Infantile Scimitar Syndrome With Contralateral Pulmonary Vein Stenosis and Refractory Pulmonary Hypertension

Abstract

Infantile scimitar syndrome is associated with pulmonary hypertension which can be difficult to manage. We present a three-month-old infant with scimitar syndrome, who eventually developed refractory pulmonary hypertension, posing a significant management challenge. Further workup demonstrated contralateral pulmonary vein stenosis, which is rarely described in scimitar syndrome. Our index case highlights the importance of follow-up cardiac catheterizations in these patients with severe pulmonary hypertension.