• Users Online: 226
  • Print this page
  • Email this page
Submit article

 
Table of Contents
CASE REPORT
Year : 2020  |  Volume : 2  |  Issue : 2  |  Page : 95-97

Left sympathetic denervation as an effective therapy in a young patient with long QT syndrome


1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Date of Submission16-May-2020
Date of Decision06-May-2020
Date of Acceptance23-May-2020
Date of Web Publication21-Aug-2020

Correspondence Address:
Dr. Oruganti Sai Satish
Department of Cardiology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500 082, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ACCJ.ACCJ_11_20

Rights and Permissions
  Abstract 


Long QT syndrome (LQTS) is a rare inherited cardiac channelopathy with an abnormally prolonged QT interval with an increased predisposition for fatal ventricular arrhythmias in the presence of a structurally normal heart. Beta-blockers are the treatment of choice and are successful in 75%–80% of patients. Although beta-blocker therapy significantly reduces the risk of sudden cardiac death (SCD) in this population, it is not completely protective, and cardiac events still occur. Implantable cardioverter-defibrillator (ICD) therapy is highly successful in preventing SCD in high-risk LQTS patients. However, it is not without complications, especially in children and young adults, in whom the initial decision to implant an ICD carries long-term implications. In this subgroup of patients, the left cardiac sympathetic denervation showed promising evidence. Here, we describe a case of a 19-year-old female with refractory ventricular tachycardia due to LQTS, successfully managed by the left cardiac sympathetic denervation and beta-blockers alone.

Keywords: Left cardiac sympathetic denervation, long QT syndrome, sudden cardiac death


How to cite this article:
Sunitha A, Satish OS, Satya Gopal PS, Rao MA. Left sympathetic denervation as an effective therapy in a young patient with long QT syndrome. Ann Clin Cardiol 2020;2:95-7

How to cite this URL:
Sunitha A, Satish OS, Satya Gopal PS, Rao MA. Left sympathetic denervation as an effective therapy in a young patient with long QT syndrome. Ann Clin Cardiol [serial online] 2020 [cited 2020 Dec 6];2:95-7. Available from: http://www.onlineacc.org/text.asp?2020/2/2/95/292966




  Introduction Top


Long QT syndrome (LQTS) is a congenital disorder accompanied by a high incidence of ventricular arrhythmias and sudden cardiac death (SCD).[1] Beta-blockers are the treatment of choice and are successful in 75%–80% of patients, but 20%–25% continue to be symptomatic in spite of treatment.[2] Although implantable cardioverter-defibrillator (ICD) is now indicated for patients who had an aborted SCD, more debatable in the management of young patients, in whom device-related morbidity is likely to be more.[3] The arrhythmogenic potential of the left stellate ganglion has justified the therapeutic role of the left cardiac sympathetic denervation (LCSD ) in high-risk LQTS patients, those in whom cardiac events are not prevented by drugs, and ICD cannot be a practical choice; LCSD may be useful.[4] Here, we present a 19-year-old female with symptomatic refractory ventricular tachycardia (VT) due to LQTS and successfully managed by LCSD and propranolol therapy alone.


  Clinical Summary Top


A 19-year-old female presented to our institute with a history of recurrent episodes of palpitation with syncope precipitated by exertion and anxiety. A review of her medical documents showed that she was evaluated for syncope at 12 years of age in another hospital and was started on oral propranolol with the diagnosis of LQTS, despite which she continued to have episodes of syncope. On evaluation, her elder brother also had recurrent episodes of syncope from 8 years of age who had sudden death at the age of 10 years while playing and was also a diagnosed case of LQTS. There was no history of deafness in the patient or her brother. Our patient electrocardiogram (ECG) showed a baseline QTc of 580 ms with broad-based T waves suggestive of LQT1 [Figure 1]a. Exercise stress test-induced VT in stage 2 [Figure 2] and persistent, prolonged QTc and ventricular premature beats (VPBs) in the recovery phase. Holter analysis revealed frequent VPBs of different morphologies and runs of nonsustained VT [Figure 3]. Her Schwartz score was 8.5, which suggested a high-risk LQTS [Table 1]. Although ICD was a treatment option for her, because of her young age and long-term ICD-related issues and financial constraints, she was considered for LCSD. LCSD was done under general anesthesia through a supraclavicular extrapleural approach avoiding thoracotomy. The lower part of the stellate ganglion and the left sympathetic chain between T2 and T3 were resected. Immediate postoperative ECG showed a QTc of 361 ms [Figure 1]b. She was discharged on oral propranolol 5 mg eighth hourly and followed up at 2 months, 12 months, and 3 years postoperatively. The patient was clinically asymptomatic with a QTc of 400 ms, 380 ms at 2 and 12 months, respectively [Figure 1]c and [Figure 1]d. Exercise stress testing and Holter recording were uneventful during follow-up.
Figure 1: (a) Baseline electrocardiogram QTc = 580 ms. (b) Immediate postoperative electrocardiogram QTc = 361 ms. (c) Two-month follow-up electrocardiogram QTc = 400 ms. (d) Twelve-month follow-up electrocardiogram QTc = 380 ms

Click here to view
Figure 2: Exercise-induced right bundle branch block morphology ventricular tachycardia with northwest axis

Click here to view
Figure 3: Holter tracing showing multiple episodes of isolated ventricular premature beat's, ventricular bigeminy, trigeminy, couplets, and nonsustained ventricular tachycardia (arrow)

Click here to view
Table 1: Schwartz score

Click here to view



  Discussion Top


LQTS is a familial condition causing syncope and SCD by polymorphic VT (Torsades de pointes) in otherwise normal young people.[5] Clinical diagnosis is made from a combination of clinical/family history, and the ECG showing a QTc interval ≥470 ms in women and 460 ms in men in the absence of factors that are known to prolong the QT interval.

