Ann Thorac Surg 2009;88:277-278. doi:10.1016/j.athoracsur.2008.12.034
© 2009 The Society of Thoracic Surgeons
Case Reports
Hybrid Treatment of Superior Vena Cava Syndrome in a Child
Robert L. Hannan, MDa,*,
Jennifer A. Zabinsky, MEnga,
Antero Hernandez, RCISb,
Evan M. Zahn, MDb,
Redmond P. Burke, MDa
a Department of Cardiovascular Surgery, Congenital Heart Institute, Miami, Florida
b Department of Cardiology, Congenital Heart Institute, Miami Children's Hospital, Miami, Florida
Accepted for publication December 9, 2008.
* Address correspondence to Dr Hannan, Division of Cardiovascular Surgery, Miami Children's Hospital, 3100 SW 62nd Ave, Miami, FL 33155 (Email: rhannan001{at}aol.com).
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Abstract
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A 10-year-old boy with a history of renal failure and hemodialysis by indwelling superior vena cava (SVC) catheters was diagnosed with SVC obstruction and clinically severe SVC syndrome. During attempted recanalization of the SVC in the cardiac catheterization laboratory, he suffered a perforation of his SVC with pericardial tamponade. After treatment of the perforation and relief of tamponade, he underwent a hybrid procedure to recanalize his SVC. A needle and then guidewire were passed directly from the right atrium through the SVC obstruction and were used to successfully dilate and stent the obstruction.
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Introduction
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Superior vena cava (SVC) syndrome results from obstruction or compression of the SVC. It is characterized by swelling of the face and arms, dyspnea, cough, and cognitive dysfunction. The SVC syndrome in children occurs from a variety of causes, including malignancy [1, 2], indwelling catheters resulting in thrombosis [3, 4], infection [5], and idiopathic thrombosis [4, 6]. The incidence of thrombosis and occlusion due to indwelling catheters in children has not been described.
The treatment of symptomatic SVC syndrome in children can be difficult. Some patients can be managed medically with anticoagulation [5]. When medical therapy is insufficient, surgery or catheter-based interventions are required [3]. Surgical SVC reconstruction has been performed with bypass grafts, including spiral saphenous vein, superficial femoral vein, or expanded polytetrafluoroethylene [4, 6]. Potential drawbacks of surgical reconstruction include the requirement for cardiopulmonary bypass and the lack of growth potential in bypass grafts. Catheter-directed thrombolysis or endovascular stent placement, or both, are described [1, 2, 7, 8]. These catheter-based treatments may be problematic if the obstruction can not be crossed [7].
We report a case of SVC syndrome that was successfully treated in the cardiac catheterization laboratory with a hybrid approach.
A 10-year-old boy with a history of chronic renal failure due to posterior urethral valves and hemodialysis by indwelling SVC catheters presented with cough, hemoptysis, and hematemesis, as well as head, neck, and arm swelling. He was transferred to the hospital intubated due to respiratory failure. A chest roentgenogram showed bilateral pleural effusion. An echocardiogram revealed complete occlusion of the SVC, and peripheral venous studies showed occlusion of both iliac veins. Both problems were presumably due to thrombosis from indwelling hemodialysis catheters with probable fibrosis from repeated intraluminal trauma.
He was brought to the cardiac catheterization laboratory for attempted recanalization of the SVC. Venous access was obtained in the right internal jugular vein. The SVC pressure was 44 mm Hg. The SVC obstruction was identified at the right atrial junction (Fig 1A). Attempts to cross the SVC obstruction with the Baylis Nykanen radiofrequency ablation wire were unsuccessful. An attempt was made to cross the obstruction with a pediatric Brockenbrough needle using biplane fluoroscopy and transesophageal echocardiography. It seemed that the needle had been passed into the right atrium, but after passing a guidewire it was clear that the needle and wire were in the pericardial space. On withdrawal of the needle there was an abrupt decrease in blood pressure, and echocardiography demonstrated a pericardial effusion. A pericardial drain was placed, but thrombus immediately formed and could not be drained from the pericardial space. The cardiac surgery team was immediately available during this high-risk intervention. A median sternotomy was performed, and the site of the perforation at the junction of the SVC and the pericardial reflection laterally was identified and oversewn. Angiography had demonstrated that the SVC obstruction was relatively short and discrete. Palpation confirmed the discrete nature and rock hard consistency of the obstruction. A pursestring suture was placed in the right atrium. While palpating the SVC obstruction, an 18-gauge spinal needle was passed from the right atrium through the obstruction into the superior portion of the SVC where blood was aspirated. A wire was placed through the needle and then snared through the sheath in the SVC. The guidewire was then exteriorized and used for serial dilation of the very resistant stenosis in the SVC. After serial balloon dilations, a stent was placed in the SVC with excellent flow from the SVC into the right atrium (Fig 1B). The SVC pressure was 13 mm Hg. The stent used has a maximum diameter of 18 mm. The chest was closed, and the patient made an uneventful recovery with complete resolution of his SVC syndrome. Two years later he remains free of symptoms.
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Fig 1. (A) Superior vena cava (SVC) obstruction at right atrial junction. (B) SVC obstruction relieved by stent.
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Comment
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The SVC syndrome in children can be difficult to manage. The catheter-based technique of SVC stenting has the advantage of being able to expand the stent in the future, but requires safe passage of an initial wire through the obstruction. Typically this involves catheter placement both above and below the obstruction, which was not possible in this case due to bilateral iliac vein obstruction. Our treatment of the complication of SVC perforation and pericardial tamponade led us to a hybrid approach. The relative simplicity and efficacy of this approach may be useful in the future when an obstructed SVC can not be safely traversed in the catheterization laboratory, especially when venous access is unavailable from below or when the nature of obstruction prevents safe passage. It avoids placement of an interposition graft with no growth potential, avoids cardiopulmonary bypass, and allows placement of a stent capable of reaching adult size.
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References
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- Kee ST, Kinoshita L, Razavi MK, et al. Superior vena cava syndrome: treatment with catheter-directed thrombolysis and endovascular stent placement Radiology 1998;206:187-193.[Abstract/Free Full Text]
- Barshes NR, Annambhotla S, El Sayed HF, et al. Percutaneous stenting of superior vena cava syndrome: treatment outcome in patients with benign and malignant etiology Vascular 2007;15:314-321.[Medline]
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Abstract
Introduction
