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Management of pregnancy in women with pulmonary hypertension secondary to SLE and anti-phospholipid syndrome

W L Martin

J Waugh

Department of Fetal Medicine, Birmingham Women’ Hospital, Birmingham, UK

I Rushton

Department of Pathology, Birmingham Women's Hospital, Birmingham, UK

M Lewis

Department of Anaesthesia, Birmingham Women's Hospital, Birmingham, UK

T Clutton-Brock

Department of Anaesthesia and Intensive Care, Queen Elizabeth Hospital, Birmingham, UK

J N Townend

Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK

M D Kilby

Department of Fetal Medicine, Birmingham Women’ Hospital, Birmingham, UK

C Gordon

Department of Rheumatology, Division of Immunity and Infection, University of Birmingham, Edgbaston, Birmingham, B15 2TT, UK. p.c.gordon{at}bham.ac.uk

Pulmonary hypertension is found in about 10% of patients with systemic lupus erythematosis (SLE). Pulmonary hypertension may be present at the time of diagnosis or may develop after the diagnosis of SLE or anti-phospholipid syndrome (APS). It often presents in the reproductive years and has a significant impact on pregnancy outcome, being a significant cause of indirect maternal deaths. In our observationalcase series of three patients there were two deaths (66%). In cases 1 and 2 the pulmonary hypertension developed during pregnancy and deteriorated rapidly with markedly abnormal mean pulmonary artery pressures of 80 and 70 mmHg respectively prior to death. Both patients died within 48 hours of delivery. In case 3 the pulmonary hypertensionwas milder and was diagnosed very early in pregnancy. The patient received multidisciplinary care from the first trimester and the management of the pregnancy, delivery and the early puerperium was planned. Careful epidural anaesthesia was used and the patient had invasive monitoring on the intensive therapy unit (ITU) for 72 hours. Women with pulmonary hypertensionneed to be aware of the high risk of maternal mortality associated with pregnancy but we believe that an improvement in outcome can be achieved by careful assessment and the use of a multidisciplinary approach from early in pregnancy.

Key Words: anti-phospholipid syndrome • lupus • pregnancy • pulmonary hypertension • therapy

Lupus, Vol. 11, No. 6, 392-398 (2002)
DOI: 10.1191/0961203302lu216xx


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