Document Detail

Tricuspid valve dysplasia with severe tricuspid regurgitation: fetal pulmonary artery size predicts lung viability in the presence of small lung volumes.
MedLine Citation:
PMID:  20090295     Owner:  NLM     Status:  MEDLINE    
Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes. The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. Use of fetal echocardiography with measurement of pulmonary artery size combined with prenatal MRI scanning of lung volumes resulted in an improved understanding of this anomaly and directed the management strategy towards a successful Fontan circulation.
A T Nathan; B S Marino; T Dominguez; S Tabbutt; S Nicolson; D D Donaghue; T L Spray; J Rychik
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-01-12
Journal Detail:
Title:  Fetal diagnosis and therapy     Volume:  27     ISSN:  1421-9964     ISO Abbreviation:  Fetal. Diagn. Ther.     Publication Date:  2010  
Date Detail:
Created Date:  2010-03-24     Completed Date:  2010-06-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107463     Medline TA:  Fetal Diagn Ther     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  101-5     Citation Subset:  IM    
Copyright Information:
2010 S. Karger AG, Basel.
Cardiac Center, Children's Hospital of Philadelphia, University of Pennsylvania, 19104, USA.
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MeSH Terms
Cardiomegaly / embryology,  surgery,  ultrasonography
Cardiopulmonary Bypass
Ebstein Anomaly / diagnosis*,  surgery
Fetal Diseases / ultrasonography
Gestational Age
Heart Atria / surgery,  ultrasonography
Infant, Newborn
Lung / embryology*,  ultrasonography
Magnetic Resonance Imaging
Prenatal Diagnosis / methods*
Pulmonary Artery / embryology*,  surgery
Tricuspid Valve / abnormalities*,  embryology,  surgery
Tricuspid Valve Insufficiency / surgery,  ultrasonography*
Ultrasonography, Prenatal

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

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