Effects of inhaled nitric oxide on Fontan hemodynamics in adults.

The pulmonary vasculature plays a pivotal role in the nonpulsatile systemic venous return post-Fontan palliation. Elevated pulmonary vascular resistance index (PVRi) carries a worse prognosis post-Fontan, but the benefits of pulmonary vasodilators remain controversial. Moreover, the potential for worsening ventricular filling pressures with pulmonary vasodilation has been highlighted. We reviewed our experience with inhaled nitric oxide (iNO) administration during cardiac catheterization in 30 adults (age 32.7 ± 8.5 years) post-Fontan. The main findings of the study are: (1) iNO decreased pulmonary artery pressures, transpulmonary gradient, and PVRi without increasing pulmonary artery wedge pressure, (2) cardiac index was unchanged with iNO, and (3) different from acquired left heart disease, iNO did not result in further elevations in pulmonary artery wedge pressure in those with elevated ventricular filling pressures. iNO administration in adults post-Fontan was safe; whether baseline PVRi and response to iNO could be used to predict response to pulmonary vasodilators post-Fontan requires further investigation.