Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation

Gaetano M. De Ferrari, Veronica Dusi, Carla Spazzolini, J. Martijn Bos, Dominic J. Abrams, Charles I. Berul, Lia Crotti, Andrew M. Davis, Michael Eldar, Maria Kharlap, Asaad Khoury, Andrew D. Krahn, Antoine Leenhardt, Christopher R. Moir, Attilio Odero, Louise Olde Nordkamp, Thomas Paul, Ferran Rosés I Noguer, Maria Shkolnikova, Jan TillArthur A M Wilde, Michael J. Ackerman, Peter J. Schwartz

Research output: Contribution to journalArticle

Abstract

Background-Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Blockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results-We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P

Original languageEnglish
Pages (from-to)2185-2193
Number of pages9
JournalCirculation
Volume131
Issue number25
DOIs
Publication statusPublished - 2015

Fingerprint

Sympathectomy
Implantable Defibrillators
Flecainide
Inborn Genetic Diseases
Patient Discharge
Syncope
Therapeutics
Polymorphic catecholergic ventricular tachycardia
Heart Arrest
Disease-Free Survival
Cardiac Arrhythmias
Shock
Survival Rate
Recurrence

Keywords

  • Adrenergic beta-antagonists
  • Arrhythmias
  • Cardiac
  • Death
  • Genetics
  • Sudden
  • Sympathetic nervous system

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine
  • Medicine(all)

Cite this

Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation. / De Ferrari, Gaetano M.; Dusi, Veronica; Spazzolini, Carla; Bos, J. Martijn; Abrams, Dominic J.; Berul, Charles I.; Crotti, Lia; Davis, Andrew M.; Eldar, Michael; Kharlap, Maria; Khoury, Asaad; Krahn, Andrew D.; Leenhardt, Antoine; Moir, Christopher R.; Odero, Attilio; Nordkamp, Louise Olde; Paul, Thomas; Rosés I Noguer, Ferran; Shkolnikova, Maria; Till, Jan; Wilde, Arthur A M; Ackerman, Michael J.; Schwartz, Peter J.

In: Circulation, Vol. 131, No. 25, 2015, p. 2185-2193.

Research output: Contribution to journalArticle

De Ferrari, GM, Dusi, V, Spazzolini, C, Bos, JM, Abrams, DJ, Berul, CI, Crotti, L, Davis, AM, Eldar, M, Kharlap, M, Khoury, A, Krahn, AD, Leenhardt, A, Moir, CR, Odero, A, Nordkamp, LO, Paul, T, Rosés I Noguer, F, Shkolnikova, M, Till, J, Wilde, AAM, Ackerman, MJ & Schwartz, PJ 2015, 'Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation', Circulation, vol. 131, no. 25, pp. 2185-2193. https://doi.org/10.1161/CIRCULATIONAHA.115.015731
De Ferrari, Gaetano M. ; Dusi, Veronica ; Spazzolini, Carla ; Bos, J. Martijn ; Abrams, Dominic J. ; Berul, Charles I. ; Crotti, Lia ; Davis, Andrew M. ; Eldar, Michael ; Kharlap, Maria ; Khoury, Asaad ; Krahn, Andrew D. ; Leenhardt, Antoine ; Moir, Christopher R. ; Odero, Attilio ; Nordkamp, Louise Olde ; Paul, Thomas ; Rosés I Noguer, Ferran ; Shkolnikova, Maria ; Till, Jan ; Wilde, Arthur A M ; Ackerman, Michael J. ; Schwartz, Peter J. / Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation. In: Circulation. 2015 ; Vol. 131, No. 25. pp. 2185-2193.
@article{b344d41e32fc416797373beeafadd5c8,
title = "Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation",
abstract = "Background-Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Blockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results-We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24{\%}) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87{\%} and 81{\%}. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100{\%} to 32{\%} (P",
keywords = "Adrenergic beta-antagonists, Arrhythmias, Cardiac, Death, Genetics, Sudden, Sympathetic nervous system",
author = "{De Ferrari}, {Gaetano M.} and Veronica Dusi and Carla Spazzolini and Bos, {J. Martijn} and Abrams, {Dominic J.} and Berul, {Charles I.} and Lia Crotti and Davis, {Andrew M.} and Michael Eldar and Maria Kharlap and Asaad Khoury and Krahn, {Andrew D.} and Antoine Leenhardt and Moir, {Christopher R.} and Attilio Odero and Nordkamp, {Louise Olde} and Thomas Paul and {Ros{\'e}s I Noguer}, Ferran and Maria Shkolnikova and Jan Till and Wilde, {Arthur A M} and Ackerman, {Michael J.} and Schwartz, {Peter J.}",
year = "2015",
doi = "10.1161/CIRCULATIONAHA.115.015731",
language = "English",
volume = "131",
pages = "2185--2193",
journal = "Circulation",
issn = "0009-7322",
publisher = "Lippincott Williams and Wilkins",
number = "25",

}

TY - JOUR

T1 - Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation

AU - De Ferrari, Gaetano M.

AU - Dusi, Veronica

AU - Spazzolini, Carla

AU - Bos, J. Martijn

AU - Abrams, Dominic J.

AU - Berul, Charles I.

AU - Crotti, Lia

AU - Davis, Andrew M.

AU - Eldar, Michael

AU - Kharlap, Maria

AU - Khoury, Asaad

AU - Krahn, Andrew D.

AU - Leenhardt, Antoine

AU - Moir, Christopher R.

AU - Odero, Attilio

AU - Nordkamp, Louise Olde

AU - Paul, Thomas

AU - Rosés I Noguer, Ferran

AU - Shkolnikova, Maria

AU - Till, Jan

AU - Wilde, Arthur A M

AU - Ackerman, Michael J.

AU - Schwartz, Peter J.

PY - 2015

Y1 - 2015

N2 - Background-Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Blockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results-We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P

AB - Background-Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Blockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results-We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P

KW - Adrenergic beta-antagonists

KW - Arrhythmias

KW - Cardiac

KW - Death

KW - Genetics

KW - Sudden

KW - Sympathetic nervous system

UR - http://www.scopus.com/inward/record.url?scp=84935448774&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84935448774&partnerID=8YFLogxK

U2 - 10.1161/CIRCULATIONAHA.115.015731

DO - 10.1161/CIRCULATIONAHA.115.015731

M3 - Article

C2 - 26019152

AN - SCOPUS:84935448774

VL - 131

SP - 2185

EP - 2193

JO - Circulation

JF - Circulation

SN - 0009-7322

IS - 25

ER -