Outcomes of pregnancy in women after Fontan palliation

Marielle Gouton, Magalie Ladouceur, Jacky Nizard, Marc Dommergues

Centre Chirurgical Marie Lannelongue – Le Plessis-RobinsonHôpital Européen Georges Pompidou – Paris

Groupement Hospitalier La Pitié-Salpêtrière – ParisFrance

The 3rd International Congress on Cardiac Problems in Pregnancy – Venice 2014

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 complicationsPre-partum, peri-partum, post-partum, follow-up

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

Women n (%)Cardiac malformation Tricuspid atresia DILV DORV Single Ventricle (undefined) Large VSD – straddling valves

(n=27)13 (48%) 2 (7.5%) 2 (7.5%) 9 (33%) 1 (4%)

Type of Fontan palliation Fontan Kreutzer Extracardiac TCPC Intracardiac TCPC Unknown

326115

Mean age at Fontan palliation, ±SD 15±6 yConversion Fontan or Kreutzer in to TCPC 5CV complications before pregnancy SV tachycardia Heart failure Thromboembolism

6 (23%)314

Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

Population

Results : 41 pregnancies

Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

Type of palliatio

nAbortio

nMiscarriag

eFetal death

Live births Total

Fontan / Kreutzer 0 2 1 4 7

TCPC 2 10 0 17 29

? 1 1 0 3 5

Total 3 13 1 24 41

58 %

33 %

Maternal complications10 / 41 (25 %)

Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

Type nSupra Ventricular Tachycardia 2Thromboembolism 2Hypoxemia 1

Others Dizziness Renal insufficiency Anemia (requiring transfusion) Hepatic cytolysis (x3) Hypoalbuminemia

11111

Maternal death 0

Obstetrical complications 25 / 41 (61 %)

Type n

Miscarriage 13SGA 2Fetal death 1Utero placentar anomaly 1

Amniotic anomaly 4Abruptio 2Premature rupture of membranes 5Preterm labor 5Delivery bleeding 1

Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

Anticoagulation ?

Type n Live births (%)

Maternal complicatio

n / thrombotic

No anticoagulation 2 2 (100) 1 / 0

Aspirin 5 2 (40) 2 / 1

Vitamin K inhibitor 6 2 (33) -

Heparin (preventive) 10 8 (80) 4 / 0

Heparin (curative) 8 6 (75) 3 /1

? 9 3 (33) -Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014

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)

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

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

Fontan and twin pregnancy ?Yes, they can

Outcomes of pregnancy in women after Fontan palliation – 3rd International Congress on CPP – Venice 2014