From the University of California Sulpizio Cardiovascular Center, San Diego, California.

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1 Endovascular Stenting of the Superior Vena Cava-Right Atrial Junction in Combination With Laser Lead Extraction for Iatrogenic Superior Vena Cava Syndrome Mitul P. Patel, MD; Brian Kolski, MD; Ehtisham Mahmud, MD From the University of California Sulpizio Cardiovascular Center, San Diego, California. ABSTRACT: Iatrogenic superior vena cava (SVC) syndrome is a well described complication of pacemaker implantation leading to upper extremity swelling and symptoms of severe congestion from elevation in venous pressures, especially with obstruction of the SVC at the right atrial junction. We present a case of iatrogenic SVC syndrome that developed as a result of significant vascular fibrosis at the superior vena cava-right atrial junction due to multiple pacemaker leads. In a unique therapeutic approach, after laser lead extraction, endovascular stenting of the SVC-right atrial junction followed by lead replacement was performed using a hybrid surgical and endovascular approach. VASCULAR DISEASE MANAGEMENT 2014;11(6):E130-E135 Key words: endovascular therapy, superior vena cava, stenting Iatrogenic superior vena cava (SVC) syndrome is a well described complication of pacemaker implantation. 1 Patients with multiple transvenous pacemaker leads can develop upper-extremity swelling and symptoms of severe congestion from elevation in venous pressures, due to obstruction of the SVC and impaired emptying into the right atrium (RA). Endovascular and surgical treatment of iatrogenic SVC syndrome has also been described with multiple approaches to alleviate the obstruction into the RA. 2-4 We present a case of iatrogenic SVC syndrome that developed as a result of significant vascular fibrosis at the SVC-RA junction due to multiple pacemaker leads. Using a unique therapeutic approach, after initial laser lead extraction, endovascular stenting of the SVC-RA junction and lead replacement using a hybrid surgical and endovascular approach was performed. CASE DESCRIPTION A 47-year-old male presented with facial plethora and upper-extremity swelling. He was known to be pacemaker dependent for the previous 22 years due to a high-grade atrioventricular conduction block with original pacemaker leads placed in 1990 from the left subclavian vein approach. However, due to Vascular Disease Management June

2 tomography angiography revealed severe obstruction at the SVC-RA junction (Figure 1). Figure 1. Computed tomography demonstrating multiple pacemaker leads resulting in stenosis at the superior vena cava-right atrial junction. Figure 2. Fluoroscopic image demonstrating 12 Fr and 14 Fr laser lead extraction sheaths advanced over existing pacemaker leads. lead failure in 2003, he required placement of 2 new right-sided leads. As a result, he was known to have 2 nonfunctioning left subclavian leads and 2 functioning right subclavian pacemaker leads. Computed TREATMENT Due to nonfunctioning pacemaker leads and SVC syndrome, definitive therapy was determined to include laser lead extraction in combination with possible superior vena cava stenting and replacement of the patient s dual chamber pacemaker leads. The procedure was performed in a hybrid catheterization laboratory/ operating room with general anesthesia through both right- and left-sided chest surgical incisions. Initially the right sided atrial lead was extracted using a 12 Fr laser sheath (Spectranetics), and the right-sided ventricular lead with a 14 Fr laser sheath (Spectranetics) using multiple applications of laser energy (Figure 2). 5 The same technique was attempted to remove the left-sided leads, but severe calcification and fibrosis were encountered and ultimately overcome with a 13 Fr Evolution mechanical dilator (Cook Medical) with multiple alternating applications of mechanical dilations and rotations. After lead extraction, intraoperative venography was performed with digital subtraction and revealed severe stenosis in the distal SVC at the right atrial junction (Figure 3A). A resting mean gradient of 8mm Hg was also measured between the SVC and RA. A 55 cm 7 Fr sheath (Cook Medical) was then placed in the right femoral vein, and a 45 cm 7 Fr sheath (Cook Medical) was also placed in the left subclavian vein. The subclavian sheath was used for balloon dilatation and stent delivery while the femoral sheath was used for venography. Using a cm Supra Core wire (Abbott Vascular) and under fluoroscopic and transesophegeal echocardiographic Vascular Disease Management June

