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.