Patients with PAH should be counselled about the risks associated with pregnancy, and they
should be offered effective progesterone-only-based contraception because of the risk of thrombosis
[6]. The oral agent Bosentan also induces liver enzymes and reduces the efficacy of hormonal
contraception, and it has been associated with serious birth defects if taken during early pregnancy.
DepoProvera and the Mirena coil are unaffected, but the efficacy of Cerazette and Nexplanon may be
reduced. Therefore, patients taking this are advised to use two effective forms of contraception.
Whilst the use of pulmonary vasodilator therapy has been associated with improved maternal outcomes,
these agents all have a significant associated risk of teratogenicity, and therefore pregnancy
should still be discouraged.
Patients with PAH who become pregnant should be offered termination. Patients who wish to
continue with pregnancy should be managed in specialist pulmonary hypertension centres by a
multidisciplinary team. As the numbers of patients with successful outcomes remain small, there is
little literature to conclusively determine optimal management strategies, and therefore each patient
requires individual assessment and management according to local specialist experience. The general
principles of management are to deliver at around 35 weeks by caesarean section with monitoring in
intensive care for 2 weeks following delivery. The risk of mortality remains increased for 6 weeks after
delivery