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(Circulation. 1998;98:562-566.)
© 1998 American Heart Association, Inc.

Clinical Investigation and Reports

Pulmonary Atresia With Intact Ventricular Septum

Impact of Fetal Echocardiography on Incidence at Birth and Postnatal Outcome

P. E. F. Daubeney, MRCP; G. K. Sharland, MD; A. C. Cook, BSc; B. R. Keeton, FRCP; R. H. Anderson, MD; S. A. Webber, MRCP; ; for the UK and Eire Collaborative Study of Pulmonary Atresia With Intact Ventricular Septum

From Wessex Cardiothoracic Centre, Southampton General Hospital (P.E.F.D., B.R.K., S.A.W.), Southampton, UK; Fetal Cardiology, Guy's Hospital, (G.K.S., A.C.C.), London, UK; and the Department of Paediatrics, Imperial College at the National Heart and Lung Institute, (R.H.A.), London, UK. Correspondence to Dr S.A. Webber, Division of Cardiology, Children's Hospital of Pittsburgh, 3705 Fifth Ave at De Soto St, Pittsburgh, PA 15213.

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.

Key Words: pulmonary heart disease • pulmonary atresia • echocardiography • diagnosis • pediatrics

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