Three-Sinus Repair of Elastin Arteriopathy Associated Supravalvar Pulmonary Stenosis With Bilateral Branch Pulmonary Artery Involvement Ed Peng, FRCS(CTh),

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Three-Sinus Repair of Elastin Arteriopathy Associated Supravalvar Pulmonary Stenosis With Bilateral Branch Pulmonary Artery Involvement Ed Peng, FRCS(CTh), David N. Campbell, MD, James Jaggers, MD, Max B. Mitchell, MD The Annals of Thoracic Surgery Volume 100, Issue 2, Pages 741-743 (August 2015) DOI: 10.1016/j.athoracsur.2015.02.098 Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 (Left) Angiogram demonstrates supravalvar pulmonary stenosis with severe bilateral branch pulmonary artery (PA) disease in a 4-month-old child with sporadic elastin arteriopathy and suprasystemic right ventricular pressure. A blind ending ductus ampulla is a common feature in patients with bilateral branch PA involvement (arrow). (Middle) Divided pulmonary trunk demonstrates extreme thickening of the arterial wall (external diameter 10 mm, internal diameter 4 mm). (Right) Thickened arterial wall is thinned at the pulmonary artery bifurcation. The Annals of Thoracic Surgery 2015 100, 741-743DOI: (10.1016/j.athoracsur.2015.02.098) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 (Left) The thickened wall of each sinus is incised, and a shield-shape patch of pulmonary homograft is used to augment each sinus. (Right) Completion of three-sinus repair. The main PA is reconstructed with three separate patches to provide symmetrical augmentation. The Annals of Thoracic Surgery 2015 100, 741-743DOI: (10.1016/j.athoracsur.2015.02.098) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 In the most severe form of elastin arteriopathy, extensive bilateral branch PA arterioplasty is performed. Branch PA plasty is extended to or beyond the diseased artery into the lobar branches as needed. The upper lobar branches were augmented with separate patches to obtain better patch configuration. The Annals of Thoracic Surgery 2015 100, 741-743DOI: (10.1016/j.athoracsur.2015.02.098) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions