February 1, 2025Open Access
Managing long QT syndrome patients, cooking, and common sense
Key Result
A dynamic approach of yearly risk reassessment and therapy optimization in 946 long QT syndrome patients resulted in zero deaths over a 10-year follow-up, avoiding unnecessary ICD implantations.
Structured PICO
Does a dynamic clinical management approach safely reduce unnecessary ICD implantations in LQTS patients compared to strict risk score-based recommendations?
P
Population
Almost 1000 genotyped long QT syndrome (LQTS) patients
I
Intervention
Dynamic clinical management approach including yearly reassessment (ECG, 24h Holter, exercise stress test) and therapy optimization (adding left cardiac sympathetic denervation, mexiletine, or ICD to standard beta-blocker therapy)
C
Comparator
Strict adherence to risk scores (e.g., 1-2-3 LQTS score or M-FACT) for ICD implantation
O
Outcome
Death, arrhythmic events, and ICD shockshard clinical
A dynamic clinical management approach with yearly reassessment and therapy optimization safely reduces unnecessary ICD implantations in LQTS patients, challenging current guideline recommendations.
Limitations
- The M-FACT score was used as a proxy for other risk scores rather than being the primary target of validation
- Findings are based on a retrospective analysis of a previously published cohort
Abstract
Abstract This essay stems from a controversial recommendation present in the 2022 European Guidelines which indicated the appropriateness of considering an implantable cardioverter defibrillator (ICD) implant even for still asymptomatic long QT syndrome (LQTS) patients deemed to be at high risk by the 1–2–3 LQTS score based on QTc and genotype calculated prior to the institution of therapy. As 15 years ago, we also had proposed, but never used, a risk score called M-FACT to identify patients at high risk of an appropriate ICD shock, we felt the responsibility of assessing what would have happened to our patients if we had rigorously used that score. We performed a study recently published in the European Heart Journal which brought to general attention two concepts important for clinical management. One is that all LQTS patients should be seen at least once a year for a reassessment of arrhythmic risk based on standard electrocardiogram, 12-lead 24 h Holter recording and an exercise stress test. The other is that, based on these yearly visits, we perform ‘therapy optimization’ by adding to the standard β-blocker therapy either left cardiac sympathetic denervation or mexiletine or an ICD implant. On almost 1000 LQTS patients, all genotyped, this dynamic approach was accompanied by not a single death, few events, and out of 142 patients who should have received an ICD based on the score, only 22 did and only 3 had an ICD shock. These data and concepts call for a reconsideration of the recommendation made by the guidelines.