ABSTRACT
Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.
Subject(s)
Heart Defects, Congenital , Pulmonary Veins , Scimitar Syndrome , Stenosis, Pulmonary Vein , Constriction, Pathologic , Humans , Infant , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Scimitar Syndrome/complications , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/surgery , Stenosis, Pulmonary Vein/diagnostic imaging , Stenosis, Pulmonary Vein/etiology , Stenosis, Pulmonary Vein/surgeryABSTRACT
Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.