Role of sildenafil in a neonate with double outlet left ventricle and pulmonary hypertension

Double outlet left ventricle (DOLV) is a rare congenital heart defect where the aorta and the pulmonary trunk arise predominantly from the morphologic left ventricle. The clinical manifestations depend upon the location of the ventricular septal defect in relation to the great arteries, degree of pulmonary and aortic outflow tract obstruction, and other associated cardiac defects. We describe a neonate with DOLV, sub-aortic ventricular septal defect, and side-by-side great vessels who continued to require oxygen for desaturation to 60% in the neonatal period. His clinical symptoms were suggestive of persistent pulmonary hypertension, with 5-8% higher saturations in the leg compared to arm. He was started on oral sildenafil and his oxygen requirement decreased from 2 L/min to 0.1 L/min within 24 hours of initiating sildenafil. <Learning objective: The clinical manifestation of double outlet left ventricle mimic transposition of great vessels, tetrology of Fallot, or ventricular septal defect depending upon the cardiac lesion. The use of sildenafil has been expanded in the management of infants with non-cardiac as well as cardiac-related pulmonary hypertension. Sildenafil was successfully used in an infant with double outlet left ventricle, sub-aortic ventricular septal defect and rightward aortic root in the management of increased oxygen requirement attributed to pulmonary hypertension. However, close monitoring is required due to the risk of pulmonary over circulation.>