Objectives, This study sought to determine risks and outcome of pregna
ncy and delivery after the modified Fontan operation. Background. Incr
easingly, female Fontan patients reaching child-bearing years are inte
rested in having children, To date, the number of reported pregnancies
is small, and pregnancy has therefore been discouraged. Methods. One
hundred ten of 126 female patients from the Fontan registries of the M
ayo Clinic and University of California Los Angeles Medical Center res
ponded to a mailed questionnaire, An additional six patients with a re
ported pregnancy from other centers were identified and reviewed to as
sess pregnancy outcomes. Results. Among the participating centers, a t
otal of 33 pregnancies after Fontan operation for various types of uni
ventricular heart disease were reported, There were 15 (45%) live birt
hs from 14 mothers, with 13 spontaneous abortions and 5 elective termi
nations, In the 14 women with live births, the median number of years
between operation and pregnancy was 4 (range 2 to 14), Reported prepre
gnancy problems in these gravidas included atrial butter in one patien
t and ventricular dysfunction, aortic regurgitation and atrioventricul
ar valve regurgitation in another, One patient developed supraventricu
lar tachycardia during pregnancy and had conversion to sinus rhythm, N
o maternal cardiac complications were reported during labor, delivery
or the immediate puerperium. There were six female and nine male infan
ts (mean gestational age 36.5 weeks; median weight 2,344 g), One infan
t had an atrial septal defect, At follow-up, mothers and infants were
alive and well. Conclusions. Pregnancy after the Fontan operation appe
ars to have been well tolerated in 13 of 14 gravidas, There does appea
r to be an increased risk of miscarriage, The tendency to routinely di
scourage pregnancy may need to be reconsidered.