Doubly Committed and Juxtaarterial Ventricular Septal Defect: Outcomes of the Aortic and Pulmonary Valves Paul J. Devlin, BA, Hyde M. Russell, MD, Michael.

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Doubly Committed and Juxtaarterial Ventricular Septal Defect: Outcomes of the Aortic and Pulmonary Valves Paul J. Devlin, BA, Hyde M. Russell, MD, Michael C. Mongé, MD, Angira Patel, MD, John M. Costello, MD, MPH, Diane E. Spicer, BS, Robert H. Anderson, MD, Carl L. Backer, MD The Annals of Thoracic Surgery Volume 97, Issue 6, Pages 2134-2141 (June 2014) DOI: 10.1016/j.athoracsur.2014.01.059 Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 (A) In the normal heart, the free-standing muscular subpulmonary infundibulum lifts the pulmonary trunk away from the base of the heart. The leaflets of the aortic and pulmonary valves are separated, and are at different levels. (B) This subpulmonary infundibular sleeve is not present in the heart with the doubly committed juxtaarterial ventricular septal defect, pictured at right, a feature that brings the aortic and pulmonary valves into alignment, and allows for the fibrous continuity between the leaflets of the two valves. The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Two images of doubly committed juxtaarterial ventricular septal defects (VSDs). (A) A doubly committed juxtaarterial VSD viewed from the right side of the heart. This shows the VSD located superior to the limbs of the septal band, and inferior to the conjoined leaflets of the pulmonary and aortic valves (yellow dotted line). Note the continuity between the caudal limb of the septal band and the ventriculoinfundibular fold, which produces a muscular posteroinferior rim to the defect. The prolapsed aortic valve is visualized through the defect. (B) A doubly committed juxtaarterial VSD viewed from the left side of the heart. Here one can appreciate its positioning just inferior to the aortic valve, and its proximity to the right coronary leaflet of the aortic valve. The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 Operative view of the doubly committed and juxtaarterial ventricular septal defect (VSD) after institution of cardiopulmonary bypass, aortic cross-clamping, and cardioplegic arrest. The exposure is through the longitudinally opened pulmonary trunk, showing the right coronary leaflet of the aortic valve prolapsing into the defect and partially occluding it. (Ao = aorta; PA = pulmonary artery; RV = right ventricle.) (Reprinted with permission from Mavroudis et al [23].) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 4 Interrupted pledget-supported sutures are placed circumferentially around the ventricular septal defect (VSD) and then through a polytetrafluoroethylene patch. Lateral projection showing the relationship of the sutures to the semilunar hinge of the leaflet of the pulmonary valve. These sutures are placed through the base of the pulmonary valvar leaflets where there is absence of a fibrous outlet septum. (Reprinted with permission from Mavroudis et al [23].) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 5 Degree of aortic valvar insufficiency preoperatively (Preop), immediately postoperatively (Postop), and on late follow-up. (Blue bars = zero; dark blue bars = mild; light blue bars = severe; orange bars = moderate; red bars = trivial.) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 6 Degree of aortic insufficiency present preoperatively (Preop), immediately postoperatively (Postop), and on late follow-up for individual patients. Each line represents 1 patient; some patient lines overlap. On follow-up, which had a mean time of 4.9 years, no patients had any more than mild aortic insufficiency. Dashed line indicates patient who underwent concurrent aortic valve repair and follow-up aortic valve replacement. Dotted line indicates patient who underwent concurrent aortic valve replacement. (Twenty-four patients who never had any degree of aortic insufficiency are not included in this graph.) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 7 Degree of pulmonary valvar insufficiency present preoperatively (Preop), postoperatively (Postop), and on late follow-up for individual patients. Each line represents 1 patient; some patients' lines overlap. On follow-up, which had a mean time of 4.9 years, no patient had any more than mild pulmonary valvar insufficiency. (Twenty-five patients who never had any degree of pulmonary valve insufficiency are not included in this graph.) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions

Fig 8 Pathophysiology of aortic valvar insufficiency in juxtaarterial and doubly committed ventricular septal defect (VSD). (A, C, E) In early systole, blood ejected from the left ventricle is shunted through the VSD, forming a high velocity, low pressure shunt. As a result, the anatomically unsupported right coronary leaflet, due to the loss of septal support, is pulled into the right ventricle by the Venturi effect (essentially being sucked into the low pressure zone within the defect). (B, D, F) In diastole, the intraaortic pressure forces the aortic valvar leaflet to close, but the unsupported prolapsed leaflet is pushed down into the VSD, away from the opposed coronary leaflet, resulting eventually in aortic insufficiency. (Reprinted from Tweddell et al [13], with permission from Elsevier.) The Annals of Thoracic Surgery 2014 97, 2134-2141DOI: (10.1016/j.athoracsur.2014.01.059) Copyright © 2014 The Society of Thoracic Surgeons Terms and Conditions