Sixty years of continuous pacemaker dependency

Key Teaching Points.

• •Permanent pacing was first described in 1958. This case report describes the life of a woman who underwent implantation with a pacemaker for complete heart block in childhood shortly after this revolutionary advance. This is the longest pacemaker dependency reported in the literature.
• •Pacemaker implantation is accompanied by multiple challenges, as demonstrated in this report. In her lifetime, she experienced short-lived pacemaker batteries, lead fracture, venous occlusion, pacing-induced cardiomyopathy, and valve interference.
• •Caring for patients with cardiac implanted electronic devices requires recognition and management of the various obstacles. These obstacles can require lead extraction, cardiac resynchronization therapy (or conduction system pacing) upgrades, and treatment of tricuspid valve disease.
• •Current and future technologic advances come with the promise of preventing many of the challenges of permanent pacing. These advances include improved battery longevity, conduction system pacing, and leadless pacing.

Introduction

Prior to 1958, the prospect for long-term survival with complete heart block was bleak. In that year, Dr. Ake Senning implanted the first permanent pacemaker,^1,2 offering hope to many affected by bradycardia. Previously, the longest reported duration of continuous pacing was 53 years.³ The following report describes a woman who we believe has the longest period of pacemaker dependency.

Case report

The patient was born July 17, 1954, with a ventricular septal defect. In 1957, she underwent ventricular septal defect repair complicated by complete heart block. She recalls oral isoproterenol given by her mother when her heart rate became too slow. In 1963, she underwent pacemaker implantation. Epicardial pacing leads were connected to a Medtronic Chardack Greatbatch pulse generator (Medtronic, Minneapolis, MN). She saved the original generator (Figure 1).

Figure 1.

The patient’s initial Chardack Greatbatch Pulse Generator (Medtronic, Minneapolis, MN) implanted at Henry Ford Hospital in Detroit, Michigan, in 1963.

During subsequent years, she underwent approximately 2 dozen generator replacements and, eventually, received a transvenous lead implant. She recalls early pacemakers with battery longevity of only a few months.

In 2006, she presented with syncope owing to a ventricular lead fracture requiring lead extraction and implantation of a new dual-chamber pacing system. All leads were successfully removed with the exception of the tined tip of a right ventricular apical lead. During the next 4 years, left ventricular function declined and, despite optimal medical therapy, her ejection fraction decreased to 35% in 2010.

In 2010, she was referred for a cardiac resynchronization upgrade, but was found to have left upper extremity venous occlusion. She underwent extraction of all leads for access with implantation of a biventricular defibrillator system. There were no viable lateral cardiac veins, and the patient underwent epicardial left ventricular lead placement. After cardiac resynchronization defibrillator implant, her left ventricular function normalized by 2013.

Beginning in 2016, the patient experienced symptomatic persistent atrial fibrillation and typical atrial flutter requiring cardioversion. She developed recurrent severe left ventricular dysfunction with an ejection fraction of 35%.

In 2018, she presented with lower extremity edema and ascites attributed to severe tricuspid regurgitation. Minimally invasive tricuspid valve replacement was performed with repositioning of both the right atrial pacing lead and the right ventricular defibrillator lead.

Significant scarring and retraction of the tricuspid leaflets were noted, requiring resection of the leaflets and placement of a 27-mm MagnaEase bioprosthetic valve (Edwards Lifesciences, Irvine, CA) with repositioning of the right ventricular lead outside the sewing ring. For unclear reasons, the right atrial lead was repositioned such that the tip was now placed in the cavotricuspid isthmus.

She remained well except for occasional symptomatic atrial fibrillation and flutter until 2021 when she underwent pulmonary vein isolation and cavotricuspid isthmus ablation. Unfortunately, ablation of the cavotricuspid isthmus resulted in exit block of the displaced right atrial lead, leading to atrioventricular dissociation; this posed a significant challenge because she was known to have left upper extremity venous occlusion and the right ventricular lead was “jailed” beneath the bioprosthetic tricuspid valve. Therefore, a 4F SelectSecure Model 3830 pacing lead (Medtronic, Minneapolis, MN) was placed in the right atrial appendage via the right axillary vein and tunneled across her chest to the left-sided generator. The chest x-ray (Figure 2) demonstrates multiple active and inactive pacing leads dating back to her childhood.

Figure 2.

Chest x-ray demonstrating a biventricular defibrillator (Boston Scientific, Marlborough, MA) using a right ventricular defibrillator lead from the left axillary vein, a pacing lead in the right atrial appendage tunneled from the right axillary vein, and an epicardial left ventricular pacing lead. Also noted are abandoned epicardial wires, a residual pacemaker tip, and an abandoned right atrial lead with its tip in the cavotricuspid isthmus.

In 2025, 62 years after the initial pacemaker implant, the patient lives with her husband and is enjoying retirement from a career in business management. Despite experiencing many of the typical challenges of complete heart block, her story remains a testament to the advances in pacemaker technology and a reminder of the challenges brought about by a lifetime of pacemaker dependency.

Discussion

The introduction of permanent pacemaker implantation in 1958 has provided lifesaving and life-enhancing benefits for millions. With these benefits, however, have come multiple potential limitations, as illustrated by our patient who has been pacemaker dependent since 1963. During these years, she has overcome a variety of pacing-related complications, including rapid battery depletion, lead fracture, pacing-induced cardiomyopathy, vascular occlusion, and tricuspid valvular regurgitation. Technological advances have mitigated many of the recognized pacing-induced complications. Battery longevity of current pacemakers often exceeds 10 years.⁴ Conduction system pacing has reduced the rates of pacing-induced cardiomyopathy.⁵ Leadless single- and dual-chamber pacemakers are now available, and come with the promise of reduced risks associated with upper extremity vascular access and tricuspid valve entrapment.^6,7

Conclusion

This report describes the course of a woman with complete heart block who has been pacemaker dependent for more than 62 years. This is the longest reported duration of pacemaker dependent longevity in the medical literature.

Disclosures

The author has no conflicts to disclose.