Treatment of Congenital Long QT Syndrome Presenting with Syncope (Fainting) Before Medical Treatment
This protocol addresses symptomatic congenital long QT syndrome in patients who have experienced
syncope prior to the initiation of medical treatment — a presentation that signals elevated
arrhythmic risk and guides specific management decisions.
Clinical Scenario
Syncope / Fainting
The patient has congenital long QT syndrome and has had one or more syncopal episodes
before any medical therapy has been started. This symptomatic status — syncope prior
to treatment — defines the risk stratum and the therapeutic pathway.
Approach — Partial Overview
For patients who go on to require device therapy and subsequently experience refractory
events despite pharmacological management, the protocol includes a specific
surgical intervention targeting cardiac sympathetic innervation. The full eligibility
criteria, sequencing, and decision algorithm are in the complete protocol.
References
DOI: 10.1093/eurheartj/ehac262
- Syncope prior to medical treatment.
- ICD implantation is recommended in patients with LQTS who are symptomatic while receiving beta-blockers and genotype-specific therapies.
- LCSD is indicated in patients with symptomatic LQTS when: (a) ICD therapy is contraindicated or declined; (b) patient is on beta-blockers and genotype-specific drugs with an ICD and experiences multiple shocks or syncope due to VA.
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