Transjugular Helix Leadless Pacing System Implantation in Adult Congenital Heart Disease Patient with Previous Tricuspid Valve Surgery for Ebstein Anomaly

Abstract

Adult congenital heart disease (ACHD) represents a significant portion of congenital anomalies, and with improved treatments leading to an increased life expectancy, its prevalence has been increasing over the past few decades. Nonetheless, a considerable number of patients with ACHD require cardiac rhythm management devices during their lifetime. Traditionally, transvenous pacemaker placement has been the standard mode of treatment for these patients. However, some patients with ACHD have anatomical barriers that obscure this mode of treatment. Leadless pacing systems (LPSs) have changed the field of pacing. Currently, two different LPSs are available. In a real-world setting, implanting an LPS in patients after tricuspid valve (TV) surgery seems to be a straightforward procedure with a low risk of complications, with patients showing no valvular dysfunction after the intervention. LPS implantation is an option to avoid device-related complications in patients with previous TV surgery. Moreover, it has been demonstrated that even the jugular approach seems as safe as the femoral approach and could be considered an alternative implantation method for LPSs. The Aveir VR leadless pacemaker is a helix LPS with unique features, such as its capacity as a dual-chamber leadless pacemaker, the ability to map electrical parameters before releasing the device, and its possibility of being retrievable. Hereby, we present the case of Ebstein’s anomaly, atrial septal defect closure, and previous TV surgery with symptomatic intermittent advanced atrioventricular block. This case illustrates that a transjugular approach for LPSs is also feasible in patients with ACHD.

1. Introduction

Patients who have had tricuspid valve (TV) interventions, surgical or percutaneous, present an increased risk of conduction disorders requiring a permanent pacemaker due to the proximity of the atrioventricular node. However, the implantation of an endocardial right ventricular (RV) lead may impair the TV function through valve leaflet perforation, impingement, or entanglement in the valve apparatus. Tricuspid annular dilation, elevated pulmonary artery pressures, and multiple RV leads increase the risk of pacing lead-related tricuspid regurgitation (TR) [1,2,3]. In a randomized prospective clinical trial, neither pacing lead position nor diameter affected TR development significantly, and coronary sinus (CS) leads failed to achieve a statistically significant reduction in TR compared with RV leads [4].

Leadless pacemaker systems (LPSs) have become an effective alternative to traditional transvenous pacemaker (TVP) systems in selected patient populations [5]. In real-world settings, high implantation success rates, the low risk of major complications, and long-term follow-up data confirm the good efficacy and safety profiles [6,7]. Although limited to a small population enrolled in an international multicenter study, there are similar data on the safety and efficacy of LPSs in patients after TV surgery, and their implantation seems to be a straightforward procedure with no complications, with patients showing no valvular dysfunction after the intervention [8]. Hereby, we report a case of helix LPS implantation via the right internal jugular vein in a patient with previous surgical correction of Ebstein’s anomaly.

The Aveir VR (Abbott, Medical, Sylmar, CA, USA) is the second LPS after the Micra device (Medtronic, Minneapolis, MN, USA). It is a leadless helix system with upgrading capabilities to a dual-chamber LPS. A considerable number of patients with adult congenital heart disease (ACHD) require cardiac rhythm management devices during their lifetime. Implantation of endocardial or epicardial pacemakers presents specific challenges in this population. LPSs have the potential to overcome some of these challenges. Indeed, LPSs should be considered in cases where endocardial or epicardial implants are contraindicated or represent an otherwise suboptimal approach. They have advantages in ACHD patients with complex cardiac anatomy, difficult venous access, and the need for repeat procedures [9,10]. Our patient was a good candidate for an LPS because she had an intermittent advanced AV block with a low expected percentage of pacing and because of the previous TV intervention (TV repair plus reimplantation of the anterior TV leaflet). To the best of our knowledge, this is the first case of helix LPS implantation via the right internal jugular vein in an ACHD patient.

2. Materials and Methods

An active 41-year-old, 50 kg female patient with a history of Ebstein’s anomaly surgical correction was referred to our center for pacemaker implantation because of symptomatic intermittent advanced AV block recorded during 24 h Holter monitoring. Her previous medical history included percutaneous atrial septal defect (ASD) closure with Amplatzer in 2008. In 2013, she underwent surgical correction of Ebstein’s anomaly with plication of the RV posterior wall, TV repair with a 30 mm Medtronic Contour annulus implantation, and reimplantation of the anterior TV leaflet. A conventional TVP was excluded to reduce the risk of infection, worsening TR, long-term leaflet/lead interaction, and future damage. In addition, although previous experience reported using a left ventricle-CS lead [4], we did not consider it the first option due to the risk being comparable to that of a conventional TVP.

An epicardial pacemaker lead implantation was proposed, but after the heart team discussion, another open-heart surgery was considered intermediate/high-risk surgery, and the patient refused. For these reasons, an LPS was proposed as an option, given the anticipated low percentage of ventricular backup pacing support. Moreover, due to the possibility of a future upgrade to a dual-chamber LPS, an Aveir VR (Abbott, Medical, Sylmar, CA, USA) was chosen. The right femoral vein was occluded following a previous vein thrombosis, and the contralateral one was small, so it was decided to preserve it. After an ultrasound study of the jugular veins, we chose to implant the device via the right jugular vein because of its straighter path to the atrium and greater diameter than the left one (1.2 cm × 0.9 cm). Moreover, a thorough transthoracic echo study of the RV was performed to confirm that the Aveir VR device would fit without interaction or entrapment within the TV apparatus.

The procedure was performed in the fasting state and under moderate conscious sedation with intravenous boli of midazolam hydrochloride (0.03–0.05 mg/kg) and fentanyl citrate boli (0.7–1.4 mcg/kg) for short-term analgesia plus continuous infusion of dexmedetomidine. After the patient was prepped in a sterile fashion, mepivacaine 1% was used to infiltrate the skin between the two heads of the sternocleidomastoid muscle. Ultrasound-guided right jugular venous access was obtained utilizing the Seldinger technique, and a super-stiff Amplatz wire (Boston Scientific, Malborough, MA, USA) (180 cm, 0.035 cm) was passed through the 8 Fr sheath and advanced down to the inferior vena cava. Consecutive up-sizing via a 4 Fr size increase to a 24 Fr dilator was performed. Finally, the 27 Fr (outer diameter) Abbott Aveir sheath was passed over the wire into the mid-right atrium. Subsequently, the Aveir, on a deployment catheter (23 Fr), was passed through the 27 Fr outer sheath and moved across the TV at first into an apical RV septal position under fluoroscopic and echocardiographic guidance. Despite the good fluoroscopic position on angiograms and satisfactory R-wave sensing (7.6 mV), pacing impedance and thresholds were suboptimal on contact mapping before active fixation (3.5 V @ 0.4 ms). A second attempt was made at a higher position, and the device was deployed after a good contact mapping test with the current proprietary program surface analyzer using conductive telemetry with skin electrodes connected to the patient’s torso. The final threshold was stable at 1.25 V @ 0.4 ms, with stable impedance and good RV sensing (6.4 mV). The stability test and final position are shown in Figure 1A–D. At the end, a figure-of-eight suture was placed to remove the introducer.

3. Results

At the 1-month follow-up, the threshold was 1 @ 0.4 ms, with stable impedance and RV sensing (7.1 mV) with a percentage of pacing <1% and an expected battery life of 23.6 years. Similar stable parameters were found at the 6-month follow-up.

4. Discussion

Due to treatment improving for ACHD patients, leading to an increase in life expectancy, the requirement of cardiac rhythm management device implantation during their lifespan may occur. While the heterogeneity of patients with ACHD and the lack of randomized trials limit the creation of guidelines, device therapy is nonetheless increasing in usage for the management of ACHD patients. Implantation of endocardial or epicardial pacemakers presents specific challenges and limitations in this population. LPSs may offer a feasible solution and should be considered in cases where endocardial or epicardial implants are contraindicated or represent an otherwise suboptimal approach. Short-term risk factors associated with traditional transvenous pacemakers include pocket infections, lead dislodgement, cardiac perforation, and pneumothorax. Long-term risks include venous obstruction and tricuspid regurgitation. While some complications remain intrinsically related to the procedure, such as cardiac perforation or device dislodgments, among the advantages of LPSs is that they eliminate most of these short-term and long-term complications. Whether this approach would be an alternative in other cases or clinical scenarios must be demonstrated in a larger series of comparable cases.

LPSs have advantages in ACHD patients with complex cardiac anatomy, difficult venous access, and the need for repeat procedures. This case report shows how an LPS could be implanted in complex patients with ACHD. The patient was also a good candidate for an LPS because she had an intermittent advanced AV block with a low expected pacing percentage and because of the previous TV intervention (TV repair plus reimplantation of the anterior TV leaflet). Specifically, an advantage of Aveir in patients with ACHD is the ability to map prior to implanting. In such cases, lower voltage substrates with poor pacing capture thresholds can be checked prior to implantation; thus, less re-captures are needed. Furthermore, in this patient population where rhythm disorders may progress, adding an atrial Aveir may be important for future dual-chamber pacing needs. Finally, it has been demonstrated that the extraction of the predecessor of the Aveir LPS can be successfully performed up to 9 years post-implant [11]. It is important to consider the RV’s dimension before implanting the Aveir VR device. Indeed, a thorough transthoracic echo or a CT scan of the RV with several measurements must be performed to confirm that the Aveir VR device would fit without interaction or entrapment within the TV apparatus.

Moreover, our case is unique because the implantation was performed from the jugular vein. Although the feasibility of LPS implantation via the right internal jugular vein has been previously demonstrated [12], this is the first case of device implantation with a superior approach in ACHD. We recognize that a superior approach to implanting the atrial module would not be ideal when upgrading to a dual-chamber device. However, in our case, if a dual chamber is needed, we could use the left femoral vein, which was patent, even if small. Whether a superior approach would be an alternative in other cases or clinical scenarios must be demonstrated in a larger series of comparable cases.

5. Conclusions

In this case report, we describe, for the first time, a case of an ACHD patient with a permanent pacemaker implantation indication treated with an LPS implanted via the jugular vein. This case adds to the existing literature on the safe use of LPSs in the ACHD population, and we believe LPSs could be used in patients with ACHD with a good profile of safety and efficacy.