Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary.

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Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries Jerome Soquet, MD, Matthew Liava'a, MBChB, Lucas Eastaugh, MBBS, Igor E. Konstantinov, MD, PhD, Johann Brink, MD, Christian P. Brizard, MD, Yves d'Udekem, MD, PhD The Annals of Thoracic Surgery Volume 103, Issue 5, Pages 1519-1526 (May 2017) DOI: 10.1016/j.athoracsur.2016.08.113 Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Rehabilitation procedures. (A) Primary central shunting with a Gore-Tex vascular graft (Laks technique). (B) RV-PA conduit as an intermediate rehabilitation procedure before repair, with a Gore-Tex Vascular Graft (left) or a Contegra valved conduit (right). (AO = aorta; PA = pulmonary artery.) Reproduced from Surgery of Conotruncal Anomalies, Pulmonary Atresia, Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries, 2016, pp 149-162, Yves d'Udekem and Lucas Jon Eastaugh, with permission of Springer. The Annals of Thoracic Surgery 2017 103, 1519-1526DOI: (10.1016/j.athoracsur.2016.08.113) Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Pulmonary arteries reconstruction. (A) Patch enlargement of the central pulmonary arteries with a Gore-Tex patch. (B) Replacement from hilum-to-hilum with a Gore-Tex vascular graft. Reproduced from Surgery of Conotruncal Anomalies, Pulmonary Atresia, Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries, 2016, pp 149-162, Yves d'Udekem and Lucas Jon Eastaugh, with permission of Springer. The Annals of Thoracic Surgery 2017 103, 1519-1526DOI: (10.1016/j.athoracsur.2016.08.113) Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 Outcomes of patients operated for pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collateral artery (PA/VSD/MAPCAs) from June 2003 to December 2014 in our institution. The Annals of Thoracic Surgery 2017 103, 1519-1526DOI: (10.1016/j.athoracsur.2016.08.113) Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions

Fig 4 Angiograms of a patient who underwent the rehabilitation pathway, demonstrating a progressive proximal stenosis of bilateral MAPCAs from the upper descending aorta (A, C, E, G) and a growth of the central pulmonary arteries (B, D, F, H). (A, B) at birth. (C, D) 4 months after shunting. (E, F) 3 years after a RV-PA conduit. (G, H) 5 years after repair and proximal pulmonary arteries patching. Asterisk marks left pulmonary artery with dual supply. The MAPCA indicated by an arrow was subsequently coil-embolized. The Annals of Thoracic Surgery 2017 103, 1519-1526DOI: (10.1016/j.athoracsur.2016.08.113) Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions

Fig 5 (A) Three-dimensional reconstruction of a CT scan at birth showing hypoplastic central pulmonary arteries before repetitive shunting procedures. (B) Angiogram of the same patient 1 year after repair and hilum-to-hilum replacement of the pulmonary arteries with a 14-mm Gore-Tex vascular graft. The Annals of Thoracic Surgery 2017 103, 1519-1526DOI: (10.1016/j.athoracsur.2016.08.113) Copyright © 2017 The Society of Thoracic Surgeons Terms and Conditions