Replacement of an Implantable Cardioverter-Defibrillator (ICD) with a New Standard Subcutaneous ICD System in a Patient with Jervell and Lange-Nielsen Syndrome

Abstract

A 7-year-old female suffering from syncope attacks and deafness was genetically diagnosed with Jervell and Lange-Nielsen syndrome (JLNS). A transvenous-designed shock lead and implantable cardioverterdefibrillator (ICD) were atypically implanted subcutaneously, because the patient's body was small. Six years after implantation, we confirmed the patient's eligibility for a subcutaneous ICD (S-ICD) based on electrocardiogram screening. The implanted ICD system was replaced with a new standard S-ICD system. Implantation of the S-ICD may be considered a reliable and safe option in young patients with JLNS, even if their electrocardiograms show remarkable prolongation of the QT interval and T-wave alternans.

Epicardial vs. transvenous implantable cardioverter defibrillators in children

“…Lastly, considering the young age of patients and the lifelong risk of vascular access issues and infection, the S-ICD appears as a promising option to improve the benefit/risk balance in this specific population. However, clinical experience with the use of S-ICD in paediatric patients is still very limited, 21 , 22 this approach is generally not suitable in small children under 30 kg, 23 and we still have to learn about the potential impact of growth on long-term appropriate sensing and defibrillation field with this technology.…”