Pregnancy Nowadays in Women with Fontan Circulation: Single-Centre Experience

Background: The Fontan operation today enables survival of adult patients with single-ventricle physiology to childbearing ages. As a result, many women seek medical advice about pregnancy. There are limited data available about the current risk of maternal cardiovascular and obstetric complications, miscarriage, and prematurity risk in German Fontan patients. We present our recent experience with pregnancy outcomes in women with Fontan palliation.

Method: We recorded retrospectively pregnancy outcome, maternal cardiovascular and obstetric complications, and fetal outcome in patients with Fontan palliation.

Results: Six women with Fontan palliation (5 × systemic LV and 1 × RV) had nine pregnancies with five live births, three spontaneous miscarriages and one ectopic pregnancy, terminated with methotrexate. Median gestational age at delivery was 37 weeks (27–40 weeks) with two pregnancies resulting in preterm delivery (27th and 32nd weeks). Three infants (one preterm birth and two term births) were low weight. Three pregnancies resulted in uncomplicated deliveries, two of which with caesarean section (obstetric and cardiological indication, respectively). One of the live birth pregnancies incurred serious complications for mother and infant. The mother was admitted at 27 weeks with preeclampsia, hepatopathy, protein loss, exsudative enteropathy, and ascites. A ceasarean section was performed because of high fetoplacental resistance on Doppler and oligo/anhydramnion. Maternal laparotomy had to be performed twice because of hematoma. This infant suffered from an intracranial hemorrhage and remained severely handicapped. Another pregnancy was complicated by severe bleeding requiring transfusion (4 RBC-concentrates). One patient had palpitations during pregnancy, no severe arrhythmias were observed. There were no maternal deaths.

Conclusion: In contemporary pregnancies of our Fontan patients, we observed a higher prevalence of first trimester miscarriages (~33%), intrauterine growth restriction (60%), preterm births (40%), and postpartum hemorrhage (40%). However, no significant worsening in maternal clinical status or myocardial function were observed over a mean follow-up of 5 years (1–15 years). Successful pregnancy is achievable, but prepregnancy assessment and counseling are essential. Pregnancy and delivery should be managed in a specialized multidisciplinary center.

Publication History

Article published online:
12 February 2022