Advertisement
Research Article|Articles in Press

Bilateral Cardiac Sympathetic Denervation in Patients with Congenital Long QT Syndrome

  • Kathryn E. Tobert
    Affiliations
    Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN, USA
    Search for articles by this author
  • J. Martijn Bos
    Affiliations
    Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN, USA

    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
    Search for articles by this author
  • Christopher Moir
    Affiliations
    Department of Surgery, Mayo Clinic, Rochester, MN, USA
    Search for articles by this author
  • Stephanie F. Polites
    Affiliations
    Department of Surgery, Mayo Clinic, Rochester, MN, USA
    Search for articles by this author
  • Michael J. Ackerman
    Correspondence
    Address for Correspondence: Michael J. Ackerman, MD, PhD Mayo Clinic Windland Smith Rice Genetic Heart Rhythm Clinic and the Windland Smith Rice Sudden Death Genomics Laboratory Guggenheim 501, Mayo Clinic, 200 First Street SW, Rochester, MN 55905
    Affiliations
    Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN, USA

    Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA

    Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, MN, USA
    Search for articles by this author

      ABSTRACT

      Background

      Long QT syndrome (LQTS) is a potentially lethal, yet treatable genetic heart disease for which left cardiac sympathetic denervation (LCSD) is a class I recommendation. Recent reports have suggested bilateral CSD (BiCSD) as the initial surgical denervation therapy in LQTS.

      Objective

      To determine the frequency and settings in which BiCSD was used in a tertiary referral center with expertise in LCSD.

      Methods

      We performed retrospective review of 234 LCSD patients/1,638 (14%) LQTS patients who underwent sympathetic denervation at our institution to identify the subset with BiCSD. Cardiac events (CEs) before LCSD, after LCSD, and after completion of BiCSD were recorded and defined as being an appropriate ICD shock, arrhythmic syncope or sudden cardiac arrest.

      Results

      Only 11 patients (4.7%, 6[55%] females) had BiCSD at our institution. Patients who received BiCSD trended towards being younger at diagnosis (6±15 vs. 14±13 years, p=0.06) and being more likely to be symptomatic (73% vs. 53%, p=0.07) than the larger LCSD-only cohort. Continued CEs post-LCSD (3.8 CEs per patient on average) was the predominant determinant to return for BiCSD. Over 60 combined years of follow-up, 4 patients have not had a post-BiCSD CE, while the other 7 patients average 3.6 non-lethal CEs.

      Conclusions

      Less than 5% of all patients receiving denervation therapy underwent BiCSD. When BiCSD was chosen, it was almost always done in a staged sequential manner starting with LCSD first and when driven by the arrhythmogenicity of the LQTS substrate, despite otherwise optimized guideline-directed therapies.

      Abbreviations:

      BiCSD (Bilateral cardiac sympathetic denervation), CE (Cardiac event), LCSD (Left cardiac sympathetic denervation), LQTS (Long QT syndrome), RCSD (Right cardiac sympathetic denervation), SCA (Sudden cardiac arrest), SCD (Sudden cardiac death)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Heart Rhythm
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      REFERENCES

        • Schwartz PJ
        • Stramba-Badiale M
        • Crotti L
        • et al.
        Prevalence of the congenital long-QT syndrome.
        Circulation. 2009; 120: 1761-1767
        • Moss AJ
        • McDonald J
        Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome.
        N Engl J Med. 1971; 285: 903-904
        • Collura CA
        • Johnson JN
        • Moir C
        • Ackerman MJ
        Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery.
        Heart Rhythm. 2009; 6: 752-759
        • Bos JM
        • Bos KM
        • Johnson JN
        • Moir C
        • Ackerman MJ
        Left cardiac sympathetic denervation in long QT syndrome: analysis of therapeutic nonresponders.
        Circ Arrhythm Electrophysiol. 2013; 6: 705-711
        • Akkuş M
        • Seyrek Y
        • Kafalı HC
        • Ergül Y
        Bilateral cardiac sympathetic denervation in children with long-QT syndrome and catecholaminergic polymorphic ventricular tachycardia.
        J Electrocardiol. 2020; 61: 32-36
        • Ajijola OA
        • Vaseghi M
        • Mahajan A
        • Shivkumar K
        Bilateral cardiac sympathetic denervation: why, who and when?.
        Expert Rev Cardiovasc Ther. 2012; 10: 947-949
        • Niaz T
        • Bos JM
        • Sorensen KB
        • Moir C
        • Ackerman MJ
        Left Cardiac Sympathetic Denervation Monotherapy in Patients With Congenital Long QT Syndrome.
        Circ Arrhythm Electrophysiol. Dec 2020; 13e008830
        • Priori SG
        • Wilde AA
        • Horie M
        • et al.
        HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes.
        Heart Rhythm. 2013; 10: 1932-1963
        • Te Riele AS
        • Ajijola OA
        • Shivkumar K
        • Tandri H
        Role of Bilateral Sympathectomy in the Treatment of Refractory Ventricular Arrhythmias in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy.
        Circ Arrhythm Electrophysiol. 2016; 9e003713
        • Dusi V
        • Pugliese L
        • De Ferrari GM
        • et al.
        Left Cardiac Sympathetic Denervation for Long QT Syndrome: 50 Years' Experience Provides Guidance for Management.
        JACC Clin Electrophysiol. 2022; 8: 281-294
        • Rohatgi RK
        • Sugrue A
        • Bos JM
        • Cannon BC
        • Asirvatham SJ
        • Moir C
        • Owen HJ
        • Bos KM
        • Kruisselbrink T
        • Ackerman MJ
        Contemporary outcomes in patients with long QT syndrome.
        J Am Coll Cardiol. 2017; 70: 453-462
      1. Martinez K, Sahej Bains B, John R. Giudicessi M, PhD, J. Martijn Bos M, PhD, Raquel Neves M, Michael J. Ackerman M, PhD Spectrum and Prevalence of Side Effects and Complications with Guideline Directed Therapies for Congenital Long QT Syndrome. Heart Rhythm 2022; 19(10):1666-1672.