A 32-Year-Old Pregnant Woman Presents With Worsening Dyspnea

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A pregnant woman previously prescribed a PDE-5 inhibitor for PAH and lost to follow-up presents with worsening dyspnea.
A pregnant woman previously prescribed a PDE-5 inhibitor for PAH and lost to follow-up presents with worsening dyspnea.

Case

A 32-year-old woman presented to the emergency department 6 months ago with complaints of dyspnea on exertion and near syncopal episodes (but no syncope). A bedside echocardiogram was performed and she was found to have a dilated right ventricle, with an estimated right ventricular systolic pressure of 67 mm Hg.

She was admitted for further management. She underwent right heart catheterization, which confirmed the diagnosis of pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure of 60 mm Hg. The patient was started on a phosphodiesterase-5 (PDE-5) inhibitor and was then discharged with plans for follow-up at a PH clinic.

She is lost to follow-up and then presents to see you at 12 weeks' gestation with worsening dyspnea. Risks for PAH and pregnancy, including maternal-fetal death, are discussed at length with the patient, but she elects to continue with the pregnancy.

What would be the best approach to treating PAH during her pregnancy and improving both maternal-fetal survival?

A. Discontinue the PDE-5 inhibitor and initiate therapy with warfarin and bosentan

B. Wean the patient from the PDE-5 inhibitor and initiate therapy with intravenous heparin and riociguat

C. Wean the patient from the PDE-5 inhibitor and start epoprostenol and intravenous heparin

D. Continue the PDE-5 inhibitor and start bosentan

E. Add a calcium channel blocker to her current therapy

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