Document Detail

Prenatal and Pathological Features of Aortico-Left Ventricular Tunnel Causing Fetal Hydrops and Intrauterine Demise.
MedLine Citation:
PMID:  23286355     Owner:  NLM     Status:  Publisher    
Abstract Aortico-left ventricular tunnel (ALVT) is a rare congenital heart anomaly of unclear pathogenesis causing left ventricular volume overload. Most descriptions are from surgical corrections in the neonatal and infantile period and little is known about ALVT in utero. Here we describe a case of ALVT ending in intrauterine demise with fetal hydrops at 30 weeks gestational age. Prior echocardiography showed features suggesting the presence of an ALVT and an ALVT circumventing the left coronary cusp of the aortic valve was confirmed at autopsy. Marked cardiomegaly and dilatation was present along with diffuse myocardial infarction. Other potential causes of fetal hydrops were not found and genetic analysis, including analysis for RAS pathway mutations, did not reveal any detectable abnormalities. The presentation as intrauterine demise suggests that the architecture of this ALVT compromises cardiac function to a greater degree than the more typical defects discovered in the neonatal period.
Jefferson Terry; Mondal Tapas; Malgorzata J M Nowaczyk
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-3
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  -     ISSN:  1093-5266     ISO Abbreviation:  Pediatr. Dev. Pathol.     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-4     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
a McMaster Children's Hospital, Pathology and Molecular Medicine.
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