title: Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study.
abstract: BACKGROUND
  Despite serious long-term sequel, women with Fontan palliation have reached childbearing age. However there is paucity of data on the pregnancy outcomes and management of this condition. We aimed to determine the maternal and fetal outcomes of pregnancy in women with Fontan palliation.
  
  
  METHODS
  This multicentric, retrospective study included women with Fontan circulation followed in 13 French specialized centers from January 2000 to June 2014. All pregnancies were reviewed, including miscarriages, abortions, premature and term births. We reviewed maternal and fetal outcomes.
  
  
  RESULTS
  Thirty-seven patients had 59 pregnancies. Mean age was 27 ± 5 years at first pregnancy. There were 16 miscarriages (27%) and 36 live births with 1 twin pregnancy. Cardiac events occurred in 6 (10%) pregnancies, with no maternal death. The most common cardiac complication was atrial arrhythmia, which occurred in 3 patients. Hematological complications including thromboembolic/hemorrhagic events (n=3/7) occurred in 5 women antepartum (n=2/3), and 4 women postpartum (n=1/4). Two of the 3 thromboembolic events occurred in patients without anticoagulation. There was a high incidence of prematurity (n=25/36, 69%). Anticoagulation was associated with adverse neonatal outcome (OR=10.0, 95% CI [1.5-91.4], p<0.01). After a median follow-up of 24 months, there was no significant worsening of clinical status and thromboembolic disease noted.
  
  
  CONCLUSIONS
  Pre-selected women can successfully complete pregnancy with Fontan circulation. There is an increase in cardiac and neonatal morbidity during pregnancy. Because thromboembolism could have a severe consequence on Fontan circulation, anticoagulation should be indicated during pregnancy and postpartum period.
authors.affiliation: Centre de Référence des Malformations Cardiaques Congénitales Complexes, M3C Paris, France; Adult Congenital Heart Disease Unit, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson, France.
authors.forename: Marielle
authors.initials: M
authors.lastname: Gouton
authors.affiliation: Service de Gynécologie Obstétrique, Groupe Hospitalier Pitié-Salpêtrière, APHP, Sorbonne Universités, UPMC Univ Paris 06, CNRS UMR 7222, INSERM U1150-, Paris, France.
authors.forename: Jacky
authors.initials: J
authors.lastname: Nizard
authors.affiliation: Baylor College of Medicine, Houston, TX, USA.
authors.forename: Mehul
authors.initials: M
authors.lastname: Patel
authors.affiliation: Cardiologie Pédiatrique et Congénitale Adulte, Hôpital Cardiovasculaire Louis Pradel, Lyon, France.
authors.forename: François
authors.initials: F
authors.lastname: Sassolas
authors.affiliation: Pediatric Department, Clinique Saint Augustin, Bordeaux, France.
authors.forename: Maria
authors.initials: M
authors.lastname: Jimenez
authors.affiliation: Cardiologie Congénitale, Strasbourg, France.
authors.forename: Jelena
authors.initials: J
authors.lastname: Radojevic
authors.affiliation: Pediatric Cardiology Department, Hôpital Nord, Amiens, France.
authors.forename: Amel
authors.initials: A
authors.lastname: Mathiron
authors.affiliation: Pediatric Department 1, Hôpital Arnaud de Villeneuve, Montpellier, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Amedro
authors.affiliation: Pediatric Department 1, Centre Hospitalo-Universitaire de Rouen, Rouen, France.
authors.forename: Elise
authors.initials: E
authors.lastname: Barre
authors.affiliation: Department of Cardiology, CHU de Caen, Avenue Côte-de-Nacre, Caen, France.
authors.forename: Fabien
authors.initials: F
authors.lastname: Labombarda
authors.affiliation: Maladies Cardiovasculaires Infantiles et Congénitales, Lille, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Vaksmann
authors.affiliation: Pediatric Department, Hôpital Clocheville, Tours, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Chantepie
authors.affiliation: Cardiologie Congénitale Adulte et Pédiatrique, Hôpital Mère-enfant-maternité, Nantes, France.
authors.forename: Laurianne
authors.initials: L
authors.lastname: Le Gloan
authors.affiliation: Centre de Référence des Malformations Cardiaques Congénitales Complexes, M3C Paris, France; Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpital Européen Georges Pompidou and Necker Hospital, APHP, Paris Descartes Universités, Paris Centre de Recherche Cradiovasculaire, INSERM U970, Paris, France. Electronic address: magalie.ladouceur@egp.aphp.fr.
authors.forename: Magalie
authors.initials: M
authors.lastname: Ladouceur
mesh_terms: D000328:Adult; D005260:Female; D005602:France / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000453:epidemiology*; D011256:Pregnancy Outcome / Q000453:epidemiology*; D012189:Retrospective Studies
keywords: Anticoagulation; Congenital heart disease; Fontan circulation; Pregnancy