Epicardial implantable cardioverter-defibrillator system placed in children with Jervell–Lange-Nielsen syndrome

Authors: Marchenko A.V. 1, Shekhmamet’ev R.M. 1, Vronskiy A.S. 2, Koryagina N.A. 2, Myalyuk P.A. 1, Nekrasova A.N .1

Company: 1 Federal Centre of Cardiovacular Surgery named after S.G. Sukhanov, Perm’, 614000, Russian Federation
2 Perm State Medical University named after academician E.A. Wagner, Perm’, 614013, Russian Federation

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Type:  Case reports

DOI: https://doi.org/10.24022/ 1997-3187-2020-14-1-82-92

For citation: Marchenko A.V., Shekhmamet’ev R.M., Vronskiy A.S., Koryagina N.A., Myalyuk P.A., Nekrasova A.N. Epicardial implantable cardioverter-defibrillator system placed in children with Jervell–Lange-Nielsen syndrome. Creative Cardiology. 2020; 14 (1): 82–92 (in Russ.). DOI: 10.24022/1997-3187-2020-14-1-82-92

Received / Accepted:  21.02.2020 / 27.02.2020

Keywords: long Q–T syndrome cardioverter-defibrillator system epicardial implantable syncope

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We presents a clinical case of girls 12 and 9 years old with long Q–T syndrome. Based on the data of the anamnesis, sudden death of a relative at the age of 40, sudden death of a younger brother at the age of 7, closely related parents marriage, laboratory and instrumental examinations, objective examination, as well as congenital deafmutism in girls, a rare hereditary Jervell and Lange-Nielsen syndrome has been identified. Both girls took anaprilin, while in the older patient, during therapy, syncope attacks associated with physical and emotional stress were noted. Based on clinical recommendations, as well as data from major studies, it was decided to implant a cardioverter defibrillator on both girls. The postoperative period was uneventful, patients were discharged under the supervision of a pediatric cardiologist with recommendations: taking anaprilin 6.5 mg 4 times a day, magnesium preparations 1 tablet 3 times a day for life. Patients were dynamically observed throughout the year. The elder sister, 12 years old, 4 and 9 months after the operation, triggered a cardioverter-defibrillator due to ventricular tachycardia. Actual data of epidemiology, etiology, pathogenesis of the disease, as well as a discussion based on these recommendations and major literature analyzes are presented.


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About Authors

  • Andrey V. Marchenko, Dr. Med. Sc., Cardiovascular Surgeon, ORCID
  • http:// Roman M. Shekhmamet’ev, Head of Department, ORCID
  • Aleksey S. Vronskiy, Resident Physician, ORCID
  • Natal’ya A. Koryagina, Dr. Med. Sc., Professor, ORCID
  • Pavel A. Myalyuk, Cardiovascular Surgeon, ORCID
  • Anna N. Nekrasova, Cardiologist-Arrhythmologist, ORCID

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, Director of Bakoulev National Medical Research Center for Cardiovascular Surgery