Ann Thorac Surg 2008;85:1083-1085. doi:10.1016/j.athoracsur.2007.09.034
© 2008 The Society of Thoracic Surgeons
Case Reports
Cyanosis Produced By Superior Vena Caval Stenosis
Brian E. Kogon, MDa,*,
Courtney Plattner, BSa,
Staci Jennings, RNb,
Teresa Lyle, NPb,
Michael McConnell, MDb,
Wendy M. Book, MDb
a Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia
b Department of Cardiology, and Sibley Cardiology, Emory University, Atlanta, Georgia
Accepted for publication September 17, 2007.
* Address correspondence to Dr Kogon, Emory University, Children’s Healthcare of Atlanta, Egleston, 1405 Clifton Rd, Atlanta, GA 30322 (Email: brian_kogon{at}emoryhealthcare.org).
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Abstract
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Superior vena cava syndrome is a rare complication of pacemaker implantation. Narrowing and occlusion of the superior vena cava is often asymptomatic due to the formation of decompressive collateral pathways to the right atrium. We present a case in which an anomalous venous pathway allowed decompression of the systemic venous return into the left atrium, resulting in arterial desaturation, cyanosis, and fatigue.
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Introduction
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Superior vena cava syndrome (SVC) is due to obstruction of the SVC. This presents clinically as facial and upper extremity edema and distention of the veins in the face, neck, arms, and upper thorax. Although the majority of cases result from malignant disease, foreign bodies within the venous system, such as central lines and pacemaker leads, are sometimes responsible. Most patients with SVC obstruction due to benign disease gradually develop chest wall and mediastinal collaterals so that their symptoms improve with time. Occasionally, surgical replacement or bypass of the superior vena cava is used to relieve the SVC obstruction.
Our patient is a 72-year-old man with a history of bicuspid aortic valve and coarctation of the aorta. He had previously undergone repair of his coarctation through a left thoracotomy and bioprosthetic aortic valve replacement through a midline sternotomy. His valve replacement was complicated by surgical heart block requiring permanent pacemaker implantation. He has since had bilateral transvenous pacemaker systems placed through the subclavian vein.
He presented with shortness of breath and cyanosis, with an arterial oxygen saturation of 88% on room air. His workup included a lengthy pulmonary and cardiac evaluation.
Ultimately, an echocardiographic bubble study was performed through a left arm intravenous catheter showing opacification of the left atrium. A subsequent cardiac catheterization through the femoral vein showed superior vena cava stenosis (Fig 1). Injection through the left internal jugular vein showed decompression of the upper body venous system into the left atrium (Fig 1) through an anomalous venous connection. Magnetic resonance imaging confirmed the anatomy (Fig 2).
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Fig 1. Preoperative cardiac catheterization. (A) Injection into the superior vena cava shows four pacemaker leads. There is severe narrowing of the superior vena cava above the right atrial junction. No collateral circulation into the right atrium is identified. (B) Injection into the left internal jugular vein shows an anomalous venous connection into the left atrium.
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Fig 2. Computed tomographic scan. (A) A posterior coronal view shows normal superior vena caval drainage into the right atrium and anomalous venous drainage into the left atrium. Pacing leads are also visible. (B) Three-dimensional reconstruction further delineates the anatomy.
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He was taken to the operating room for surgical correction. A midline sternotomy was performed, and the anomalous vein was identified. With temporary occlusion, the central venous pressure rose from 20 to 35 mm Hg, necessitating passive decompression into the inferior vena cava through a veno-venous bypass system. The distal end of the anomalous vein was then transected and oversewn at its entry site into the left upper pulmonary vein. The proximal end was sewn to a 10-mm ringed polytetrafluoroethylene (polytetrafluoroethylene [Gore-Tex; W. L. Gore & Assoc, Flagstaff, AZ]) tube. This ringed tube was then tunneled underneath the aortic arch and anastomosed to the right atrium using a side-biting clamp (Fig 3).
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Fig 3. Intraoperative view and postoperative venogram. (A) A ringed Gore-Tex conduit (W. L. Gore & Assoc, Flagstaff, AZ) can be seen traversing underneath the aortic arch, connecting the anomalous vein to the right atrium. (B) A postoperative venogram confirms patency.
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Postoperatively, the patient had a venous pressure of approximately 12 mm Hg proximal to the anastomosis, and his arterial oxygen saturations were 97% to 99% on room air. A venogram showed a widely patent pathway from the anomalous vein, through the graft, into the right atrium (Fig 3). His hospital stay was unremarkable, and he was discharged on postoperative day 5. At a 4-month visit, he was asymptomatic, playing golf and volleyball daily. His oxygen saturations remained in the high 90s.
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Comment
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SVC Syndrome
Superior vena cava syndrome is a constellation of signs and symptoms that result from occlusion of the SVC. The majority of SVC occlusions are the result of malignant tumors, with reports ranging from 60% to 80% [1, 2], whereas the remainder are due to benign causes. Superior vena cava syndrome is typically asymptomatic due to the slow progression of the disease and the formation of decompressive collateral pathways into the right heart. With acute stenosis or occlusion that does not allow for collateral formation, patients may present with edema of face, neck, and upper extremities, dyspnea, and headaches.
Thrombus surrounding foreign bodies within the venous system, such as transvenous pacemaker leads seems to be an increasingly common cause of SVC syndrome. In patients with transvenous pacemakers, venous thrombosis has been reported in 30% to 45% of patients [3], whereas symptomatic SVC syndrome has been reported in 0.03% to 0.4% [4, 5].
