Severe neonatal long QT syndrome type 3 treated with genotype-directed therapy and early left cardiac sympathetic denervation: a case report

Clicks: 13
ID: 326825
2026
Free PDF
Article Quality & Performance Metrics
Overall Quality
Not rated
Combines reader engagement with the AI quality analysis. This article has not been analysed, so there is no overall score — reader engagement is measured and shown alongside.
AI Quality Assessment
Not analyzed
Readership in this journal
Steady

Ranked #302 of 408 articles by views in European Heart Journal - Case Reports

Most read Least read

Bar heights use a square-root scale. Only the 120 most-read articles are drawn; the journal has 408 in total.

Mint this article as an NFT
Not yet minted

Create a permanent, verifiable on-chain record of this article on the Scimatic Network. The NFT is held in your Journament account, and you can withdraw it to your own wallet at any time.

5 SUSD one-off · no wallet required
Sign in to mint
Abstract
Abstract Background Long QT syndrome type 3 may present in fetal or neonatal life with QT prolongation, functional 2:1 atrioventricular block, and life-threatening ventricular arrhythmias. Early recognition and individualized genotype-directed management may be lifesaving. Case summary A neonate delivered after fetal bradycardia and intermittent 2:1 atrioventricular block presented with severe QT prolongation and recurrent torsades de pointes. The functional 2:1 atrioventricular block persisted despite beta blockade and magnesium administration. Because of recurrent ventricular arrhythmias, video-assisted thoracoscopic left cardiac sympathetic denervation and implantable loop recorder insertion were performed on day 8 of life. Genetic testing identified a de novo pathogenic SCN5A_p.(Ala1656Val) missense variant. Subsequent lidocaine testing demonstrated marked QT shortening, supporting sodium-channel-mediated disease and guiding treatment with mexiletine and flecainide. Long-term follow-up required implantable cardioverter-defibrillator therapy, but arrhythmic burden progressively improved. Discussion This case support the potential role of early multimodal treatment in malignant neonatal long QT syndrome type 3, combining left cardiac sympathetic denervation, continuous rhythm monitoring, and genotype-directed sodium-channel blockade.
Reference Key
openalex_W7204488140 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Andrea Greco, Estefanía Martinez-Barrios, Agustin Leonardo Lujan, Óscar Campuzano, Georgia Sarquella‐Brugada
Are you one of these authors? Claim your profile
Journal European Heart Journal - Case Reports
Year 2026
DOI
10.1093/ehjcr/ytag641
URL
https://academic.oup.com/ehjcr/advance-article-pdf/doi/10.1093/ehjcr/ytag641/70822601/ytag641.pdf
Keywords Keywords not found

Citations

No citations found. To add a citation, contact the admin at info@scimatic.org

Comments

No comments yet. Be the first to comment on this article.