Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries

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Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries Sergio A. Carrillo, MD, Richard D. Mainwaring, MD, William L. Patrick, BS, Holly D. Bauser-Heaton, MD, PhD, Lynn Peng, MD, V. Mohan Reddy, MD, Frank L. Hanley, MD The Annals of Thoracic Surgery Volume 100, Issue 2, Pages 606-614 (August 2015) DOI: 10.1016/j.athoracsur.2015.03.110 Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Preoperative angiogram of a typical patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) and absence of intrapericardial central pulmonary arteries. The angiogram demonstrates two MAPCAs to the right lung (A, B) and one large MAPCA supplying the entire left lung (C). There is no filling of a central pulmonary artery on any of the injections. The absence of central branch pulmonary arteries was subsequently confirmed at surgery. This patient was able to undergo a single-stage complete repair. The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Preoperative cardiac catheterization of a second patient with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) and absent intrapericardial central pulmonary arteries. The angiograms demonstrate a total of 5 MAPCAs, with three to the right lung (A, B, C) and two to the left lung (D, E). This patient underwent a single-stage complete repair at 8 months of age. The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 3 (A) Preoperative angiogram of 1 of the 7 patients who underwent complete unifocalization of their major aortopulmonary collaterals (MAPCAs) but failed the intraoperative flow study and therefore did not undergo complete repair at this operation. The specific patient shown had four MAPCAs (a, b, c, d), with one (c) giving branches to both the right and left sides (so, effectively, three MAPCA branches to the right and two to the left). The angiogram demonstrates the absence of intrapericardial central pulmonary arteries, and that was confirmed at surgery. (B) Angiogram of the same patient after complete unifocalization of the MAPCAs to a central shunt. The two figures demonstrate the separate injections into the right (a) and left (b) lungs. This patient subsequently underwent completion of the repair with closure of the ventricular septal defect and insertion of a right ventricle to reconstructed pulmonary conduit. The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 4 Hemodynamic data for the 28 patients who underwent single-stage complete repair. The average right ventricle (RV) to aortic (Ao) pressure ratio was 0.33 ± 0.07. The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 5 Flow chart demonstrating the results for the 35 patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals and absent intrapericardial central pulmonary arteries. The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions

Fig 6 Actuarial analysis comparing the 28 patients who were able to undergo single-stage complete repair (heavy solid line) versus the 7 patients who underwent unifocalization of major aortopulmonary collaterals to a central shunt (dashed line). (Lighter solid line = overall.) The Annals of Thoracic Surgery 2015 100, 606-614DOI: (10.1016/j.athoracsur.2015.03.110) Copyright © 2015 The Society of Thoracic Surgeons Terms and Conditions