Treatment options for pacemaker lead-induced SVC syndrome include thrombolytics, percutaneous catheter-based intervention, and surgery.
Currently recombinant tissue plasminogen activator seems to be the most commonly used thrombolytic [6]. If occlusion is diagnosed within the first 5 days, thrombolytic therapy has an 88% success rate; unfortunately, if thrombolytic therapy is not started within the first 5 days, the success rate subsequently falls to 25% [7]. Thrombolytics have had varying success in treating thrombus formation secondary to pacemaker leads because they are typically infused peripherally and take the route of least resistance, preventing them from working effectively at the exact site of the obstruction. As in cases of catheter-induced SVC thrombosis, delivery of thrombolytics through a central line at the site of occlusion may be more successful [7].
Percutaneous intervention typically involves balloon venoplasty with or without stent placement. Although balloon venoplasty with stent placement is an effective and minimally invasive procedure, concern persists over the long-term effects of compression on the pacing wires by the stent. Extraction of the pacing wires followed immediately by venoplasty, stenting, and replacement of the pacing wires has been shown to be a safe and effective alternative method [8, 9]. Unfortunately, with percutaneous techniques, re-stenosis frequently requires subsequent interventions.
Finally, surgical options include patch venoplasty and bypass. Bypass grafting has been reported with polytetrafluoroethylene grafts, bovine pericardial conduits, and spiral saphenous vein grafts. Polytetrafluoroethylene grafts have been identified as a risk factor for worse outcomes, with four year primary patency rates of 17% compared with 67% for spiral vein grafts [10]. Subsequent percutaneous intervention after surgery can prolong these patency rates with either type of bypass graft.
Choice among these treatment modalities should be influenced by age of the patient, timing and severity of presentation, cause, and overall patient prognosis.
Systemic and Pulmonary Venous Embryology and Pathology
Anomalous connections between the systemic and pulmonary veins are uncommon.
During fetal development, the lungs are derived from the embryologic foregut and their venous drainage is through the splanchnic plexus into the cardinal and umbilicovitelline veins. The cardinal veins are precursors of the internal jugular veins, the innominate vein, and the superior vena cava, along with the coronary sinus. The umbilicovitelline veins are precursors of the inferior vena cava and portal vein. There is no direct connection between the lungs and the heart until the common pulmonary vein develops as an outpouching from the sinoatrial portion of the heart. Once pulmonary connections are established, the splanchnic connections are lost and the pulmonary venous drainage is into the left atrium [11].
In the presence of abnormal pulmonary venous anatomy, an anomalous vein connecting the systemic and pulmonary venous system in this location likely represents the "vertical vein" of supracardiac total anomalous pulmonary venous return. This is a persistent splanchnic connection that allows decompression of the pulmonary venous confluence into the innominate vein and subsequently into the right atrium. Treatment of supracardiac total anomalous pulmonary venous return is surgical and includes anastomosing the pulmonary venous confluence to the left atrium, closing the obligatory atrial septal defect, and ligating the vertical vein. This completely separates the systemic and pulmonary venous returns.
In the presence of normal pulmonary venous anatomy, an anomalous vein connecting the systemic and pulmonary venous system in this location likely represents a levoatrial cardinal vein (LACV). This is a rare congenital anomaly that connects the pulmonary veins to a derivative of the cardinal veins. It is most commonly found in conjunction with a left atrial inflow or outflow obstruction, such as mitral atresia or left heart hypoplasia, and provides a mechanism for pulmonary decompression [12]. Uncommonly, isolated cases of LACV have been reported [12–15]. The majority of patients with a LACV present with symptoms related to their cardiac defects. Patients with an isolated LACV may have a murmur, dyspnea on exertion, and right heart hypertrophy [14, 15]. Echocardiography is a quick, noninvasive diagnostic method to evaluate systemic and pulmonary venous return [12]. Care must be taken to distinguish between the LACV and the vertical vein of the supracardiac total anomalous pulmonary venous return by confirming pulmonary venous connection to the left atrium [16]. Computed tomography or magnetic resonance angiography may provide additional imaging of the venous connections if needed. Treatment for an isolated LACV is ligation. Again, this completely separates the systemic and pulmonary venous returns.
In cases of anomalous connections between the systemic and pulmonary venous circulations, blood typically flows from left to right allowing decompression of the pulmonary veins. Throughout our patient’s life, in the presence of normal central venous pressure, the degree of shunting through this anomalous vein was clinically insignificant. Only after developing pacemaker-induced SVC syndrome did clinical symptoms arise. With elevated central venous pressures, the anomalous vein provided systemic venous decompression into the pulmonary venous system, created a significant right-to-left shunt, and ultimately led to arterial desaturation, cyanosis, and fatigue. A thorough evaluation for a right-to-left shunt allowed for an accurate diagnosis and a successful surgical correction.
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References
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- Fujiwara K, Naito Y, Komai H, et al. Tetralogy of Fallot with levoatrial cardinal vein Pediatr Cardiol 1999;20:136-138.[Medline]
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- Lee ML, Wang JK, Lue HC. Levoatriocardinal vein in mitral atresia mimicking obstructive total anomalous pulmonary venous connection Int J Cardiol 1994;47:1-4.[Medline]
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Abstract
Introduction
