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