Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients

Jeremy P. Moore, Kevin M. Shannon, Roberto G. Gallotti, Christopher J. McLeod, Anca Chiriac, Edward P. Walsh, Narayanswami Sreeram, Akash R. Patel, Natasja M. De Groot, Johannes von Alvensleben, Seshadri Balaji, David S. Frankel, Christina Y. Miyake, James C. Perry, Kalyanam Shivkumar

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objectives: To determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. Methods: This was a retrospective study involving 11 congenital heart disease centers. Results: A total of 24 patients (median age 17 [interquartile range: 11 to 37] years; age range 1 to 68 years; 42% men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50%). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [interquartile range: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92%). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years. Conclusions: VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.

Original languageEnglish (US)
JournalJACC: Clinical Electrophysiology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Ebstein Anomaly
Catheter Ablation
Cardiac Arrhythmias
Tricuspid Valve
Cicatrix
Ventricular Premature Complexes
Heart Ventricles
Myocardium
Syncope
Ventricular Fibrillation
Ventricular Tachycardia
Sudden Death
Heart Arrest
Heart Diseases
Retrospective Studies
Recurrence

Keywords

  • catheter ablation
  • congenital heart disease
  • Ebstein's anomaly
  • sudden cardiac death
  • ventricular tachycardia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Moore, J. P., Shannon, K. M., Gallotti, R. G., McLeod, C. J., Chiriac, A., Walsh, E. P., ... Shivkumar, K. (Accepted/In press). Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients. JACC: Clinical Electrophysiology. https://doi.org/10.1016/j.jacep.2018.05.009

Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients. / Moore, Jeremy P.; Shannon, Kevin M.; Gallotti, Roberto G.; McLeod, Christopher J.; Chiriac, Anca; Walsh, Edward P.; Sreeram, Narayanswami; Patel, Akash R.; De Groot, Natasja M.; von Alvensleben, Johannes; Balaji, Seshadri; Frankel, David S.; Miyake, Christina Y.; Perry, James C.; Shivkumar, Kalyanam.

In: JACC: Clinical Electrophysiology, 01.01.2018.

Research output: Contribution to journalArticle

Moore, JP, Shannon, KM, Gallotti, RG, McLeod, CJ, Chiriac, A, Walsh, EP, Sreeram, N, Patel, AR, De Groot, NM, von Alvensleben, J, Balaji, S, Frankel, DS, Miyake, CY, Perry, JC & Shivkumar, K 2018, 'Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients', JACC: Clinical Electrophysiology. https://doi.org/10.1016/j.jacep.2018.05.009
Moore, Jeremy P. ; Shannon, Kevin M. ; Gallotti, Roberto G. ; McLeod, Christopher J. ; Chiriac, Anca ; Walsh, Edward P. ; Sreeram, Narayanswami ; Patel, Akash R. ; De Groot, Natasja M. ; von Alvensleben, Johannes ; Balaji, Seshadri ; Frankel, David S. ; Miyake, Christina Y. ; Perry, James C. ; Shivkumar, Kalyanam. / Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients. In: JACC: Clinical Electrophysiology. 2018.
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abstract = "Objectives: To determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. Methods: This was a retrospective study involving 11 congenital heart disease centers. Results: A total of 24 patients (median age 17 [interquartile range: 11 to 37] years; age range 1 to 68 years; 42{\%} men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50{\%}). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [interquartile range: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92{\%}). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years. Conclusions: VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.",
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author = "Moore, {Jeremy P.} and Shannon, {Kevin M.} and Gallotti, {Roberto G.} and McLeod, {Christopher J.} and Anca Chiriac and Walsh, {Edward P.} and Narayanswami Sreeram and Patel, {Akash R.} and {De Groot}, {Natasja M.} and {von Alvensleben}, Johannes and Seshadri Balaji and Frankel, {David S.} and Miyake, {Christina Y.} and Perry, {James C.} and Kalyanam Shivkumar",
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T1 - Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients

AU - Moore, Jeremy P.

AU - Shannon, Kevin M.

AU - Gallotti, Roberto G.

AU - McLeod, Christopher J.

AU - Chiriac, Anca

AU - Walsh, Edward P.

AU - Sreeram, Narayanswami

AU - Patel, Akash R.

AU - De Groot, Natasja M.

AU - von Alvensleben, Johannes

AU - Balaji, Seshadri

AU - Frankel, David S.

AU - Miyake, Christina Y.

AU - Perry, James C.

AU - Shivkumar, Kalyanam

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Objectives: To determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. Methods: This was a retrospective study involving 11 congenital heart disease centers. Results: A total of 24 patients (median age 17 [interquartile range: 11 to 37] years; age range 1 to 68 years; 42% men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50%). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [interquartile range: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92%). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years. Conclusions: VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.

AB - Objectives: To determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. Methods: This was a retrospective study involving 11 congenital heart disease centers. Results: A total of 24 patients (median age 17 [interquartile range: 11 to 37] years; age range 1 to 68 years; 42% men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50%). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [interquartile range: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92%). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years. Conclusions: VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.

KW - catheter ablation

KW - congenital heart disease

KW - Ebstein's anomaly

KW - sudden cardiac death

KW - ventricular tachycardia

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