Please cite this paper as: Kiely D, Condliffe R, Webster V, Mills G, Wrench I, Gandhi S, Selby K, Armstrong I, Martin L, Howarth E, Bu’Lock F, Stewart P, Elliot C. Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach. BJOG 2010;117:565–574.

Objective Pregnancy in women with pulmonary hypertension (PH) is reported to carry a maternal mortality rate of 30–56%. We report our experience of the management of pregnancies using a strategy of early introduction of targeted pulmonary vascular therapy and early planned delivery under regional anaesthesia.

Design Retrospective observational study.

Setting Specialist quaternary referral pulmonary vascular unit.

Population Nine women with PH who chose to proceed with ten pregnancies.

Methods A retrospective review of the management of all women who chose to continue with their pregnancy in our unit during 2002–2009.

Main outcome measures Maternal and fetal survival.

Results All women commenced nebulised targeted therapy at 8–34 weeks of gestation. Four women required additional treatment or conversion to intravenous prostanoid therapy. All women were delivered between 26 and 37 weeks of gestation. Delivery was by planned caesarean section in nine cases. All women received regional anaesthesia and were monitored during the peripartum period in a critical care setting. There was no maternal mortality during pregnancy and all infants were free from congenital abnormalities. One woman died 4 weeks after delivery following patient-initiated discontinuation of therapy. All remaining women and infants were alive after a median of 3.2 years (range, 0.8–6.5 years) of follow-up.

Conclusion Although the risk of mortality in pregnant women with PH remains significant, we describe improved outcomes in fully counselled women who chose to continue with pregnancy and were managed with a tailored multiprofessional approach involving early introduction of targeted therapy, early planned delivery and regional anaesthetic techniques.