Schwartz scoring system helps to estimate the risk for LQTS. The included parameters are ECG (QTc baseline and 4th-min recovery during stress testing – >480 ms; Torsades de pointes, T wave alternans; notched T wave in three leads; and low heart rate for age), clinical history (syncope with stress/without stress and congenital deafness), and family history (family members with definite LQTS and unexplained SCD <age 30 years). Our patient had a high-risk Schwartz score of 8.5. Among all the parameters, stress-induced syncope and SCD in first-degree relatives are the most critical factors for making the diagnosis, and both parameters were present in our patient.

Beta-blockers should be initiated in symptomatic patients and also in asymptomatic with QTc >470 ms. Propranolol and nadolol were most widely used. Both the drugs showed superiority over metoprolol. Due to the nonavailability of nadolol, we have started oral propranolol 10 mg eighth hourly initially in our patient despite which she was symptomatic.

ICD therapy in LQTS is indicated for patients with resuscitated SCD (Class I indication) and with recurrent syncope due to ventricular arrhythmias while on beta-blockers (Class IIa indication).[6] ICD should be carefully selected in young patients due to device-related morbidities such as inappropriate shocks, generator replacements, dislodgment/fracture of leads, and change in capture/sensing/defibrillation thresholds and all. In the situation of financial constraints for ICD therapy, an alternative mode of management needs to be considered as done in our patient.

LCSD was first described by Moss in 1969, as an alternative therapy in patients who are symptomatic despite on beta-blockers/contraindication for them, or ICD cannot be considered in controlling the VT storms.[7] LCSD can also be used as primary prevention when drug compliance is poor. LCSD apart from eliminating the triggers due to sympathetic activation and also modifies the arrhythmogenic substrate by QT interval shortening.[8],[9] In our patient, QTc was normalized (baseline and stress testing), and the patient was asymptomatic over 3 years of follow-up after LCSD.

Recent studies reaffirmed the potential role of LCSD in reducing recurrent cardiac events. Schwartz et al. studied 147 patients with LQTS who were treated with LCSD. They observed the drop of mean annual cardiac event per rate per patient by 91%, and the percentage of patients with >5 cardiac events reduced from 55% to 8%. This suggested that the approach of LCSD achieves better outcomes and lower complication rates.[10] The risk-to-benefit ratio of ICD in younger patients should be carefully considered as LCSD significantly reduces fatal arrhythmic events.


  Conclusion Top


LCSD is a safe and effective treatment along with drug therapy to control refractory ventricular arrhythmias in patients with LQTS, especially in young children and adolescents in whom ICD therapy is of concern. However, as LCSD may not completely eliminate the risk of arrhythmic events, the choice of LCSD should be made on an individual basis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Neira V, Enriquez A, Simpson C, Baranchuk A. Update on long QT syndrome. J Cardiovasc Electrophysiol 2019;30:3068-78.  Back to cited text no. 1
    
2.
Moss AJ, Zareba W, Jackson Hall W, Schwartz PJ, Crampton RS, Benhorin J, et al. Effectiveness and limitations of beta-blocker therapy in congenital long QT syndrome. Circulation 2000;101:616-23.  Back to cited text no. 2
    
3.
Priori SG, Aliot E, Blomstrom-Lundqvist C, Bossaert L, Breithardt G, Brugada P, et al. Task force on sudden cardiac death of the European Society of Cardiology. Eur Heart J 2001;22:1374-450.  Back to cited text no. 3
    
4.
Dusi V, De Ferrari GM, Pugliese L, Schwartz PJ. Cardiac sympathetic denervation in channelopathies. Front Cardiovasc Med 2019;6:1-13.  Back to cited text no. 4
    
5.
Schwartz PJ, Crotti L, Insolia R. Long-QT syndrome: From genetics to management. Circ Arrhythm Electrophysiol 2012;5:868-77.  Back to cited text no. 5
    
6.
HRS/EHRE/APHRS Expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndrome. Heart Rhythm 2013;10:1932-63.  Back to cited text no. 6
    
7.
Lopez Ayala P, Sgro A, Drake TM, Phan K. Left cardiac sympathetic denervation in patients with long QT syndrome and catecholaminergic polymorphic ventricular tachycardia: A systematic review and meta regression. Eur Heart J 2019; 40: Supplement 1, ehz748.0593.  Back to cited text no. 7
    
8.
Schwartz PJ, Locati EH, Moss AJ, Crampton RS, Trazzi R, Ruberti U. Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report. Circulation 1991;84:503-11.  Back to cited text no. 8
    
9.
Li C, Hu D, Shang L, Ma S, Liu W, Li Y, et al. Surgical left cardiac sympathetic denervation for long QT syndrome: Effects on QT interval and heart rate. Heart Vessels 2005;20:137-41.  Back to cited text no. 9
    
10.
Schwartz PJ, Priori SG, Cerrone M, Spazzolini C, Odero A, Napolitano C, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004;109:1826-33.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Clinical Summary
Discussion
Conclusion
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed180    
    Printed25    
    Emailed0    
    PDF Downloaded31    
    Comments [Add]    

Recommend this journal