Abstract
Background
Left cardiac sympathetic denervation (LCSD) is an effective anti-fibrillatory, minimally invasive therapy for patients with either LQTS or CPVT. As a pre-ganglionic fiber resection, re-growth or re-innervation is not possible. However, if a suboptimal CSD was performed, chances for post-LCSD breakthrough cardiac events (BCEs) are increased.
Purpose
To examine the prevalence and outcomes of patients requiring a re-do LCSD.
Methods
We performed a retrospective review of the 251 LQTS and CPVT patients who underwent CSD (mostly LCSD) at our institution to identify the subset referred for additional CSD because of recurrent BCEs after their primary LCSD that was performed elsewhere. Clinical data on symptomatic status prior to diagnosis and BCEs after first surgery as well as the surgical reports were reviewed to determine the reasons for the repeat procedure.
Results
Among the 15 patients (6% overall, 6 female; 13 LQTS, 2 CPVT) referred for additional CSD, 3 patients (20%) had a re-do LCSD performed instead of a sequential RCSD because of incomplete resection with the primary LCSD. Patient 1 is a male with Jervell-and-Lange Nielsen Syndrome (JLNS; KCNQ1-K421fs9X; KCNQ1-N365H), who first experienced syncope with torsades at age 3 after which a primary bilateral CSD was performed elsewhere. He had 2 BCEs and at age 12 underwent re-do LCSD, where his left-sided T2-T4 sympathetic chain with remnants of T1 were found to be intact. Patient 2 is a male who had 3 cardiac events prior to diagnosis (genotype negative LQTS) and LCSD at age 3. Following 3 BCEs, he underwent re-do LCSD at age 5 where scarring of T3 and intact stellate ganglion were found. Patient 3 is an adult female with CPVT (RYR2-deletion exon 3) who had multiple syncopal events, out-of-hospital arrest (leading to ICD) and 2 ICD-storms before primary bilateral CSD. Re-do LCSD was performed after identifying an intact left stellate ganglion and T2.
Conclusions
Before performing a primary bilateral CSD, proceeding to a RCSD after post-LCSD BCEs, or doing a videoscopic LCSD in the first place, it must be recognized that LCSD's success hinges on fully resecting the lower half of the stellate ganglion and the sympathetic chain through T4. Anything less is a partial denervation and likely ineffective procedure.
Funding Acknowledgement
Type of funding source: None
Stellate Ganglion Blockade and Left Cardiac Sympathetic Denervation With Left Stellate Ganglionectomy in a Patient With Refractory Electrical Storm: A Case Report
