Surgical reconstruction for superior vena cava syndrome following pacemaker lead implantation

Abstract

Pacemaker-related superior vena cava (SVC) syndrome, caused by fibrosis along pacemaker leads, was identified in a 65-year-old woman with chronic renal failure and atrial fibrillation. Initial management with two vascular bypass grafts failed due to thrombosis, resulting in symptom recurrence. Surgical intervention was subsequently performed, involving SVC reconstruction below the azygos vein with a pericardium patch and concurrent tricuspid valve repair. This case illustrates the challenges associated with pacemaker-induced SVC syndrome and demonstrates the potential advantages of reconstructive surgery, which, by restoring venous anatomy, may improve long-term outcomes when performed in an experienced center.

Introduction

Superior vena cava (SVC) syndrome occurs when blood flow through the SVC is obstructed. Diagnosis is typically made through clinical evaluation and confirmed with radiological imaging. While some patients may remain asymptomatic due to collateral circulation, others may experience symptoms such as shortness of breath; swelling in the head, neck, and arms; and flushing. Approximately 65–70% of SVC syndrome cases result from intrathoracic malignancies, such as primary lung cancer or lymphoma [1, 2]. Multimodality imaging such as cardiac magnetic resonance, computed tomography (CT), and positron emission tomography can help to identify features that are suggestive of malignancy and guide prompt patient management [3].

The growing use of intravascular devices, such as central venous catheters and pacemaker leads, has significantly increased the occurrence of non-malignant SVC syndrome. These benign causes are now estimated to be responsible for up to 40% of all SVC syndrome cases [4]. Lastly, a rare cause of SVC syndrome is fibrosing mediastinitis, an uncommon disorder of inflammation and fibrosis involving the mediastinum, hypothesized to result from a severe fibroinflammatory host response to various antigens like Histoplasma, Mycobacterium, or Treponema [5].

The management of SVC syndrome is evolving. In the past, radiation therapy was considered the treatment gold standard [3] but nowadays, endovascular stent placement has become the first-line treatment in cases that require rapid symptom relief, even though there are no randomized control trials establishing it as superior to radiation or chemotherapy [6]. The endovascular approach, which has been evolving since the mid-1980s, has an impressive technical success rate, effectively alleviates symptoms in most patients, and maintains a low complication profile, with surgery reserved for unsuccessful endovascular interventions [7].

We hereby present a case of SVC syndrome caused by pacemaker lead–induced fibrosis, initially treated by two bypass grafts, followed by reconstruction of the SVC due to severe stenosis.

Case presentation

The patient is a 65-year-old woman with a history of cardiovascular risk factors including dyslipidaemia, chronic renal failure requiring dialysis, anemia, and atrial fibrillation; she also had secondary hyperparathyroidism and cervical osteoarthritis. Initially managed with medical therapy, the arrhythmia became poorly controlled, and following an unsuccessful attempt at cardioversion, a decision was made to adopt an ablate-and-pace strategy. Consequently, a dual-chamber pacemaker was implanted, with one lead positioned in the right atrium and the other in the right ventricle. After atrioventricular node ablation, the device was programmed in DDD mode at a base rate of 60 bpm. Approximately 3 years after pacemaker implantation, progressive fibrosis along the leads led to stenosis of the SVC. The patient presented with the classic symptoms of SVC obstruction syndrome: facial edema, non-pulsatile distended neck veins, and hoarseness. She was admitted to a hospital in Southeast Asia. Due to the lack of local technical backup in handling this type of scenario, the patient was not able to undergo an endovascular procedure, the initial management involved cardiac surgery through a median sternotomy, cardiopulmonary bypass, and two vascular bypasses: one from the innominate vein to the right atrium (using polytetrafluoroethylene (PTFE) graft) and the other from the right internal jugular vein to the right atrium (using a Dacron graft). The postoperative course was uneventful, and the patient was discharged with an anticoagulant regimen (warfarin). Unfortunately, the first bypass thrombosed approximately 30 days post-operation, and a follow-up CT scan at 4 months showed thrombosis of the second bypass as well. The patient developed severe recurrence of SVC syndrome, manifesting as neck vein distension, plethoric facies, and chest pain. She was transferred to our hospital: the CT scan revealed near-total occlusion of the mid-distal portion of the SVC, along with occlusion of the previous bypass grafts (Fig. 1). Surgical intervention was deemed necessary and was performed via redo sternotomy under cardiopulmonary bypass. An arterial line was established in the ascending aorta, and venous lines were placed in the inferior vena cava and close to the innominate vein. During the surgery, the SVC was reconstructed immediately below the azygos vein. After removing the two pacing electrodes and the surrounding fibrosis, a 6 cm × 2 cm oval-shaped patch of non-autologous pericardium was used to restore the optimal diameter of the SVC until the innominate vein (Fig. 2). Concurrently, tricuspid valve repair was performed using a size 28-mm Physio Carpentier ring (Edwards Lifesciences LLC, Irvine, CA, USA) with separate U-stitches, and a small septal cleft was closed using 5–0 Prolene suture. A new pacemaker was implanted in the epigastric region with two epicardial electrodes placed on the diaphragmatic surface of the right ventricle and the right atrium. The postoperative course was complicated by prolonged mechanical ventilation; the patient was later transferred to a specialized rehabilitation facility. Six months after the reconstruction of the SVC, a CT scan showed full vessel patency, and her symptoms had completely resolved.

