1. Outcomes of pregnancy in women after Fontan palliation Marielle Gouton, Magalie Ladouceur, Jacky Nizard, Marc Dommergues Centre Chirurgical Marie Lannelongue – Le Plessis-Robinson Hôpital Européen Georges Pompidou – Paris Groupement Hospitalier La Pitié-Salpêtrière – Paris France The 3rd International Congress on Cardiac Problems in Pregnancy – Venice 2014

2. Design Outcomes of all pregnancies in women with Fontan palliation in France Methods : Retrospective study French M3C centers Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 2001-2013 All known pregnancies Cardiac, obstetrical and neonatal complications Pre-partum, peri-partum, post-partum, follow-up

3. Population 27 women, 41 pregnancies (1 twin-pregnancy) 4 / 27 (15 %) had previous infertility 9 had 2 pregnancies or more Age at first pregnancy : 20 – 41 y (mean 27± 5) Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

4. Womenn (%) Cardiac malformation Tricuspid atresia DILV DORV Single Ventricle (undefined) Large VSD – straddling valves (n=27) 13 (48%) 2 (7.5%) 9 (33%) 1 (4%) Type of Fontan palliation Fontan Kreutzer Extracardiac TCPC Intracardiac TCPC Unknown 3 2 6 11 5 Mean age at Fontan palliation, ±SD15±6 y Conversion Fontan or Kreutzer in to TCPC5 CV complications before pregnancy SV tachycardia Heart failure Thromboembolism 6 (23%) 3 1 4 Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 Population

5. Results : 41 pregnancies Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 Type of palliation AbortionMiscarriage Fetal death Live births Total Fontan / Kreutzer 02147 TCPC21001729 ?11035 Total31312441 58 % 33 %

6. Maternal complications 10 / 41 (25 %) Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 Typen Supra Ventricular Tachycardia2 Thromboembolism2 Hypoxemia1 Others Dizziness Renal insufficiency Anemia (requiring transfusion) Hepatic cytolysis (x3) Hypoalbuminemia 1111111111 Maternal death0

7. Obstetrical complications 25 / 41 (61 %) Typen Miscarriage13 SGA2 Fetal death1 Utero placentar anomaly1 Amniotic anomaly4 Abruptio2 Premature rupture of membranes5 Preterm labor5 Delivery bleeding1 Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

8. Anticoagulation ? TypenLive births (%) Maternal complication / thrombotic No anticoagulation22 (100) 1 / 0 Aspirin52 (40) 2 / 1 Vitamin K inhibitor62 (33) - Heparin (preventive)108 (80) 4 / 0 Heparin (curative)86 (75) 3 /1 ?93 (33) - Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

9. Post-natal follow-up Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 Mode of delivery : Vaginal : 12 Cesarean section : 12 Mean gestational age : 34 (±3) w Mean birth weight : 2024 (±690) g (10th percentile) Prematurity : 17 / 24 (70 %) 33-36+6 w : 11 28-32+6 w : 5 22-27+6 w : 1 Neonatal death : 1 (birth at 25 w) Twins : birth by cesarean section at 32 w Recurrence of congenital heart disease : 1 left superior vena cava (fetal death : tricuspid atresia)

10. Maternal follow-up Post-partum complications Parietal hematoma :1 Fontan failure: 1 ↗ NYHA: 1 21 / 27 women (75%) have ≥ 1 newborn 0 newborn : 6 1 newborn : 18 (1 neonatal death) 2 newborns : 3 Late follow-up : 43 months (1 – 144) Fontan failure : 2 No maternal death Le switch a 30 ans ! Atelier-débat - CCML 6 et 7 février 2014

11. Fontan and pregnancy: Is it possible ? Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014 Yes, but only if : Pre-conception visit Cardiac function Ventricular function (echo, MRI) TCPC function AV valve insufficiency Counseling High risk of maternal complications Arrhythmia Thromboembolism Therefore: anticoagulation High risk of miscarriage (33%) High risk of prematurity (70%) Risk of recurrence Multidisciplinary follow-up (tertiary hospital) Adult cardiac congenitalist Obstetrician Anesthesiologist Neonatologist Vaginal delivery if obstetrician agrees

12. Fontan and twin pregnancy ? Yes, they can Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014