| Summary: | Abstract Background Pregnancy in patients with pulmonary arterial hypertension associated with congenital heart disease is associated with a high risk of maternal mortality. Case presentation An illustrative case study based on experience gained from management of patients through pregnancy is discussed. A 22-year-old female with a history of a closed patent ductus arteriosus in childhood had been diagnosed with significant pulmonary arterial hypertension at the age of 19. Her clinical condition had improved following the commencement of oral combination therapy with sildenafil 25 mg three times daily and macitentan 10 mg once daily. How should she be counselled regarding the risks of pregnancy and what are the most appropriate forms of contraception? What should the approach be if, despite adequate counselling, she subsequently becomes pregnant? Data to help guide decision-making are reviewed and a clinical approach is suggested. Conclusions Patients with pulmonary arterial hypertension should be advised against pregnancy and be given good contraceptive advice. If a patient with pulmonary arterial hypertension becomes pregnant and, after being fully counselled, chooses to proceed with their pregnancy, then they should be managed by an experienced multidisciplinary team.
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