Oral sildenafil ameliorates impaired pulmonary circulation early after bidirectional cavopulmonary shunt.

We report a case of successful administration of oral sildenafil (ie, a phosphodiesterase-5 inhibitor) in an infant for impaired pulmonary circulation that caused early clinical deterioration after a bicavopulmonary shunt. The transpulmonary pressure gradient (ie, a clinical indicator of pulmonary circulation) was initially normalized by inhaled nitric oxide; however, an increase in transpulmonary pressure gradient and oxygen desaturation occurred after extubation and discontinuation of inhaled nitric oxide on postoperative day 1. Subsequent administration of oral sildenafil in stepwise doses resulted in normalization of transpulmonary pressure gradient and improved oxygen saturation with successful discontinuation of intravenous vasodilators. Our results suggest that oral sildenafil may be a potent adjunctive therapy for impaired postoperative pulmonary circulation after right heart bypass surgery.