Management of Eisenmenger Syndrome in Pregnancy With Sildenafil and l-arginine

In Brief BACKGROUND: Eisenmenger syndrome in pregnancy may be a life-threatening disease despite recent additions to the treatment options. CASE: We present a woman with severe pulmonary hypertension due to Eisenmenger syndrome treated during pregnancy and delivery and postpartum with l-arginine and sildenafil to enhance the nitric oxide pathway. This combination was associated with significant improvement in the mother's clinical and hemodynamic condition and fetal well-being. CONCLUSION: The concomitant use of sildenafil and l-arginine for the management of pulmonary hypertension in pregnancy, combined with multidisciplinary care, permitted a good outcome for the mother and her infant. Enhancement of the nitric oxide–cyclic guanine monophosphate pathway by sildenafil and l-arginine may constitute effective therapy for pulmonary hypertension in pregnancy.