title: Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease.
abstract: There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD.
  
  
  
  Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes.
  
  
  
  28 pregnancies in 20 women (26±6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks' gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p<0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred.
  
  
  
  Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.
authors: Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C.|Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpital Européen Georges Pompidou and Necker Hospital, APHP, Paris Descartes University, Paris, France.|Paris Cardiovascular Research Center (PARCC) INSERM U970, Paris, France.
authors: Magalie
authors: M
authors: Ladouceur
authors: Department of Obstetrics and Gynecology, Groupe hospitalier Pitié-Salpêtrière Paris, APHP, France.
authors: Louise
authors: L
authors: Benoit
authors: Cardiologie congénitale, Strasbourg, France.
authors: Jelena
authors: J
authors: Radojevic
authors: Cardiologie Pédiatrique et Congénitale, Service de cardiologie, CHU Rennes, France.
authors: Adeline
authors: A
authors: Basquin
authors: Cardiology Department, CHU Clermont-Ferrand, France.
authors: Claire
authors: C
authors: Dauphin
authors: Congenital Heart Diseases Department, Marie Lannelongue Hospital, Plessis-Robinson, France. Inserm UMR 1048, équipe 8-I2MC-institut des maladies métaboliques et cardiovasculaires, Paul-Sabatier University, Toulouse, France.
authors: Sébastien
authors: S
authors: Hascoet
authors: Cardiology Department, Hôpital Pasteur, Université de Nice Sophia-Antipolis, Nice, France.
authors: Pamela
authors: P
authors: Moceri
authors: Pediatric and Congenital Cardiology Unit, M3C Regional Reference Center, Montpellier University Hospital, Montpellier, France.
authors: Charlene
authors: C
authors: Bredy
authors: Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C.|Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpital Européen Georges Pompidou and Necker Hospital, APHP, Paris Descartes University, Paris, France.
authors: Laurence
authors: L
authors: Iserin
authors: Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C.|Congenital Heart Diseases department, Marie Lannelongue Hospital, Plessis-Robinson, France.
authors: Marielle
authors: M
authors: Gouton
authors: Department of Obstetrics and Gynecology, Groupe hospitalier Pitié-Salpêtrière Paris, APHP, France.|Sorbonne Universités, UPMC Univ Paris 06, CNRS UMR 7222, INSERM U1150, Paris, France.
authors: Jacky
authors: J
authors: Nizard
mesh_terms: D000022:Abortion, Spontaneous / Q000209:etiology; D000328:Adult; D002585:Cesarean Section; D016208:Databases, Factual; D005260:Female; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D007234:Infant, Premature; D007236:Infant, Small for Gestational Age; D050498:Live Birth; D008428:Maternal Mortality; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D011256:Pregnancy Outcome; D018566:Pregnancy, High-Risk; D047928:Premature Birth / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult