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Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease
  1. Magalie Ladouceur1,2,3,
  2. Louise Benoit4,
  3. Jelena Radojevic5,
  4. Adeline Basquin6,
  5. Claire Dauphin7,
  6. Sébastien Hascoet8,
  7. Pamela Moceri9,
  8. Charlene Bredy10,
  9. Laurence Iserin1,2,
  10. Marielle Gouton1,11,
  11. Jacky Nizard4,12
  1. 1Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C
  2. 2Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpital Européen Georges Pompidou and Necker Hospital, APHP, Paris Descartes University, Paris, France
  3. 3Paris Cardiovascular Research Center (PARCC) INSERM U970, Paris, France
  4. 4Department of Obstetrics and Gynecology, Groupe hospitalier Pitié-Salpêtrière Paris, APHP, France
  5. 5Cardiologie congénitale, Strasbourg, France
  6. 6Cardiologie Pédiatrique et Congénitale, Service de cardiologie, CHU Rennes, France
  7. 7Cardiology Department, CHU Clermont-Ferrand, France
  8. 8Congenital 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
  9. 9Cardiology Department, Hôpital Pasteur, Université de Nice Sophia-Antipolis, Nice, France
  10. 10Pediatric and Congenital Cardiology Unit, M3C Regional Reference Center, Montpellier University Hospital, Montpellier, France
  11. 11Congenital Heart Diseases department, Marie Lannelongue Hospital, Plessis-Robinson, France
  12. 12Sorbonne Universités, UPMC Univ Paris 06, CNRS UMR 7222, INSERM U1150, Paris, France
  1. Correspondence to Dr Magalie Ladouceur, Adult Congenital Heart Disease Unit, Department of Cardiology, Hôpital Européen Georges Pompidou, 20 rue Leblanc, Paris 75015, France; magalie.ladouceur@


Objective 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.

Methods 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.

Results 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.

Conclusions 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.

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  • Twitter Follow Magalie Ladouceur at @gabapt20

  • Contributors Concept and design: ML and JN. Data analysis/interpretation: ML, LB, JN, JR, AB and CD. Drafting article: ML, JN and PM. Critical revision: CB, LI, MG, JR, PM and SH: Statistics: SH. Approval of article: all authors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval Review board of each institution participating in the study.

  • Provenance and peer review Not commissioned; externally peer reviewed.