John L. Colquitt, MD, Shaine A. Morris, MD, MPH, Susan W

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Survival in Children With Down Syndrome Undergoing Single-Ventricle Palliation John L. Colquitt, MD, Shaine A. Morris, MD, MPH, Susan W. Denfield, MD, Charles D. Fraser, MD, Yunfei Wang, PhD, W. Buck Kyle, MD The Annals of Thoracic Surgery Volume 101, Issue 5, Pages 1834-1841 (May 2016) DOI: 10.1016/j.athoracsur.2015.11.047 Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Management and outcomes in children with Down syndrome and FSV. 1Included 1 Norwood arch reconstruction. 2One patient still has PAB; SCPC delayed because of PVR = 10; group also includes five arch repairs. 3Attempted biventricular repair was unsuccessful and was taken down to PAB with subsequent demise. 4Takedown performed for failing SCPC physiology; 1 of 2 patients died. 5Developed progressive cyanosis/exercise intolerance many years after PAB; could not septate, instead underwent palliative shunting. *Three patients who underwent SCPC are considered candidates for and await TCPC; if successful this percentage would increase to 32%. (BiV = biventricular; BT = Blalock-Taussig; FSV = functional single ventricle; PAB = pulmonary artery band; SCPC = superior cavopulmonary connection; TCPC = total cavopulmonary connection.) The Annals of Thoracic Surgery 2016 101, 1834-1841DOI: (10.1016/j.athoracsur.2015.11.047) Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Receiver operating characteristic curve for PVR in the first year of life and death/cardiac transplantation. A value of ≥3 WUm2 for PVR had a sensitivity of 86% and specificity of 74%. (AUC = area under the curve; PVR = pulmonary vascular resistance; WUm2 = Wood unit × m2.) The Annals of Thoracic Surgery 2016 101, 1834-1841DOI: (10.1016/j.athoracsur.2015.11.047) Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 Kaplan-Meier survival in patients (A) with and (B) without Down syndrome with single-ventricle anatomy who had pulmonary vascular resistance measured in the first year of life, stratified by pulmonary vascular resistance ≥3 WUm2. (PVR = pulmonary vascular resistance; WUm2 = Wood unit × m2.) The Annals of Thoracic Surgery 2016 101, 1834-1841DOI: (10.1016/j.athoracsur.2015.11.047) Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions