Persistent left superior vena cava (PLSVC) is a rare congenital vascular anomaly, occurring in 0.3 to 0.5% of individuals in the general population [1]. Transvenous permanent pacemaker implantation (PPI) in patients with PLSVC is challenging because of the complex anatomy. The coronary sinus (CS) may be dilated, which render pacing leads positioning from the left subclavian region difficult, especially the ventricular lead. The literature regarding PPI in patients with PLSVC is sparse and limited to a few case reports [2‐8]. The use of active fixation leads with special curved stylet may help in overcoming this technical difficulty [2]. The purpose of this study was to describe the methods and evaluate the efficacy of patients with PLSVC who underwent PPI with double active fixation leads at our institutions.

Nine patients (6 women and 3 men) with PLSVC who underwent dual-chamber pacemaker implantation using double active fixation leads were included in the study retrospectively. Patients’ characteristics and procedure data were summarized in Table 1. The average age of the 9 patients with PLSVC was 68 ± 11 years (52–77 years). Indications for pacing were symptomatic third-degree atrioventricular (AV) block in one (11%) and sick sinus syndrome in eight patients (89%). One patient was referred for dual-chamber pacemaker implantation due to permanent complete AV block after mitral valve replacement. The pacemaker pocket was performed entirely in the left subclavian region irrespectively of the PLSVC.

A temporary pacing lead was inserted through the left subclavian vein in 2 patients due to the frequent symptomatic sinus pause prior to the PPI procedure. Venography was also performed to confirm the presence of PLSVC only in these two patients (Fig. 2). The ventricular lead was advanced into the RVOT and placed in the RVOT septum with a “C” shaped stylet easily in 7 patients (Fig. 3). Several attempts failed to introduce and advance the ventricular lead into the RVOT in the remaining 2 patients, not even using differently shaped stylets. For this reason, the ventricular leads were fixed in the RV apical region and the inferior free wall of sub-tricuspid annulus respectively. The atrial leads were placed at the right lateral wall with an original soft straight stylet in all patients (100%).

Total procedure time and fluoroscopy time was 85.3 ± 11.3 min and 4.5 ± 1.1 min respectively. There were no complications during any of the procedures.

Our results demonstrated the clinical safety of PPI through PLSVC, which may be technically feasible and effective. Double active fixation leads appear to be an effective procedure, especially in patients with PLSVC and we recommend this therapeutic approach rather than the choice of the contralateral right-sided implantation.

Active fixation pacing leads are more commonly used, as they are associated with easy fixation, reliability, lower rates of dislodgement and selective pacing, especially with abnormal anatomy. Moreover, active fixation leads offer the convenience of lead extraction. Therefore, double active fixation leads are highly recommended for patients with PLSVC and other abnormal anatomy. Stable lead thresholds were noted even on long-term follow-up that ranged up to 6 years.

The presence of this vascular anomaly is often detected incidentally during placement of cardiovascular implantable electronic devices and deca-polar coronary sinus catheter via left subclavian vein or surgery. PLSVC can also be detected by an experienced ultrasonologist during routine transthoracic echocardiographic examination. In our center, the incidence of PLSVC during device implantation was only found in 0.37% of patients, a similar rate to previous studies [1].

To our knowledge, this is the largest series of implantation of pacemakers in patients with PLSVC. In our study, the ventricular lead of most cases was placed at the septum of RVOT. This position of the ventricular lead seems hemodynamically more profitable than the classic RVA pacing. Furthermore, all PPI was performed in the left subclavian region irrespective of the PLSVC. This was an operator preference. In our center, operators are used to operating on the right subclavian region. At the same time, the absence of right superior vena cava (RSVC) also exists in some patients. Moreover, the vast majority of Chinese patients are right-handed. Although venography prior to the incision of the pocket device may be helpful in identifying the anatomy and the condition of the veins [9], the majority of our patients underwent device implantation safely and successfully without venography. Less experienced surgeons more often choose a right-sided device implantation after confirming a PLSVC by venography, and sometimes the leads can be easily replaced in the right atrium and the right ventricle via the RSVC. However, switching to the right subclavian region may add a new incision and increase the inconvenience for the patients particularly in right-handed patients.

In general, a specific J-shaped stylet designed to facilitate positioning of active fixation ventricular lead into the RV septum was used in patients without PLSVC [10, 11]. Instead, the major challenge for the operator is to advance the ventricular lead into the RVOT via the PLSVC. The existence of an acute angle between the CS ostium and the tricuspid valve makes the advancement and the placement of the lead into the right ventricle technically difficult. Various techniques such as the use of standard, special shaped and right ventricular septal stylets facilitating ventricular lead implantation have been reported [2]. In previous case reports [2, 3, 12], the ventricular leads were fixed only in the RVA rather than the RVOT septum and a right-sided implantation of the ventricular lead is suggested in case of unsuccessful stable ventricular lead position via the PLSVC [3]. In our study, all stable ventricular leads were positioned successfully and safely via a PLSVC. In most of the patients, the ventricular lead was advanced into the RVOT easily with a “C” shaped stylet. Active fixation lead offers the advantage of flexibility of choosing an optimal pacing site in the setting of heart abnormalities and particularly for patients with PLSVC. However, despite several attempts with different shaped stylets, we failed to advance the ventricular leads into the RVOT in two patients which is probably due to the moderate to severe enlargement of the cardiac chamber.

It is well known that the atrial active fixation lead was withdrawn into the right atrium from the right SVC and deployed at or close to the appendage using a J-shaped stylet in patients without PLSVC. Due to enlarged CS ostium, all atrial pacing leads were advanced into the right atrium via PLSVC with an original straight stylet, and the leads were placed at the right lateral wall without specifically shaped stylet in all patients.

Although successful PPI with acceptable pacing parameters were achieved in all patients with no periprocedural complications, optimal pacing or right ventricular mid-septal pacing was much more difficult to achieve than patients without PLSVC. Furthermore, total procedure time and fluoroscopy time in patients PLSVC was significantly longer than those patients without PLSVC. During a mean follow-up of 4 years, no late complications including device malfunction and lead dislodgement were observed. Stable pacing thresholds were noted even on long-term follow-up that ranged up to 6 years. The incidence of complications may be not related to the location of ventricular lead.

PPI through PLSVC may be technically feasible, safe, and effective. A venography in patients with PLSVC prior to pacemaker implantation is not necessary. Double active fixation leads may be standard for patients with PLSVC, and most of the ventricular leads could be placed at the RVOT septum.