3 Figure 3. Digital subtraction angiography with simultaneous contrast injection in the superior vena cava (SVC) and right atrium (RA) after laser lead extraction (A). Intraoperative transesophageal echocardiogram demonstrating turbulent flow at the SVC-RA junction (B). (TEE) guidance, sequential balloon inflations were performed at the SVC-RA junction initially with a 5.0 mm x 40 mm EverCross balloon (Covidien), followed by 8.0 mm x 40 mm and subsequently 10.0 mm x 40 mm EverCross balloons. Ultimately, a 14.0 mm x 40 mm self-expanding Protege stent (Covidien) was positioned fluoroscopically across the stenosis. The stent was positioned precisely at the right atrial junction without protrusion into the atrium as confirmed by TEE (Figure 3B). After optimal positioning, the stent was deployed and postdilated with a 12.0 mm x 20 mm FoxCross balloon (Abbott Vascular) (Figure 4). Final angiography and echocardiography (Figure 5) revealed excellent stent expansion with complete resolution of the resting pressure gradient. Intra-procedural hemodynamics revealed a decrease in central venous pressure from 14 mm Hg to 6 mm Hg following stent deployment and postdilatation. Subsequently, new pacing leads were placed using the right subclavian approach. At 4 months follow-up, the patient was asymptomatic with complete resolution of the SVC syndrome. DISCUSSION In this unique report we show that endovascular stenting at the SVC-RA junction can be performed with Vascular Disease Management June

4 Figure 4. Postdilation with a 12 mm x 20 mm FoxCross balloon (Abbott Vascular) after deployment of a 14 mm x 40 mm self-expanding Protegé stent (Covidien). current multimodality imaging. With the increased implantation of pacemaker and defibrillator leads, iatrogenic SVC syndrome is a rare but clinically relevant complication without a straightforward treatment. Although laser and mechanical lead extraction can be effectively used to remove multiple leads causing the mechanical obstruction in the SVC, subsequent treatment options are limited when the fibrosis extends from the SVC into the right atrium. This report highlights an endovascular therapy that was possible with a multidisciplinary approach using the heart team concept in a hybrid catheterization laboratory and operating room with interventional cardiology, electrophysiology, cardiothoracic anesthesiology, and cardiac surgery clinicians working together. One of the major challenges in this procedure was optimal visualization of the right atrium and SVC junction. In addition, after extraction of all the leads, there were multiple web-like channels composed of scar tissue and organized thrombus further obstructing the transition from the SVC to the RA and posing the additional challenge of identifying the proper lumen for stent deployment. Simultaneous digital subtraction angiography via catheters positioned in the caudal and cephalad positions and intraoperative TEE allowed for optimal identification of the SVC-RA junction. Hemodynamic evaluation to measure pressure gradients from the SVC to the RA across the obstruction was performed with separate pressure transducers from the cephalad and caudal sheaths. Finally, the site of obstruction and maximal gradient were also identified by a high-velocity transesophageal echocardiography Doppler flow signal. Diminution of this gradient was seen after sequential balloon dilations. While the multidisciplinary approach to the management of SVC syndrome has been previously described, 6,7 the use of intraoperative transesophageal echocardiography and stenting of the SVC-RA junction are unique aspects of this case. Transesophageal echocardiography during laser lead extraction improves the safety of a procedure known to have a 1% to 2% risk of pericardial tamponade. 8,9 In addition to assuring safety, the use of TEE was instrumental in optimal positioning and deployment of the self-expanding stent at the SVC-RA junction, preventing extension into the right atrium. Significant extension of the stent into the right atrium could Vascular Disease Management June

5 Figure 5. Final digital subtraction angiography after superior vena cava (SVC) stenting of the SVC- RA junction (A). Final intraoperative transesophageal echocardiogram in the bicaval view displaying the SVC-RA junction (red arrow) (B). prevent stent endothelialization and predispose to potential thrombus formation and distal embolization into the pulmonary vascular bed. Likewise, the hybrid catheterization lab and operating room added an element of safety for a procedure known to have bleeding rates requiring thoracotomy up to 2.6% 9-12 while also allowing the versatility of high-quality imaging for optimal endovascular intervention. The concept of the heart team has recently been highlighted in relation to treating patients with critical aortic stenosis with transcatheter aortic valve replacement (TAVR). Interventional cardiologists, vascular surgeons, and cardiothoracic surgeons work using a team approach to devise a treatment and procedure plan. Similarly, hybrid coronary and peripheral revascularization procedures are performed when heart and vascular teams work to optimize patient outcomes. 13,14 This report demonstrates an example of the heart team approach in that endovascular SVC stenting after multiple laser and mechanical lead extractions followed by permanent pacemaker implantation was only possible through this approach. With this multidisciplinary approach between cardiovascular medicine and surgery in a hybrid catheterization laboratory and operating room, an optimal patient outcome was achieved. Vascular Disease Management June

