Pulmonary vein stenosis (PVS) is a progressive disease with pulmonary hypertension (PH) as a major cause of morbidity and mortality. Traditional management of PH with inhaled nitric oxide (iNO) is typically avoided in PVS patients because, while iNO may reduce pulmonary vascular resistance, PH persists as pulmonary blood flow increases in the presence of a downstream resistive lesion. We report 3 cases with primary PVS and PH in which iNO was used to successfully decrease mean pulmonary artery pressures with clinical improvement. Based on this experience, we suggest that iNO can be used to treat PH in select patients with PVS.
From the Department of Cardiac Anesthesia, Boston Children’s Hospital, Boston, Massachusetts.
Accepted for publication July 27, 2018
The authors declare no conflicts of interest.
Address correspondence to Morgan L. Brown, MD, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02446. Address e-mail to Morgan.firstname.lastname@example.org.