Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology

Karen Sliwa; Iris M van Hagen; Werner Budts; Lorna Swan; Gianfranco Sinagra; Maryanne Caruana; Manuel Vazquez Blanco; Lodewijk J Wagenaar; Mark R Johnson; Gary Webb; Roger Hall; Jolien W Roos-Hesselink; ROPAC investigators

doi:10.1002/ejhf.594

Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology

Eur J Heart Fail. 2016 Sep;18(9):1119-28. doi: 10.1002/ejhf.594. Epub 2016 Jul 7.

Authors

Karen Sliwa^{1

2

3}, Iris M van Hagen⁴, Werner Budts⁵, Lorna Swan⁶, Gianfranco Sinagra⁷, Maryanne Caruana⁸, Manuel Vazquez Blanco⁹, Lodewijk J Wagenaar¹⁰, Mark R Johnson¹¹, Gary Webb¹², Roger Hall¹³, Jolien W Roos-Hesselink^{4

14}; ROPAC investigators

Affiliations

¹ Hatter Institute for Cardiovascular Research in Africa & IDM, Department of Medicine, Faculty of Health Sciences, University of Cape Town, South Africa.
² Soweto Cardiovascular Research Unit, University of the Witwatersrand, Johannesburg, South Africa.
³ Inter-Cape Heart Group, Medical Research Council, South Africa.
⁴ Department of Cardiology, Thoraxcentre, Erasmus Medical Centre, Rotterdam, the Netherlands.
⁵ Department of Cardiology, University Hospitals Leuven, Leuven, Belgium.
⁶ Adult Congenital Heart Disease Centre, The National Heart and Lung Institute, Imperial College London, Royal Brompton Hospital, London, UK.
⁷ Cardiovascular Department, Ospedali Riuniti and University of Trieste, Trieste, Italy.
⁸ Department of Cardiology, Mater Dei Hospital, Birkirkara Bypass, Malta.
⁹ Division of Cardiology, Hospital de Clinicas, University of Buenos Aires, Buenos Aires, Argentina.
¹⁰ Department of Cardiology, Thoraxcentrum Twente, Medisch Spectrum Twente, Enschede, the Netherlands.
¹¹ Department of Obstetrics, Imperial College School of Medicine, Chelsea and Westminster Hospital, London, UK.
¹² Cincinnati Adult Congenital Heart Disease Program, Cincinnati Children's Hospital Heart Institute, Cincinnati, OH, USA.
¹³ Department of Cardiology, Norwich Medical School, University of East Anglia, Norwich, UK.
¹⁴ European Society of Cardiology, Sophia Antipolis Cedex, France.

PMID: 27384461
DOI: 10.1002/ejhf.594

Abstract

Aims: To describe the outcomes of pregnancy in women with pulmonary hypertension.

Methods and results: In 2007 the European Registry on Pregnancy and Heart Disease was initiated by the European Society of Cardiology. Consecutive patients with all forms of cardiovascular disease, presenting with pregnancy, were enrolled with the aim of investigating the pregnancy outcomes. This subgroup of the cohort included 151 women with pulmonary hypertension (PH) either diagnosed by right heart catheterization or diagnosed as possible PH by echocardiographic signs, with 26% having pulmonary arterial hypertension (PAH), in three subgroups: idiopathic (iPAH), associated with congenital heart disease (CHD-PAH), or associated with other disease (oPAH), and 74% having PH caused by left heart disease (LHD-PH, n = 112). Maternal mean age was 29.2 ± 5.6 years and 37% were nulliparous. Right ventricular systolic pressure was <50 mmHg in 59.6% of patients, 50-70 mmHg in 28.5% and >70 mmHg in 11.9%. In more than 75% of patients, the diagnosis of PH had been made before pregnancy. Maternal death up to 1 week after delivery occurred in five patients (3.3%), with another two out of 78 patients who presented for follow-up (2.6%), dying within 6 months after delivery. The highest mortality was found in iPAH (3/7, 43%). During pregnancy, heart failure occurred in 27%. Caesarean section was performed in 63.4% (23.9% as emergency). Therapeutic abortion was performed in 4.0%. Complications included miscarriage (5.6%), fetal mortality (2%), premature delivery (21.7%), low birth weight (19.0%), and neonatal mortality (0.7%).

Conclusion: Mortality in this group of patients with various forms of PH was lower than previously reported as specialized care during pregnancy and delivery was available. However, maternal and fetal mortality remains prohibitively high in women with iPAH, although this conclusion is restricted by limited numbers. Early advice on contraception, pregnancy risk and fetal outcome remains paramount.

Keywords: Congenital heart disease; Left heart disease; Management; Rheumatic heart disease.

MeSH terms

Abortion, Spontaneous / epidemiology
Abortion, Therapeutic / statistics & numerical data
Adult
Cesarean Section / statistics & numerical data*
Female
Fetal Mortality
Gestational Age
Heart Defects, Congenital / complications
Heart Failure / epidemiology*
Humans
Hypertension, Pulmonary / epidemiology*
Hypertension, Pulmonary / etiology
Infant
Infant Mortality
Infant, Low Birth Weight
Infant, Newborn
Maternal Mortality
Pregnancy
Pregnancy Complications, Cardiovascular / epidemiology*
Premature Birth / epidemiology
Registries*
Young Adult