Document Detail

A case of severe Ebstein's anomaly with incompetent pulmonary valve.
MedLine Citation:
PMID:  12026209     Owner:  NLM     Status:  MEDLINE    
A case of Ebstein's anomaly with functional pulmonary atresia diagnosed in utero is presented. The diagnosis was confirmed by postnatal echocardiographic, angiographic, and postmortem pathologic findings. On echocardiography the septal leaflet of the tricuspid valve was displaced towards the right ventricular apex. The tricuspid valve was moderately regurgitant and the arterial duct was patent. Continuous wave or color Doppler revealed serious reduction in forward flow from right ventricle through the pulmonary arteries; however, massive pulmonary regurgitation was observed. Pulmonary circulation was dependent on the ductal flow due to functional pulmonary atresia. Angiography revealed the massively enlarged right atrium, the absence of forward flow through the tricuspid valve, transfer of contrast material through the atrial septal defect to the left atrium, and the retrograde inflow of the pulmonary arteries from the aorta via the patent arterial duct. Ebstein's anomaly accompanied by functional pulmonary atresia is very rare. The fetal and neonatal presentation of this anomaly is associated with poor outcome.
Süheyla Ozkutlu; Naci Ceviz; Canan Ayabakan; Zuhal Akçören
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Turkish journal of pediatrics     Volume:  44     ISSN:  0041-4301     ISO Abbreviation:  Turk. J. Pediatr.     Publication Date:    2002 Apr-Jun
Date Detail:
Created Date:  2002-05-23     Completed Date:  2002-06-11     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0417505     Medline TA:  Turk J Pediatr     Country:  Turkey    
Other Details:
Languages:  eng     Pagination:  162-7     Citation Subset:  IM    
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
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MeSH Terms
Ebstein Anomaly / ultrasonography*
Fatal Outcome
Fetal Diseases / ultrasonography*
Heart Catheterization
Infant, Newborn
Oligohydramnios / ultrasonography
Prenatal Diagnosis
Pulmonary Atresia / ultrasonography*
Pulmonary Valve / pathology,  radiography,  ultrasonography
Tricuspid Valve / abnormalities
Ultrasonography, Prenatal

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