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Hybrid Stage 1 Palliation in a Patient With Hypoplastic Left Heart Syndrome and Unusual Decompressing Levoatrial Cardinal Vein in Turner Syndrome

A newborn with prenatal diagnosis of Turner syndrome with hypoplastic left heart syndrome (HLHS) was born at term at our institution without perinatal complications. Prostaglandin (PGE) infusion was started, and the initial transthoracic echocardiogram (TTE) confirmed HLHS with severe mitral and aortic valve stenosis. There was a restrictive atrial communication with a large tortuous, decompressing levoatrial cardinal vein (LACV) that drained in a very unusual fashion to the superior vena cava (Figure 1A and B and Video 1). Family wished to pursue every available option, despite known poor outcomes with traditional stage 1 Norwood palliation in the 1 Pediatric Cardiology, University of Florida, Gainesville, FL, USA 2 UF Health Congenital Heart Center, University of Florida College of Medicine, Gainesville, FL, USA 3 Department of Pediatrics, Congenital Heart Center, University of Florida, Gainesville, FL, USA