ISMICS - Surgical Treatment In Patients With Cpvt And Long Qt Syndrome: Cardiac Sympathetic Denervation

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Surgical Treatment In Patients With Cpvt And Long Qt Syndrome: Cardiac Sympathetic Denervation
Gizem Kececi Ozgur, Jr., Dilarar Gursoy, Ayse Gul Ergonul, Can Hasdemir, Kutsal Turhan.
Ege University Faculty of Medicine, bornova, Turkey.

BACKGROUND:Catecholaminergic polymorphic ventricular tachycardia(CPVT) and long QT syndrome(LQTS) are channelopathies of genetic origin characterized by life-threatening ventricular arrhythmias triggered by increased sympathetic activity,eg,physical exercise and emotional stress.Treatment options include pharmacotherapy,implantable cardioverter-defibrillators(ICD),and cardiac sympathetic denervation.Cardiac sympathetic denervation is a surgical procedure emerging as a treatment option in patients with life-threatening arrhythmias despite medical therapy.In this study, we present our experience with videothoracoscopic cardiac sympathetic denervation in our institution. METHODS:Six patients who underwent videothoracoscopic cardiac sympathetic denervation due to recurrent ventricular fibrillation storm despite medical treatment in our clinic between 2008 and 2021 were reviewed retrospectively. RESULTS:Four of the patients were female and two were male, ages ranged from 18 to 39.There were diagnoses of CPVT in one case, LQTS in four cases, and LQTS/fBrugada syndrome in one case. ICD implantation was applied to all patients. VF attacks were detected in numbers ranging from 2 to 25 before the procedure. Videothoracoscopic left cardiac sympathetic denervation (LCTD) was performed in all patients. T2-T3-T4-T5 ganglia were excised and Kuntz nerve fibers were coagulated. In addition, right cardiac sympathetic denervation (RCTD) was applied after LCTD in one Patient with CPVT, because the shocks continued 3 months after LCTD. There were no perioperative and postoperative surgical complications in any of the patients. The Patient with bilateral sympathectomy had rare ICD shocks in the post-procedure period which could be easily controlled with oral Propafenone. VF-induced shocks ended in the patient with LQTS/Brugada syndrome. However, he died six weeks after the procedure due to resistant monomorphic VT. No cardiac events were observed after the procedure in other four patients who were operated for LQTS. Only one VF attack developed in one patient 5 years after the procedure. Afterwards no cardiac event was observed. CONCLUSIONS:Videothoracoscopic cardiac sympathetic denervation can be applied as a safe and effective treatment for recurrent VF attacks, especially in patients with long QT syndrome, despite the maximum tolerated dose of beta-blockers.
Tablo-1
PATİENT AGE SEX Presenting Symptoms Triggering Factors for Syncope/CA Diagnosis Medical Therapy Surgical Procedure LSCD -Indication Follow-Up
1 21 M Syncope Stress for Syncope CPVT = Catecholaminergic Polymorphic VT Propafenone LSCD+RSCD Recurrent VF X25 No cardiac event was observed under drug treatment in the postoperative period
2 25 F Syncope Stress for Syncope Long QT Syndrome Propranolol LSCD Recurrent VF X3 There is no any postoperative problems except for one shock due to VF.
3 39 F Syncope Stress for Syncope Long QT Syndrome Metoprolol LSCD Recurrent VF X4 No postoperative cardiac events
4 31 F Cardiac Arrest Stress for Syncope Long QT Syndrome Propranolol LSCD Recurrent VF X2 No postoperative cardiac events
5 23 F Cardiac Arrest Festival Activity Long QT Syndrome Propranolol LSCD Recurrent VF X3 No postoperative cardiac events
6 18 M Cardiac Arrest Stress for Cardiac Arrest Long QT Syndrome + Brugada Syndrome Ranolazine/Nadolol LSCD Recurrent VF X6 Shock from VF over but died from VT storm 6 weeks after LSCD

Tablo-1
PATİENT	AGE	SEX	Presenting Symptoms	Triggering Factors for Syncope/CA	Diagnosis	Medical Therapy	Surgical Procedure	LSCD -Indication	Follow-Up
1	21	M	Syncope	Stress for Syncope	CPVT = Catecholaminergic Polymorphic VT	Propafenone	LSCD+RSCD	Recurrent VF X25	No cardiac event was observed under drug treatment in the postoperative period
2	25	F	Syncope	Stress for Syncope	Long QT Syndrome	Propranolol	LSCD	Recurrent VF X3	There is no any postoperative problems except for one shock due to VF.
3	39	F	Syncope	Stress for Syncope	Long QT Syndrome	Metoprolol	LSCD	Recurrent VF X4	No postoperative cardiac events
4	31	F	Cardiac Arrest	Stress for Syncope	Long QT Syndrome	Propranolol	LSCD	Recurrent VF X2	No postoperative cardiac events
5	23	F	Cardiac Arrest	Festival Activity	Long QT Syndrome	Propranolol	LSCD	Recurrent VF X3	No postoperative cardiac events
6	18	M	Cardiac Arrest	Stress for Cardiac Arrest	Long QT Syndrome + Brugada Syndrome	Ranolazine/Nadolol	LSCD	Recurrent VF X6	Shock from VF over but died from VT storm 6 weeks after LSCD

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