Pregnancy and delivery in women after Fontan palliation.
OBJECTIVE
To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders.


METHODS
Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18-45 years): atriopulmonary anastomosis (n = 23), atrioventricular connection (n = 5) and total cavopulmonary connection (n = 10).


RESULTS
Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n = 2), uterus bicornis (n = 1) and related to endometriosis (n = 1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n = 15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population).


CONCLUSIONS
Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.
Drenthen
W
W
Department of Cardiology, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands. w.drenthen@thorax.umcg.nl
Pieper
P G
PG
Roos-Hesselink
J W
JW
van Lottum
W A
WA
Voors
A A
AA
Mulder
B J M
BJ
van Dijk
A P J
AP
Vliegen
H W
HW
Sollie
K M
KM
Moons
P
P
Ebels
T
T
van Veldhuisen
D J
DJ
D000293:Adolescent; D000328:Adult; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006801:Humans; D007247:Infertility, Female / Q000209:etiology; D008599:Menstruation Disturbances / Q000209:etiology; D008875:Middle Aged; D010166:Palliative Care; D011247:Pregnancy; D011248:Pregnancy Complications / Q000209:etiology*; D011249:Pregnancy Complications, Cardiovascular; D011256:Pregnancy Outcome