Document Detail

Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome.
MedLine Citation:
PMID:  12586257     Owner:  NLM     Status:  MEDLINE    
We reported echocardiographic findings and outcomes of fetuses with absent pulmonary valve syndrome diagnosed during fetal life. Cases were identified from a prospectively acquired computerized database of 18,308 pregnancies referred to a fetal cardiology center between January 1988 and July 2000. Twenty fetuses were identified with a median gestation of 23 weeks (range 18 to 36) at presentation. In 18 cases (90%), there was an associated ventricular septal defect. Eighteen cases (90%) had branch pulmonary artery diameters above the normal range. In four cases (20%), an arterial duct was present. A chromosome 22q11 deletion was identified in 2 of 9 cases (22%) in which this deletion was sought. There were 6 terminations of pregnancy (30%), 3 intrauterine deaths (15%), 5 neonatal deaths (25%), 3 infant deaths (15%), and 3 patients who did not die (15%). Ten of the 11 "liveborn" infants required early ventilation. The outcome of absent pulmonary valve syndrome diagnosed prenatally appears poor. The high morbidity and mortality is due to both cardiac disease and associated bronchomalacia.
Reza S Razavi; Gurleen K Sharland; John M Simpson
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of cardiology     Volume:  91     ISSN:  0002-9149     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  2003 Feb 
Date Detail:
Created Date:  2003-02-14     Completed Date:  2003-03-13     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  429-32     Citation Subset:  AIM; IM    
Fetal Cardiology Unit, Department of Congenital Heart Disease, Guy's Hospital, London, United Kingdom.
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MeSH Terms
Abnormalities, Multiple / genetics,  mortality,  therapy,  ultrasonography*
Abortion, Therapeutic
Chromosome Deletion
Chromosomes, Human, Pair 22 / genetics
Echocardiography / methods*
Fetal Death / etiology
Gestational Age
Heart Septal Defects, Ventricular / genetics,  mortality,  therapy,  ultrasonography*
Intensive Care, Neonatal
Pregnancy Outcome
Prospective Studies
Pulmonary Atresia / genetics,  mortality,  therapy,  ultrasonography*
Respiration, Artificial
Survival Analysis
Treatment Outcome
Ultrasonography, Prenatal / methods*

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

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