• D G Kiely, R Condliffe, V Webster, G H Mills, I Wrench, S V Gandhi, K Selby, I J Armstrong, L Martin, E S Howarth, F A Bu'lock, P Stewart, and C A Elliot.
• Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. david.kiely@sth.nhs.uk
• BJOG. 2010 Apr 1;117(5):565-74.

ObjectivePregnancy 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.DesignRetrospective observational study.SettingSpecialist quaternary referral pulmonary vascular unit.PopulationNine women with PH who chose to proceed with ten pregnancies.MethodsA retrospective review of the management of all women who chose to continue with their pregnancy in our unit during 2002-2009.Main Outcome MeasuresMaternal and fetal survival.ResultsAll 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.ConclusionAlthough 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.

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