Superior Vena Cava Rupture

By Mark W. Burket, MD
 

CASE PRESENTATION
A 49-year-old, white female was referred for evaluation of suspected superior vena cava (SVC) syndrome. She first noted facial puffiness 10 months prior to admission, and her condition progressed to swelling involving her head, neck, and arms. Facial and hand cyanosis were present.

Her history was significant for breast cancer, for which she had undergone mastectomy and lymph node resection 23 years previously. She had not undergone central venous catheterization or radiation therapy. Recent evaluations had shown no evidence of tumor recurrence. Upon discovery of swelling and cyanosis, her oncologist ordered a contrast chest CT scan. This revealed severe stenosis of the SVC, with no evidence of tumor recurrence, extrinsic compression, or thrombus.

Angiography revealed critical stenosis of the SVC (Figure 1), with reversal of flow in the azygous vein.
Angioplasty was then undertaken after administering conscious sedation and systemic heparinization and upsizing to an 8-F, 80-cm sheath. A 10-mm-diameter balloon was used for a single inflation at 10 atm. The patient reported mild pain during balloon expansion. Approximately 1 minute later, she became apneic and pulseless.

HOW WOULD YOU PROCEED?
1. What is the most likely cause of the patient’s cardiovascular collapse?
2. What is the next procedural step that should be
initiated?
3. Is there a surgical treatment option for this patient?
4. What is the most definitive endovascular treatment option?
5. Should the heparin be reversed with protamine?

CLINICAL COURSE
Cardiopulmonary resuscitation was initiated. While maintaining guidewire placement across the involved region of the SVC, angiography was performed through the vascular sheath. This clearly demonstrated contrast extravasation from the SVC through a large rent in the vessel, with contrast swirling over the cardiac silhouette (Figure 2). The balloon used for the original treatment was reintroduced and inflated to 6 atm. Pericardiocentesis was performed and 1,000 mL of bloody fluid was removed. The balloon was deflated and quickly removed, and a flexible 10-F, 65-cm sheath was rapidly placed. A 10-mm-diameter, 50-mm-long Wallgraft (Boston Scientific Corporation, Natick, MA) was deployed in the SVC (Figure 3). An additional inflation using the original balloon catheter was performed.

OUTCOME
Final angiography demonstrated resolution of the original stenosis, no evidence of contrast extravasation, and restoration of normal antegrade flow in the azygous vein (Figure 4). The patient regained consciousness after these procedures, and her blood pressure and pulse returned to preprocedural values. There was no additional drainage from the pericardial catheter. She had no further hemodynamic instability throughout her hospital course.

Three months later, the patient developed recurrent signs and symptoms of SVC syndrome, and she underwent successful repeat balloon angioplasty without adjunctive stent placement for a discrete region of in-stent restenosis. During the subsequent 17 months of follow-up, the patient developed no recurrent signs or symptoms of SVC syndrome or pericardial tamponade. Chest CT scans have shown no pericardial effusion. The patient did, however, develop recurrent breast carcinoma with bone and subcutaneous metastases at 17 months, as demonstrated by CT scanning.

DISCUSSION
Angioplasty and stent placement is now considered by many to be the procedure of choice for the treatment of SVC syndrome.1 Serious complications are rare, but fatal pericardial tamponade has been reported.2,3 No cases of survival after pericardial tamponade have been reported.

The most significant lesson to be gleaned from this case is that rapid recognition and definitive treatment of SVC laceration can save lives. The proper steps must be executed quickly and in the correct order. The crucial first step in this case was to establish a definitive diagnosis. The differential included SVC laceration with hemothorax, SVC laceration with pericardial tamponade, pulmonary embolus, medication reaction, or a vasovagal episode. Each would require a different treatment approach. For this reason, angiography was required immediately, during which the patient was supported by cardiopulmonary resuscitation.

Once angiography confirmed the diagnosis, treatment could be initiated promptly. The fastest way to seal a perforated vessel, in this or any other case, is to reintroduce and inflate the balloon that created the problem in the first place. Once blood loss had been checked, pericardiocentesis allowed for the rapid restoration of hemodynamic stability. Definitive treatment of the tear could then be undertaken.

In the past, the only options to treat such a catastrophe were surgical repair or the emergent construction of a stent graft using autologous vein or PTFE.4,5 All of these options were complex and involved substantial delays before repair could be achieved. Management changed drastically with the introduction of the Wallgraft, self-expanding PET-covered stent, and more recently the Viabahn self-expanding PTFE-covered stent (W.L. Gore & Associates, Flagstaff, AZ). Either of these devices can be introduced percutaneously and deployed rapidly. Neither has FDA approval for this indication.

The cause of this patient’s SVC stenosis remains unknown. Tumor, previous central line use, and previous radiotherapy are all well-known causes of SVC stenosis, but none were present in this patient. Despite the recurrence of tumor elsewhere, no tumor was detected in the mediastinum.

Was the balloon too large for this patient? Intracardiac echocardiography indicates that a 10-mm balloon is actually quite conservative. In a study examining the diameter at the SVC-right atrial junction, a mean value of 16.4 mm was measured. Measurements were obtained in a predominantly female population, with a mean age of 28 years.6 Nonetheless, a smaller diameter balloon may have averted this complication. In conclusion, anticipation of this catastrophic complication and its rapid treatment is essential for patient survival. 

Mark W. Burket, MD, is Director of Vascular Medicine at the Medical College of Ohio, Toledo, Ohio. He holds no financial interest in any product or manufacturer mentioned herein. Dr. Burket may be reached at (419) 383-3697; mburket@mco.edu.

1. Smayra T, Otal P, Chabbert V, et al. Long-term results of endovascular stent placement in the superior caval venous system. Cardiovasc Intervent Radiol. 2001;24:388-394.
2. Martin M, Baumgartner I, Kolb,M. Fatal pericardial tamponade after Wallstent implantation for malignant superior vena cava syndrome. J Endovasc Ther. 2002;9:680-684.
3. Boardman P, Ettles DF. Cardiac tamponade: a rare complication of attempted stenting in malignant superior vena caval obstruction. Clin Radiol. 2000;55:645-647.
4. Patel NH, McLennan G, Shah H. Introduction of a PTFE-covered long, spiral-articulated Palmaz stent through a 10-F sheath using umbilical wrapping technique. J Vasc Intervent Radiol. 1999;10:1063-1066.
5. Reddy GS, Rothstein CP, Saker MB, et al. Placement of a PTFE-covered Wallstent through a 12 F sheath for the exclusion of a common iliac artery aneurysm. Cardiovasc Intervent Radiol. 1999;22:152-154.
6. Callans DJ, Ren J, Schwartzman D, et al. Narrowing of the superior vena cava-right atrium junction during radiofrequency catheter ablation for inappropriate sinus tachycardia: analysis with intracardiac echocardiography. J Am Coll Cardiol. 1999;33:1667-1770.


Figure 1. Initial angiogram demonstrating severe SVC stenosis.


Figure 2. Angiography after angioplasty demonstrating contrast leak from the SVC.


Figure 3. A 10-mm-diameter, 50-mm-length Wallgraft deployed in the SVC.


Figure 4. Final angiography demonstrated resolution of the original stenosis, no evidence of contrast extravasation, and restoration of normal antegrade flow in the azygous vein.

 

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