Abstract
Data on lead management in patients with congenital complete heart block (CCHB) with cardiac implantable electronic devices are lacking. The purpose of this study was to describe the natural history and outcomes in patients with CCHB with cardiac implantable electronic devices undergoing transvenous lead extraction (TLE). Data on all attempted TLE procedures in patients with CCHB at 2 institutions between 2011 and 2021 were collected from a retrospective registry. Overall, 16 patients (mean age at transvenous device implant 13.8 ± 4.7 years) were included. Before TLE, patients underwent an average of 2.25 ± 1.3 generator changes, 3 (19%) underwent cardiac resynchronization therapy upgrade, and 7 (44%) underwent a lead revision with subsequently abandoned leads. Mean patient age at TLE was 34.4 ± 9.4 years with a mean duration of lead implant of 19.2 ± 6.9 years. Lead malfunction (n = 11 [69%]) and infection (n = 5 [31%]) were the most common indications for TLE. A total of 38 leads were removed, with complete procedural success achieved in 14 of 16 (87.5%). Two (12.5%) major complications occurred, including right ventricular laceration and superior vena cava tear requiring sternotomies. All patients survived at 1-year follow-up. Patients with CCHB represent a unique cohort highlighted by several generator changes, lead revisions, and abandoned leads at a young age, along with a long duration of lead dwelling time and a high prevalence of lead malfunction requiring TLE. There may be a high risk of major complications during TLE, suggesting TLE should be performed only in experienced centers. Larger studies are needed to confirm these findings.
Highlights
Isolated congenital complete heart block (CCHB) is a rare disorder that occurs in 1 in every 15,000 to 20,000 live births.[1]
Two (12.5%) major complications occurred, including right ventricular laceration and superior vena cava tear requiring sternotomies
Patients with CCHB represent a unique cohort highlighted by several generator changes, lead revisions, and abandoned leads at a young age, along with a long duration of lead dwelling time and a high prevalence of lead malfunction requiring transvenous lead extraction (TLE)
Summary
Isolated congenital complete heart block (CCHB) is a rare disorder that occurs in 1 in every 15,000 to 20,000 live births.[1]. Maternal antibodies are responsible for the majority of cases of fetal CCHB, nonimmune isolated CCHB may be diagnosed throughout childhood, and pacemaker implantation is the only known effective treatment that has been shown to increase survival and prevent syncopal events.[2–5]. As the number of children and young adults with pacemakers continues to increase, some of Funding Sources: The authors have no funding sources to disclose. Address reprint requests and correspondence: Dr Douglas Darden, Division of Cardiovascular Medicine, University of California San Diego, 9452 Medical Center Dr, Room 3E-313, La Jolla, CA 92037. Data on lead management in patients with congenital complete heart block (CCHB) with cardiac implantable electronic devices are lacking
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