosis,  genetics,  surgery
Phenotype
Treatment Outcome

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


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