Fig. 1.

Preoperative computed tomography scan after expanded polytetrafluoroethylene bypass occlusions. A The image shows superior vena cava stenosis, at the level of innominate vein confluence and above azygos vein confluence, caused by pacemaker leads. B Focal thrombus determines sub-occlusion of right venous bypass. C Left venous bypass is totally occluded. D Virtual reality reconstruction; major venous collateral system drains blood from supradiaphragmatic compartment to inferior veins

Fig. 2.

Intraoperative image of superior vena cava reconstruction. Intraoperative view showing the successful restoration of superior vena cava patency using an oval-shaped patch of non-autologous pericardium

Discussion

Symptomatic SVC syndrome is observed in approximately 1 out of every 250 to 40,000 patients who receive a pacemaker [8]. Recently, a proposed approach was only anticoagulation in first-line treatment [9]; however, its efficacy, types, and treatment duration are not studied in detail. Lead extraction from the SVC, particularly when leads are thrombosed, is feasible and safe in centers with appropriate expertise. Pre-procedural cardiac imaging is essential for a thorough understanding of the anatomy and effective risk assessment [3]. Contrast-enhanced venography should be used to confirm the level and degree of obstruction and can allow for concurrent endovascular therapy with balloon angioplasty and stenting if possible [7]. Nevertheless, cases of very narrowed SVC, the presence of long-standing leads, adhesions, and the relationship between the leads and the tricuspid valve pose significant challenges for this kind of patient. Surgical intervention not only addresses the removal of thrombosed leads but also allows for potential repair of the SVC and tricuspid valve and facilitates the implantation of an epicardial pacemaker if needed. Reconstruction for malignant SVC syndrome can be performed using a prosthetic patch, bovine pericardium, or autogenous material such as saphenous vein or pericardium [10]. There are limited studies on this topic [10–12], but we believe that repairing the affected vein is generally preferable to bypassing it because vein reconstruction with a patch helps maintain the vessel’s physiological structure. Reconstructive surgery restores the natural diameter and shape of the vein, promoting better long-term outcomes while also reducing the risk of future complications such as thrombosis or restenosis.

Conclusions

Pacemaker-induced SVC syndrome remains a rare but serious complication, particularly in the context of long-standing transvenous leads. While endovascular approaches represent the first-line treatment in many cases, surgical intervention becomes necessary when these fail or are not feasible. Our experience highlights the value of surgical reconstruction of the SVC, which not only restores physiological venous flow but may also improve long-term outcomes by reducing the risk of restenosis and thrombosis.

Author contribution

Michele D’Alonzo, Mariantonietta Piscitelli, and Antonio Fiore contributed to the study conception and design. Material preparation and data collection were performed by Mariantonietta Piscitelli and Michela Romandini. Visualization was handled by Francesco Cilia. The first draft of the manuscript was written by Michele D’Alonzo. All authors read and approved the final manuscript.

Funding

None.

Data statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Declarations

Ethical compliance

Ethical approval was waived by the local Ethics Committee in view of the retrospective nature of the study.

Informed consent

The patient involved in the study provided informed consent for data collection during and after hospitalization for research purposes.

Conflict of interest

The authors declare no conflict of interest.

Human and animal rights

This retrospective case report involving human participant was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.