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      Serial Hemodynamics and Complications of Pregnancy in Severe Pulmonary Arterial Hypertension

      case-report
      ,
      Cardiology
      S. Karger AG
      Pulmonary arterial hypertension, Pregnancy

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          Abstract

          We report a case of a woman who became pregnant after the diagnosis of moderate to severe pulmonary hypertension and underwent successful full-term pregnancy. Pulmonary hemodynamics were monitored before pregnancy and in the peripartum period. The patient was followed closely by the cardiology and high-risk obstetric specialists in the outpatient setting until she underwent c-section with epidural anesthesia. Outpatient medical management included twice daily subcutaneous enoxaparin and once daily amlodipine. Immediately prior to cesarian section, and for several days postoperatively, invasive hemodynamic monitoring was employed to titrate medical therapy. During delivery, strict attention focused on limiting intravenous fluids in order to avoid right ventricular volume overload. The postoperative course was complicated by a spontaneous, acute rise in pulmonary vascular resistance, which was managed with intravenous epoprostenol. In addition, abdominal bleeding, likely related to postoperative anticoagulation and platelet dysfunction, was controlled with transfusion and spontaneously resolved after discontinuing the anticoagulation. This case presents a favorable outcome in a pregnant patient undergoing cesarian section despite several complications related to pulmonary hypertension and right ventricular dysfunction, which are often fatal.

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          Most cited references14

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          Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996.

          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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            Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines.

            Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. This chapter will provide evidence-based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.
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              The use of iloprost in early pregnancy in patients with pulmonary arterial hypertension.

              In patients with pulmonary hypertension, pregnancy is associated with a high risk of maternal death. Such patients are counselled to avoid pregnancy, or if it occurs, are offered early interruption. Some patients, however, decide to continue with their pregnancy and others may present with symptoms for the first time whilst pregnant. Pulmonary vasodilator therapy provides a treatment option for these high-risk patients. The present study describes three patients with pulmonary arterial hypertension of various aetiologies who were treated with the prostacyclin analogue iloprost during pregnancy, and the post-partum period. Nebulised iloprost commenced as early as 8 weeks of gestation and patients were admitted to hospital between 24-36 weeks of gestation. All pregnancies were completed with a duration of between 25-36 weeks and all deliveries were by caesarean section under local anaesthetic. All patients delivered children free from congenital abnormalities, and there was no post-partum maternal or infant mortality. In conclusion, although pregnancy is strongly advised against in those with pulmonary hypertension, the current authors have achieved a successful outcome for mother and foetus with a multidisciplinary approach and targeted pulmonary vascular therapy.
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                Author and article information

                Journal
                CRD
                Cardiology
                10.1159/issn.0008-6312
                Cardiology
                S. Karger AG
                0008-6312
                1421-9751
                2008
                March 2008
                17 September 2007
                : 109
                : 4
                : 237-240
                Affiliations
                Division of Cardiology, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, Calif., USA
                Article
                107786 Cardiology 2008;109:237–240
                10.1159/000107786
                17873487
                30ca22a7-59bf-4988-baa1-d1569980dc04
                © 2007 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 08 February 2007
                : 10 February 2007
                Page count
                Tables: 1, References: 17, Pages: 4
                Categories
                Novel Insights from Clinical Experience

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                Pulmonary arterial hypertension,Pregnancy

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