Document Detail

Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum.
MedLine Citation:
PMID:  9714114     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. METHODS AND RESULTS: From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100,000 live births). The incidence was 4.1 cases per 100,000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland (P=0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100,000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths (P=0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. CONCLUSIONS: PAIVS is rare, occurring in 1 in 22,000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.
P E Daubeney; G K Sharland; A C Cook; B R Keeton; R H Anderson; S A Webber
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Circulation     Volume:  98     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1998 Aug 
Date Detail:
Created Date:  1998-09-15     Completed Date:  1998-09-15     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  562-6     Citation Subset:  AIM; IM    
Wessex Cardiothoracic Centre, Southampton General Hospital, UK.
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MeSH Terms
Abortion, Induced / statistics & numerical data
Fetal Diseases / therapy
Heart Septal Defects, Ventricular / epidemiology*,  therapy,  ultrasonography*
Infant, Newborn
Pregnancy Outcome
Pulmonary Atresia / diagnosis*,  epidemiology*,  therapy
Survival Analysis
Ultrasonography, Prenatal*

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