6 CONCLUSION In this case of iatrogenic SVC syndrome, which developed as a result of vascular fibrosis at the superior vena cava-right atrial junction, a multidisciplinary heart team successfully treated the patient using a hybrid surgical and endovascular approach. After laser lead extraction, endovascular stenting of the SVC-right atrial junction was performed using angiography and transesophageal echocardiography for optimal imaging followed by lead replacement. n Editor s Note: Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein. Manuscript received June 1, 2013; provisional acceptance given July 29, 2013; final version accepted January 15, Address for correspondence: Ehtisham Mahmud, MD, Division of Cardiovascular Medicine, Sulpizio Cardiovascular Center, University of California, San Diego, 9434 Medical Center Drive, La Jolla, CA 92037, United States. emahmud@mail.ucsd.edu. Acknowledgements: The authors would like to thank Drs. Ulrika Green and Victor Pretorius for their contributions to this manuscript. REFERENCES 1. Stilo F, Lentini S, Spinelli F. The superior vena cava syndrome (SVCS) as a complication of pacemaker implantation. Pacing Clin Electrophysiol. 2010;33(10): Chang SS, Chen JY, Chang KC, Stephen Huang SK. Massive thrombotic occlusion of the superior vena cava caused by a single pacemaker permanent lead successfully treated by percutaneous venoplasty. J Cardiovasc Electrophysiol. 2010;21(6): Deo SV, Burkhart HM, Araoz PA, Brady PA. Innominate vein-right atrial bypass for relief of superior vena cava syndrome due to pacemaker lead thrombosis. J Card Surg. 2010;25(6): Klop B, Scheffer MG, McFadden E, Bracke F, van Gelder B. Treatment of pacemaker-induced superior vena cava syndrome by balloon angioplasty and stenting. Neth Heart J. 2011;19(1): Smith MC, Love CJ. Extraction of transvenous pacing and ICD leads. Pacing Clin Electrophysiol. 2008;31(6): Bakir I, La Meir M, Degrieck I, Marien C, Van den Hauwe K, Wellens F. Contralateral replacement of pacemaker and leads following laser sheath extraction and concomitant stenting for superior vena cava syndrome. Pacing Clin Electrophysiol. 2005;28(10): Bolad I, Karanam S, Mathew D, John R, Piemonte T, Martin D. Percutaneous treatment of superior vena cava obstruction following transvenous device implantation. Catheter Cardiovasc Interv. 2005;65(1): Swanton BJ, Keane D, Vlahakes GJ, Streckenbach SC. Intraoperative transesophageal echocardiography in the early detection of acute tamponade after laser extraction of a defibrillator lead. Anesth Analg. 2003;97(3): Kratz JM, Toole JM. Pacemaker and internal cardioverter defibrillator lead extraction: A safe and effective surgical approach. Ann Thorac Surg. 2010;90(5): Kennergren C. Excimer laser assisted extraction of permanent pacemaker and ICD leads: Present experiences of a European multi-centre study. Eur J Cardiothorac Surg. 1999;15(6): Wilkoff BL, Love CJ, Byrd CL, et al. Transvenous lead extraction: Heart Rhythm Society expert consensus on facilities, training, indications, and patient management: This document was endorsed by the American Heart Association (AHA). Heart Rhythm. 2009;6(7): Sohail MR, Uslan DZ, Khan AH, et al. Management and outcome of permanent pacemaker and implantable cardioverter-defibrillator infections. J Am Coll Cardiol. 2007;49(18): Kiaii B, McClure RS, Kostuk WJ, et al. Concurrent robotic hybrid revascularization using an enhanced operative suite. Chest. 2005;128(6): Bonatti J, Schachner T, Bonaros N, et al. Robotic totally endoscopic coronary artery bypass and catheter based coronary intervention in one operative session. Ann Thorac Surg. 2005;79(6): Vascular Disease Management June