Superior Vena Cava Syndrome: An Increasingly Frequent Complication of Cardiac Procedures

Cheng E Chee; Haraldur Bjarnason; Abhiram Prasad

Disclosures

Nat Clin Pract Cardiovasc Med. 2007;4(4):226-230. 

In This Article

Summary and The Case

Summary

Background A 29-year-old woman presented with chest pressure, progressive dyspnea, fatigue, and swelling of her face, neck and upper extremities. Four days earlier, she had undergone a procedure for treatment of inappropriate sinus tachycardia, during which atrioventricular node ablation and implantation of a single-lead VVI pacemaker had been performed following an unsuccessful attempt at sinus node modification.
Investigations Physical examination, contrast-enhanced CT of the chest, Doppler ultrasonography of the upper extremities, venography, coagulation studies, echocardiography.
Diagnosis Superior vena cava syndrome due to thrombotic obstruction---a complication of a cardiac procedure.
Management Anticoagulation, catheter-directed thrombolytic therapy, mechanical thrombectomy, pericardiocentesis.

The Case

A 29-year-old woman was admitted in October 2005, following an emergency transfer from her local hospital. She presented with chest pressure, progressive dyspnea, fatigue and swelling of her face, neck and upper extremities. Four days earlier, she had undergone an attempt at sinus node modification for treatment of inappropriate sinus tachycardia which, over a period of 5 years, had been refractory to multiple antiarrhythmic agents including β-blockers, calcium channel antagonists, digoxin and sotalol. Sinus node modification was unsuccessful and, consequently, atrioventricular node ablation and implantation of a single lead VVI pacemaker were performed in the same procedure. The patient was otherwise healthy, with no personal or family history of thrombosis or malignancy. Regular medication consisted of a weekly Ortho Evra® (norelgestromin/ethinyl estradiol transdermal system; Johnson and Johnson Corporation, New Brunswick, NJ) patch for contraception, which she had used for 8 weeks. She had stopped smoking 5 months earlier, with a previous 2 pack year smoking history, drank alcohol occasionally and denied illicit drug use.

On examination, the patient was afebrile with a blood pressure of 107/60 mmHg, a regular pulse of 90 beats/min, a respiratory rate of 14 breaths/min, and oxygen saturation at 97%, having received 4 l of oxygen via a nasal cannula. Her face, neck and arms were swollen due to edema, and the skin was cyanotic with prominent superficial veins (Figure 1A). There was no venous pulsation in the neck, indicating the likely occlusion of the jugular veins. These signs were consistent with superior vena cava (SVC) syndrome. Precordial examination was notable for a healing left pectoral scar and an intact implanted pacemaker. Heart sounds were normal without murmurs. Chest examination revealed dullness to percussion in the lower zones, with bronchial breathing over both bases. The remainder of the examination was unremarkable.

Figure 1.

Photographs of the patient showing the reduction in swelling of the face, neck and upper extremities. (A) At initial presentation and (B) after treatment (hospital day 8).

A contrast-enhanced CT scan of the patient's chest showed right subclavian and SVC thrombosis. An electrocardiogram demonstrated third degree atrioventricular block with a ventricular-paced rhythm. Transthoracic echocardiography showed an ejection fraction of 72% (normal value >55%) with a small pericardial effusion without tamponade physiology. Doppler ultrasonography of the upper extremities revealed hypoechoic thrombi in the internal jugular, subclavian and axillary veins, extending into both arms to involve the brachial veins. Thrombosis of both superficial cephalic and basilic veins was also observed. Venography confirmed thrombotic occlusion of the SVC (Figure 2A).

Figure 2.

A series of venograms showing the patient's superior vena cava. (A) Thrombotic occlusion was evident on presentation. (B) Thrombolysis restored patency but there was a persistent stenosis (arrow) at the superior vena cava–right atrial junction, shown here at 3 month follow-up. (C) Angioplasty was performed with a modest result (D).

The patient was anticoagulated with heparin (activated partial thromboplastin time target 60-80 s) and the extensive SVC thrombus was treated with catheter-directed thrombolytic therapy for 72 h (0.25 mg/h alteplase in each infusion catheter). Unifuse™ (AngioDynamics Inc, Queensbury, NY) infusion catheters were used, with one catheter placed via each antecubital fossa, extending into the contralateral jugular vein. The catheter position was adjusted daily over 3 days, on the basis of findings on the venograms, to allow delivery of the fibrinolytic to the site of the residual thrombus. On the second day of thrombolytic therapy, venography demonstrated that the left internal jugular remained occluded; the vessel was treated successfully with mechanical thrombectomy (AngioJet®, Possis Medical, Inc., Minneapolis, MN). The approach was successful in restoring patency of all the thrombosed vessels over 3 days; however, the final venogram, performed to assess vessel patency revealed a focal stenosis at the SVC-right atrium junction, with a 14 mmHg pressure gradient across the stenosis (normal value <3 mmHg). In the light of the recent ablation procedure around the SVC, angioplasty was not performed at this time because of the risk of rupture. Special coagulation studies (lupus anticoagulant, factor V Leiden mutation, prothrombin Gly20210A mutation, protein C, and protein S levels) did not indicate the presence of a prothrombotic state. The patient's symptoms and swelling of the face and upper extremities resolved completely 1 week after presentation (Figure 1B). She was discharged after 8 days in hospital having been prescribed warfarin (5 mg daily with goal international normalized ratio 2.5-3.5) for at least 3 months until her scheduled follow-up. She was advised against using oral or patch contraception to minimize the risk of recurrent thrombosis.

The patient returned 3 months after initial discharge for a scheduled follow-up visit. An upper extremity venogram confirmed both jugular and subclavian veins to be completely patent, but there was a persistent stenosis of the SVC with a 6 mmHg mean gradient (Figure 2B). The stenosis was treated by angioplasty (Figure 2C) using a 12 mm diameter, followed by a 14 mm diameter, 4-cm-long balloon. As the follow up venogram was being performed (Figure 2D), the patient suddenly became hypotensive and hypoxic. An echocardiogram confirmed the suspected diagnosis of a hemopericardium and cardiac tamponade. Successful emergency pericardiocentesis was performed by draining approximately 100 ml of blood. A contrast-enhanced CT of the chest did not detect continued bleeding into the pericardial cavity, and the pericardial pig-tail catheter did not drain additional blood. The pig-tail catheter was removed 2 days later once serial, daily echocardiograms confirmed resolution of the pericardial effusion. Two days after the tamponade episode, the patient developed transient chest pain due to pericarditis, which was treated with indomethacin for 2 weeks. In the treating physician's opinion, there was a significant risk of recurrent SVC thrombosis, so anticoagulation with warfarin (5 mg alternating with 7.5 mg daily, international normalized ratio goal 2.5-3.5) was cautiously resumed on the day of hospital discharge (day 5), without heparin bridging, for a duration of at least 9 months. Close follow-up was arranged to detect recurrent pericardial bleeding. The patient recovered well and returned to work.

One year after her initial presentation, the patient was still asymptomatic. Doppler ultrasonography of the upper extremities confirmed continued patency of the veins, and anticoagulation with warfarin was discontinued.

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