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      Intravenous Epoprostenol for Management of Pulmonary Arterial Hypertension during Pregnancy

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          Abstract

          Background Pulmonary arterial hypertension carries a high risk of mortality in pregnancy. Recent advances in treatment may improve disease course and allow for successful management of the pregnancy.

          Case Report We present the case of a 20-year-old gravida 1, para 0 with diagnosis of severe primary pulmonary hypertension. The patient was managed with epoprostenol (prostacyclin) infusion via an indwelling catheter, which was initiated at 23 weeks' gestation. The dose was adjusted to the patient's symptoms and a successful vaginal delivery was achieved at 36 weeks' gestation. Although maternal postpartum course was uncomplicated, unexplained neonatal demise occurred at 11 days of life.

          Conclusion Successful management of pulmonary hypertension in pregnancy can be accomplished with a multidisciplinary approach and intensive therapy. Long-term effects of epoprostenol on fetal or neonatal well-being are unknown.

          Most cited references7

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          Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?

          Konstantinos Dimopoulos, James E. J. Bedard, Michael A Gatzoulis (2009)
          Pregnancy in women with pulmonary arterial hypertension (PAH) is considered to be associated with prohibitive maternal mortality. During the past decade, new advanced therapies for PAH have emerged and progress in high-risk pregnancy management has been made. We examined whether these changes have improved outcomes in parturients with PAH. A systematic review of all cases of parturients with idiopathic pulmonary hypertension (iPAH), congenital heart disease associated with PAH (CHD-PAH), or PAH of other aetiology (oPH) published in the past decade (1997-2007) was performed. Outcome data from this study were then compared with relevant data published between 1978 and 1996. Forty-eight case reports or case series met the inclusion criteria, totalling 73 parturients with PAH. Seventy-two per cent of patients with iPAH were receiving advanced therapies, compared with 52% of CHD-PAH and 47% of oPH. Although a publication bias cannot be excluded, overall maternal mortality was significantly lower compared with previous era (25 vs. 38%, P = 0.047) and was 17% in iPAH, 28% in CHD-PAH, and 33% in oPH. Seventy-eight per cent of deaths occurred within the first month after delivery. Primigravidae and parturients who received general anaesthesia were at higher risk of death (OR 3.70, 95% CI 1.15-12.5, P = 0.03 and OR 4.37, 95% CI 1.28-16.50, P = 0.02, respectively). Maternal mortality in parturients with PAH remains prohibitively high, despite lower death rates than previous decades. Early advice on pregnancy risks, including contraception, remains paramount. Women with PAH who become pregnant warrant a multidisciplinary approach with consideration of advanced therapies.
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            Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996.

            Branko Weiss, Lea Zemp, Burkhardt Seifert … (1998)
            Published reports were reviewed to evaluate the characteristics of peripartal management and the late pregnancy outcome in women with pulmonary vascular disease (PVD). Pulmonary hypertension poses one of the highest risks for maternal mortality, but actual data on the maternal and neonatal prognosis in this group are lacking. Reports published from 1978 through 1996 of Eisenmenger's syndrome (n = 73), primary pulmonary hypertension (PPH) (n = 27) and secondary vascular pulmonary hypertension (SVPH) (n = 25) complicating late pregnancy were included and analyzed using logistic regression analysis. Maternal mortality was 36% in Eisenmenger's syndrome, 30% in PPH and 56% (p < 0.08 vs. other two groups) in SVPH. Except for three prepartal deaths due to Eisenmenger's syndrome, all fatalities occurred within 35 days after delivery. Neonatal survival ranging from 87% to 89% was similar in the three groups. Previous pregnancies, timing of the diagnosis and hospital admission, operative delivery and diastolic pulmonary artery pressure were significant univariate (p < 0.05) maternal risk factors. Late diagnosis (p = 0.002, odds ratio 5.4) and late hospital admission (p = 0.01, odds ratio 1.1 per week of pregnancy) were independent predictive risk factors of maternal mortality. In the last two decades maternal mortality was comparable in patients with Eisenmenger's syndrome and PPH; however, it was relevantly higher in SVPH. Maternal prognosis depends on the early diagnosis of PVD, early hospital admission, individually tailored treatment during pregnancy and medical therapy and care focused on the postpartal period.
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              Pulmonary hypertension and pregnancy.

              Kathleen B. Madden (2009)
              Pulmonary hypertension is defined by a mean pulmonary artery pressure of greater than 25 mmHg at rest or 30 mmHg with exercise. It can occur in association with a variety of medical conditions. The most serious elevation in pulmonary artery pressures are seen in a group of conditions that share the histological entity of plexogenic pulmonary arteriopathy. Pulmonary hypertension may be missed or diagnosed late in the course of the illness. It is associated with a poor prognosis. Pulmonary hypertension carries a significant risk to mother and child during pregnancy and pregnant women with pulmonary hypertension require careful monitoring within the framework of a multidisciplinary team. Specific targeted therapy for pulmonary hypertension may be required during pregnancy. Many agents are contraindicated because of risks of teratogenicity or secretion into breast milk. The optimum mode of delivery is not clear but early input from the high-risk obstetric anaesthesia team is essential.
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                Author and article information

                Journal
                Journal ID (nlm-ta): AJP Rep
                Journal ID (iso-abbrev): AJP Rep
                Title: AJP Reports
                Publisher: Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                ISSN (Print): 2157-6998
                ISSN (Electronic): 2157-7005
                Publication date (Electronic): 18 March 2013
                Publication date (Print): October 2013
                Volume: 3
                Issue: 2
                Pages: 71-74
                Affiliations
                [1 ]Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, MedStar Washington Hospital Center, MedStar Health, Washington, District of Columbia
                [2 ]Department of Cardiology, MedStar Washington Hospital Center, MedStar Health, Washington, District of Columbia
                Author notes
                Address for correspondence Julia Timofeev, MD 110 Irving Street NW WashingtonDC 20010 julia.timofeev@ 123456medstar.net
                Article
                Publisher ID: 12r0048
                DOI: 10.1055/s-0033-1338169
                PMC ID: 3799704
                SO-VID: 89cb79bb-6574-4cb5-879f-dd13b330842d
                Copyright © © Thieme Medical Publishers
                History
                Date received : 21 December 2012
                Date accepted : 02 February 2013
                Categories
                Subject: Article

                Keywords: epoprostenol,pulmonary hypertension,prostaglandin,prostacyclin
                Data availability:
                Keywords: epoprostenol, pulmonary hypertension, prostaglandin, prostacyclin

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