Home inhaled nitric oxide therapy in a child with pulmonary arterial hypertension associated with pulmonary venous obstruction

Abstract We present the case of a child with severe pulmonary arterial hypertension associated with postoperative pulmonary venous obstruction who were treated with home inhaled nitric oxide therapy. The patient underwent corrective surgery at the age of 7 days; however, he developed postoperative pulmonary venous obstruction. Cardiac catheterization at the age of 2 months showed that mean pulmonary venous pressure and pulmonary vascular resistance were 53 mmHg and 9.0 Wood unit m2, respectively. He underwent pulmonary venous reconstruction, but it resulted in recurrent pulmonary venous obstruction. Subsequently, he underwent stent implantation in the pulmonary vein. Histopathological findings of the lung specimen showed that intimal hyperplasia in the pulmonary arteries and arterialization in the pulmonary veins, suggesting intractable pulmonary arterial hypertension. Inhaled nitric oxide decreased mean pulmonary arterial pressure from 81 mmHg to 51 mmHg. Despite the introduction of pulmonary vasodilators including tadalafil and bosentan, inhaled nitric oxide could not discontinue. We implemented home inhaled nitric oxide therapy, which allowed the patient to be discharged for home-care medication. Our case suggested that home inhaled nitric oxide therapy was feasible in a child with intractable pulmonary arterial hypertension who were expected to be poor outcome.