Home-inhaled nitric oxide therapy was effective and feasible in the patients with intractable pulmonary arterial hypertension. We present the case of a child with severe pulmonary arterial hypertension associated with post-operative pulmonary venous obstruction who was treated with home-inhaled nitric oxide therapy.

Post-operative pulmonary venous stenosis is a poor prognostic factor in single-ventricle haemodynamics. Implantation of a drug-eluting stent is a therapeutic option. However, due to their small size, they inevitably become inadequate as the patient grows. We present the first case, to the best of our knowledge, of the replacement of a small-diameter stent with a large-diameter stent during Fontan surgery.

We describe a two-year-old boy suffering from pulmonary hypertension due to pulmonary venous stenosis. Previous reports suggest that it is not easy to diagnose this entity during life, and most cases are diagnosed at operation or autopsy. Our patient was diagnosed relatively early in life and treated successfully by pericardial patch venoplasty. Histologic examination of the lung biopsy performed at operation was considered to be useftil prognostically. The minimal pulmonary vascular changes noted were consistent with a good outcome after the surgical repair.