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Trigger Type and Breakthrough Cardiac Events in Inherited Arrhythmia Syndromes After Left Cardiac Sympathetic
Vanessa Karlinski Vizentin1, Iuri Ferreira Felix2, Sahej Bains3
1Windland Smith Rice Sudden Death Genomics Laboratory, Department of Molecular Pharmacology and Experimental Therapeutics, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: https://twitter.com/VizentinVanessa.
Background:
Left cardiac sympathetic denervation (LCSD) confers a strong antifibrillatory effect and provides significant therapeutic efficacy for patients with genetic heart diseases, especially long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). We hypothesize that LCSD's efficacy may differ among patients experiencing adrenergic (AD) vs nonadrenergic (non-AD) triggered symptoms before LCSD.
Objective:
In this study, we sought to evaluate the association between AD and non-AD triggered events and the subsequent risk of breakthrough cardiac events (BCEs) after LCSD.
Methods:
A retrospective review of all previously symptomatic patients with either LQTS or CPVT who underwent LCSD at our institution since 2005 was performed. Data from electronic medical records was abstracted for cardiac event (CE) trigger type (AD or non-AD) before and after LCSD. CEs and BCEs were defined as arrhythmogenic syncope, seizure, appropriate ventricular fibrillation-terminating therapies, and sudden cardiac arrest or death.
Results:
Among 154 pre-LCSD symptomatic patients (mean age at diagnosis: 15 ± 12 years; follow-up after LCSD: 5.9 ± 4.2 years) with LQTS (n = 105; 68%) or CPVT (n = 49; 32%), 49 patients (32%) had >1 BCE. Post-LCSD BCEs were significantly higher in patients with non-AD triggered CE (n = 30/56; 54%)) compared with patients with AD-triggered CE (n = 19/98; 19%; P < 0.0001). The subset analysis found fewest BCEs in LQT1 patients with AD triggers (OR: 0.24, P < 0.0001). To date, no BCEs have occurred in LQT2, LQT3, or LQT Minor patients with pre-LCSD AD-triggered CEs.
Conclusions:
There is a marked pre-LCSD phenotypic determinant of LCSD's therapeutic efficacy. Independently from underlying genotype, patients with adrenergic triggers associated with their pre-LCSD symptoms had a greater reduction in disease-mediated BCEs.
Insights
Left cardiac sympathetic denervation (LCSD) is more effective for patients with genetic heart conditions whose symptoms are triggered by adrenergic factors. Patients with non-adrenergic triggers experienced more breakthrough cardiac events after LCSD.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Left cardiac sympathetic denervation (LCSD) is a proven therapy for genetic heart diseases like long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT).
- The study investigates if LCSD's effectiveness varies based on whether symptoms are triggered by adrenergic (AD) or non-adrenergic (non-AD) factors before the procedure.
Purpose of the Study:
- To determine the association between AD and non-AD triggered events and the risk of breakthrough cardiac events (BCEs) following LCSD.
- To evaluate the impact of pre-LCSD symptom triggers on therapeutic outcomes in patients with LQTS and CPVT.
Main Methods:
- Retrospective review of patients with LQTS or CPVT who underwent LCSD since 2005.
- Abstraction of electronic medical records to identify cardiac event (CE) trigger types (AD vs. non-AD) before and after LCSD.
- Definition of CEs and BCEs included syncope, seizures, appropriate defibrillator therapies, and sudden cardiac arrest/death.
Main Results:
- Among 154 patients (LQTS: 105, CPVT: 49), 32% experienced BCEs post-LCSD.
- Patients with non-AD triggered CEs had significantly higher BCE rates (54%) compared to those with AD-triggered CEs (19%; P < 0.0001).
- LQT1 patients with AD triggers showed the fewest BCEs (OR: 0.24, P < 0.0001); no BCEs occurred in LQT2, LQT3, or LQT Minor patients with AD triggers.
Conclusions:
- Pre-LCSD symptom triggers are a significant determinant of LCSD's therapeutic efficacy.
- Patients with AD-triggered symptoms experience a greater reduction in disease-mediated BCEs, irrespective of their specific genotype.
- LCSD offers substantial benefit, particularly for patients with AD-triggered genetic arrhythmias.
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