Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified.

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Presentation transcript:

Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation Doff B. McElhinney, MD, Gerald R. Marx, MD, Audrey C. Marshall, MD, John E. Mayer, MD, Pedro J. del Nido, MD The Journal of Thoracic and Cardiovascular Surgery Volume 141, Issue 6, Pages 1362-1370.e1 (June 2011) DOI: 10.1016/j.jtcvs.2010.08.088 Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 In patient 1, the HV were baffled through a left lateral tunnel to the left PA after prior BCPA, and A) HV flow streamed to the left lung. A small amount of contrast can be seen filling but not passing across the hypoplastic central PA segment (arrow). B, There were no PAVMs in the left lung, but there were dense and advanced PAVMs throughout the right lung (C). Central PA stenting facilitated HV flow to the right lung, but there was no improvement in hypoxemia. L, Left PA; HV, hepatic venous; R, right PA. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 Patient 2 underwent total cavopulmonary connection without prior BCPA. A, HV flow flowed exclusively to the right lung, where there were no PAVMs. B, The azygous vein entered the left SVC (arrow indicates catheter passing from the azygous vein through the left SVC to the PAs), which was connected to the PAs leftward of the HV-PA connection. C, There were diffuse left lung PAVMs, which (D) progressed over time and after placement of a central PA stent (arrows). C, HV-PA conduit; R, right PA; HV, hepatic veins; L, left PA. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 3 Patient 5 underwent Fontan completion by connection of the HV to the right PA with a 16-mm ePTFE conduit, which resulted in (A) streaming of HV flow exclusively to the right lung. B, The azygous vein connected to the left-sided SVC, and there were unilateral left lung PAVMs. C, D, There was also a duplicated IVC system with a connection between the HV and right-sided suprarenal IVC and between the IVC and azygous vein at the level of the renal veins (arrow). D, After completion of the modified Fontan circulation, there was no flow from the HV across this connecting vein and a small amount of flow from the azygous into the suprarenal right IVC. E, After occlusion of the HV-PA conduit with a vascular plug (arrows), HV flow passed down the suprarenal IVC, across the connecting vein into the azygous vein. F, After this reconfiguration, left lung PAVMs resolved and systemic oxygen saturation increased to 97%. C, HV-PA conduit; Az, azygous vein; L, left PA; V, IVC; HV, hepatic veins. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 4 Patient 8 had a left-sided HV-PA conduit placed 6 years after bilateral BCPA. The right hepatic veins continued to drain to the atrium, and intrahepatic collaterals (∗) enlarged, allowing retrograde flow from the cavopulmonary circulation through HV-PA conduit, through the liver, and into the atrium. A, The HV-atrial connection was closed with an occlusion device (arrow), but (B) streaming of HV flow to the left lung continued, and (C) right lung PAVMs persisted and progressed to a dense, advanced state. Reconfiguration was performed by placing an additional ePTFE conduit from the right HV to the right PA. RH, Right HV; L, left PA; C, HV-PA conduit; R, right PA. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 5 Post-modification computed tomography images in patients 3 (A–C) and 6 (D–F). Images in patient 3 demonstrate the HV-azygous vein conduit (C) in parasagittal (A) and paraxial (B) planes. A, C, Arrows indicate clips that were used to ligate the inferior portion of the HV-PA conduit (PC) above the anastomosis of the HV-azygous vein conduit. The images in patient 6 demonstrate the connection between the HV-azygous vein conduit (C) and the prior HV-PA conduit (O) in parasagittal (D) and axial (E) planes. In the coronal image (F), the midline connection between right-sided hemiazygous and left-sided azygous veins is depicted with asterisks (∗∗); the HV-azygous vein conduit can be seen near its entrance to the hemiazygous. Az, Azygous vein; C, HV-azygous vein conduit; HV, hepatic veins; Ao, descending aorta; PC, HV-PA conduit; O, HV-PA conduit; H, hemiazygous vein; L, liver. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Figure 6 Schematic diagrams of HV and PA flow-streaming and interactions in several representative forms of total cavopulmonary connection in patients with IVC interruption and direct atrial drainage of the HV. Solid arrows represent the distribution of azygous and SVC return, and dashed arrows represent the distribution of HV return. A, There is contralateral offset of the single SVC-PA connection and the HV-PA connections, such that all HV return is to a single lung. B, The single SVC and HV connect to the PAs without lateral offset, and there is HV flow to both lungs. C, There are bilateral SVCs and connection of the HVs to the PA in proximity to the non-dominant SVC, which favors predominantly unilateral HV flow. D, With HV-azygous connection, HV blood will enter the dominant SVC, which will provide well more than half of systemic venous inflow to the PAs. Flow from the HVs will distribute in a similar fashion to azygous and SVC return, as depicted by the superimposed solid and dashed arrows. SVC, Superior vena cava; PA, pulmonary artery; HV, hepatic venous. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions

Pike and colleagues17 (A) and Steinberg and colleagues28 (B) depicted placement of a conduit from the right-sided HV to a contralateral azygous vein. Kaneko and colleagues27 (C) connected widely spaced HVs, harvested with an atrial cuff, to the azygous vein with an autologous pericardial roll. Az, Azygous vein; H, hepatic veins; V, vagus nerve; E, esophagus; and P, pericardial roll. The Journal of Thoracic and Cardiovascular Surgery 2011 141, 1362-1370.e1DOI: (10.1016/j.jtcvs.2010.08.088) Copyright © 2011 The American Association for Thoracic Surgery Terms and Conditions