References

1 Simmoneau G, Robbins I, Beghetti M, Channick R, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–54.
10.1016/j.jacc.2009.04.012
PubMedWeb of Science®Google Scholar
2 McLaughlin VV, Archer SL, Badesch D, Barst R, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation 2009; 119: 2250–94.
10.1161/CIRCULATIONAHA.109.192230
PubMedWeb of Science®Google Scholar
3 Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113(16): 2011–20.
10.1161/CIRCULATIONAHA.105.602565
PubMedWeb of Science®Google Scholar
4 Yentis SM, Steer PJ, Plaat F. Eisenmenger’s syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol 1998; 105: 921–2.
10.1111/j.1471-0528.1998.tb10240.x
CASPubMedWeb of Science®Google Scholar
5 Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Cardiol 1998; 31: 1650–7.
10.1016/S0735-1097(98)00162-4
CASPubMedWeb of Science®Google Scholar
6 Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jais X, Humbert M, et al. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 2005; 102: 1133–7.
10.1097/00000542-200506000-00012
PubMedWeb of Science®Google Scholar
7 National Pulmonary Hypertension Centres of the UK and Ireland. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart 2008; 94: S1–41.
10.1136/hrt.2007.132118
CASPubMedWeb of Science®Google Scholar
8 Bedard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30: 256–65.
10.1093/eurheartj/ehn597
PubMedWeb of Science®Google Scholar
9 Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425–36.
10.1056/NEJMra040291
CASPubMedWeb of Science®Google Scholar
10 Galie N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30: 394–403.
10.1093/eurheartj/ehp022
PubMedWeb of Science®Google Scholar
11 Condliffe R, Kiely DG, Gibbs JSR, Corris PA, Peacock AJ, Jenkins DP, et al. Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2008; 177: 1122–7.
10.1164/rccm.200712-1841OC
PubMedWeb of Science®Google Scholar
12 Oldfield V, Lyseng-Williamson KA. Bosentan: a review of its use in pulmonary arterial hypertension and systemic sclerosis. Am J Cardiovasc Drugs 2006; 6: 189–208.
10.2165/00129784-200606030-00006
CASPubMedGoogle Scholar
13 Easterling TR, Ralph DD, Schmucker BC. Pulmonary hypertension in pregnancy: treatment with pulmonary vasodilators. Obstet Gynecol 1999; 93: 494–8.
10.1016/S0029-7844(98)00524-9
CASPubMedWeb of Science®Google Scholar
14 Nahapetian A, Oudiz RJ. Serial hemodynamics and complications of pregnancy in severe pulmonary arterial hypertension. Cardiology 2008; 109: 237–40.
10.1159/000107786
PubMedWeb of Science®Google Scholar
15 Stewart R, Tuazon D, Olson G, Duarte AG. Pregnancy and primary pulmonary hypertension : successful outcome with epoprostenol therapy. Chest 2001; 119: 973–5.
10.1378/chest.119.3.973
CASPubMedWeb of Science®Google Scholar
16 Bendayan D, Hod M, Oron G, Sagie A, Eidelman L, Shirit D, et al. Pregnancy outcome in patients with pulmonary arterial hypertension receiving prostacyclin therapy. Obstet Gynecol 2005; 106: 1206–10.
10.1097/01.AOG.0000164074.64137.f1
PubMedWeb of Science®Google Scholar
17 Lynch TD, Laffey JG. Sildenafil for pulmonary hypertension in pregnancy? Anesthesiology 2006; 104: 382.
10.1097/00000542-200602000-00037
PubMedWeb of Science®Google Scholar
18 Monnery L, Nanson J, Charlton G. Primary pulmonary hypertension in pregnancy; a role for novel vasodilators. Br J Anaesth 2001; 87: 295–8.
10.1093/bja/87.2.295
CASPubMedWeb of Science®Google Scholar
19 Hill LL, De Wet CJ, Jacobsohn E, Leighton BL, Tymkew H. Peripartum substitution of inhaled for intravenous prostacyclin in a patient with primary pulmonary hypertension. Anesthesiology 2004; 100: 1603–5.
10.1097/00000542-200406000-00037
PubMedWeb of Science®Google Scholar
20 Elliot CA, Stewart P, Webster VJ, Mills GH, Hutchinson SP, Howarth ES, et al. The use of iloprost in early pregnancy in patients with pulmonary arterial hypertension. Eur Respir J 2005; 26: 168–73.
10.1183/09031936.05.00128504
CASPubMedWeb of Science®Google Scholar
21 Badesch DB, Champion HC, Sanchez MAG, Hoeper MM, Loyd JE, Manes A, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: S55–66.
10.1016/j.jacc.2009.04.011
PubMedWeb of Science®Google Scholar
22 Thorne S, Nelson-Piercy C, MacGregor A, Gibbs S, Crowhurst J, Panay N, et al. Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006; 32: 75–81.
10.1783/147118906776276486
PubMedWeb of Science®Google Scholar
23 Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322–9.
10.1056/NEJMoa020204
CASPubMedWeb of Science®Google Scholar
24 Hoeper MM, Gall H, Seyfarth HJ, Halank M, Ghofrani HA, Winkler J, et al. Long-term outcome with intravenous iloprost in pulmonary arterial hypertension. Eur Respir J 2009; 34: 132–7.
10.1183/09031936.00130408
CASPubMedWeb of Science®Google Scholar
25 Kenyon KW, Nappi JM. Bosentan for the treatment of pulmonary arterial hypertension. Ann Pharmacother 2003; 37: 1055–62.
10.1345/aph.1C256
CASPubMedWeb of Science®Google Scholar
26 Barash PG, Nardi D, Hammond G, Walker-Smith G, Capuano D, Laks H, et al. Catheter-induced pulmonary artery perforation. Mechanisms, management and modifications. J Thorac Cardiovasc Surg 1981; 82: 5–12.
CASPubMedWeb of Science®Google Scholar
27 Cheek TG, Gutsche BB. Maternal physiologic alteration during pregnancy. In: SC Hughes, G Levinson, MA Rosen, editors. Shnider and Levinson’s Anesthesia for Obstetrics. Philadelphia, PA: Lippincott Williams & Wilkins; 2000. pp. 3–18.
Google Scholar
28 Kiely DG, Elliot CA, Webster VJ, Stewart P. Pregnancy and pulmonary hypertension: new approaches to the management of a life threatening condition. In: PJ Steer, MA Gatzoulis, P Baker, editors. Heart Disease and Pregnancy. London: RCOG Press; 2006. pp. 211–29.
Google Scholar
29 Kramer MS, Demissie K, Yang H, Platt RW, Sauve R, Liston R. The contribution of mild and moderate preterm birth to infant mortality. J Am Med Assoc 2000; 284: 843–9.
10.1001/jama.284.7.843
CASPubMedWeb of Science®Google Scholar
30 Huddy CL, Johnson A, Hope PL. Educational and behavioural problems in babies of 32–35 weeks gestation. Arch Dis Child 2001; 85: 23–8.
10.1136/fn.85.1.F23
PubMedWeb of Science®Google Scholar
31 Wang ML, Dorer DJ, Fleming MP, Catlin EA. Clinical outcomes of near-term infants. Pediatrics 2004; 114: 372–6.
10.1542/peds.114.2.372
PubMedWeb of Science®Google Scholar
32 van Oppen AC, Stigter RH, Bruinse HW. Cardiac output in normal pregnancy: a critical review. Obstet Gynecol 1996; 87: 310–8.
10.1016/0029-7844(95)00348-7
CASPubMedWeb of Science®Google Scholar
33 Weiss BM, Hess OM. Pulmonary vascular disease and pregnancy: current controversies, management strategies and perspectives. Eur Heart J 2000; 21: 104–15.
10.1053/euhj.1999.1701
CASPubMedWeb of Science®Google Scholar
34 Hunter S, Robson SC. Adaptation of the maternal heart in pregnancy. Br Heart J 1992; 68: 540–3.
10.1136/hrt.68.12.540
CASPubMedWeb of Science®Google Scholar
35 O’Hare R, McLoughlin C, Milligan K, McNamee D, Sidhu H. Anaesthesia for caesarean section in the presence of severe primary pulmonary hypertension. Br J Anaesth 1998; 81: 790–2.
10.1093/bja/81.5.790
CASPubMedWeb of Science®Google Scholar

Citing Literature

Volume117, Issue5

April 2010

Pages 565-574

Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach

Abstract

References

Citing Literature

References

Information

About Wiley Online Library

Help & Support

Opportunities

Connect with Wiley

Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach

Abstract

References

Citing Literature

References

Related

Information