Epoprostenol treatment for idiopathic pulmonary arterial hypertension in pregnancy

Abstract

Idiopathic pulmonary arterial hypertension is a rare condition associated with significant maternal mortality. We report the management of a 37-year-old multigravida with severe disease using epoprostenol, a multidisciplinary approach, and a planned delivery. Although the patient survived the pregnancy, her pulmonary function significantly worsened. Epoprostenol, a pulmonary vasodilator, should be considered when indicated during pregnancy. Neither fetal nor neonatal harm was identified.

Pregnancy and Pulmonary Arterial Hypertension

“…General anesthesia has been used successfully in the delivery of patients with PAH (Garabedian 2010, Bonnin 2005; however, general anesthesia may be associated with pulmonary vasoconstriction and other adverse hemodynamic changes associated with mechanical ventilation that may be deleterious in this patient population (Blaise 2003).…”

“…Warfarin use is contraindicated in pregnancy due to its known teratogenicity (pregnancy risk category X.) Both unfractionated heparin (Bédard 2009) and low molecular weight heparin (LMWH) (Kiely 2010, Goland 2010, Garabedian 2010 have been successfully used for thromboprophylaxis and treatment in pregnant PAH patients. If full dose anticoagulation is chosen, transition to prophylactic dosing should occur prior to delivery and may be resumed after delivery if there is no contraindication (Kiely 2010).…”

“…Based upon the current available literature, initiation of a prostacyclin with careful upward titration of the dose as tolerated is advisable (Bédard 2009, Kiely 2010, Garabedian 2010). The addition of oral sildenafil has been used successfully in the past and could be considered adjunctive therapy (Goland 2010, Kiely 2010).…”

“…Fetal monitoring and ultrasound biometry (Garabedian 2010, Kiely 2010 often is utilized to monitor fetal growth and development. If a fetal abnormality or growth restriction is suspected, further assessment is employed to guide management (Kiely 2010).…”

“…RHC is required for definitive confirmation of the diagnosis of PAH as well as the assessment of disease severity (McLaughlin 2009). A multidisciplinary team approach to the care of PAH in pregnancy has been utilized successfully (Garabedian 2010). This approach is associated with improved survival compared with historical data (Kiely 2010, Smedstad 1994.…”