Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping & paced surface ECG analysis.

<h4>Aims</h4>The concealed phase of arrhythmogenic right ventricular cardiomyopathy (ARVC) may initially manifest electrophysiologically. No studies have examined dynamic conduction/repolarization kinetics to distinguish benign right ventricular outflow tract ectopy (RVOT ectopy) from AR...

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Main Authors: Malcolm C Finlay, Akbar K Ahmed, Alan Sugrue, Justine Bhar-Amato, Giovanni Quarta, Antonis Pantazis, Edward J Ciaccio, Petros Syrris, Srijita Sen-Chowdhry, Ron Ben-Simon, Anthony W Chow, Martin D Lowe, Oliver R Segal, William J McKenna, Pier D Lambiase
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0099125
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author Malcolm C Finlay
Akbar K Ahmed
Alan Sugrue
Justine Bhar-Amato
Giovanni Quarta
Antonis Pantazis
Edward J Ciaccio
Petros Syrris
Srijita Sen-Chowdhry
Ron Ben-Simon
Anthony W Chow
Martin D Lowe
Oliver R Segal
William J McKenna
Pier D Lambiase
author_facet Malcolm C Finlay
Akbar K Ahmed
Alan Sugrue
Justine Bhar-Amato
Giovanni Quarta
Antonis Pantazis
Edward J Ciaccio
Petros Syrris
Srijita Sen-Chowdhry
Ron Ben-Simon
Anthony W Chow
Martin D Lowe
Oliver R Segal
William J McKenna
Pier D Lambiase
author_sort Malcolm C Finlay
collection DOAJ
description <h4>Aims</h4>The concealed phase of arrhythmogenic right ventricular cardiomyopathy (ARVC) may initially manifest electrophysiologically. No studies have examined dynamic conduction/repolarization kinetics to distinguish benign right ventricular outflow tract ectopy (RVOT ectopy) from ARVC's early phase. We investigated dynamic endocardial electrophysiological changes that differentiate early ARVC disease expression from RVOT ectopy.<h4>Methods</h4>22 ARVC (12 definite based upon family history and mutation carrier status, 10 probable) patients without right ventricular structural anomalies underwent high-density non-contact mapping of the right ventricle. These were compared to data from 14 RVOT ectopy and 12 patients with supraventricular tachycardias and normal hearts. Endocardial & surface ECG conduction and repolarization parameters were assessed during a standard S1-S2 restitution protocol.<h4>Results</h4>Definite ARVC without RV structural disease could not be clearly distinguished from RVOT ectopy during sinus rhythm or during steady state pacing. Delay in Activation Times at coupling intervals just above the ventricular effective refractory period (VERP) increased in definite ARVC (43 ± 20 ms) more than RVOT ectopy patients (36 ± 14 ms, p = 0.03) or Normals (25 ± 16 ms, p = 0.008) and a progressive separation of the repolarisation time curves between groups existed. Repolarization time increases in the RVOT were also greatest in ARVC (definite ARVC: 18 ± 20 ms; RVOT ectopy: 5 ± 14, Normal: 1 ± 18, p<0.05). Surface ECG correlates of these intracardiac measurements demonstrated an increase of greater than 48 ms in stimulus to surface ECG J-point pre-ERP versus steady state, with an 88% specificity and 68% sensitivity in distinguishing definite ARVC from the other groups. This technique could not distinguish patients with genetic predisposition to ARVC only (probable ARVC) from controls.<h4>Conclusions</h4>Significant changes in dynamic conduction and repolarization are apparent in early ARVC before detectable RV structural abnormalities, and were present to a lesser degree in probable ARVC patients. Investigation of dynamic electrophysiological parameters may be useful to identify concealed ARVC in patients without disease pedigrees by using endocardial electrogram or paced ECG parameters.
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spelling doaj-art-1e087bbd5b4743619cf8d7ce3ea817952025-08-20T03:46:23ZengPublic Library of Science (PLoS)PLoS ONE1932-62032014-01-0197e9912510.1371/journal.pone.0099125Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.Malcolm C FinlayAkbar K AhmedAlan SugrueJustine Bhar-AmatoGiovanni QuartaAntonis PantazisEdward J CiaccioPetros SyrrisSrijita Sen-ChowdhryRon Ben-SimonAnthony W ChowMartin D LoweOliver R SegalWilliam J McKennaPier D Lambiase<h4>Aims</h4>The concealed phase of arrhythmogenic right ventricular cardiomyopathy (ARVC) may initially manifest electrophysiologically. No studies have examined dynamic conduction/repolarization kinetics to distinguish benign right ventricular outflow tract ectopy (RVOT ectopy) from ARVC's early phase. We investigated dynamic endocardial electrophysiological changes that differentiate early ARVC disease expression from RVOT ectopy.<h4>Methods</h4>22 ARVC (12 definite based upon family history and mutation carrier status, 10 probable) patients without right ventricular structural anomalies underwent high-density non-contact mapping of the right ventricle. These were compared to data from 14 RVOT ectopy and 12 patients with supraventricular tachycardias and normal hearts. Endocardial & surface ECG conduction and repolarization parameters were assessed during a standard S1-S2 restitution protocol.<h4>Results</h4>Definite ARVC without RV structural disease could not be clearly distinguished from RVOT ectopy during sinus rhythm or during steady state pacing. Delay in Activation Times at coupling intervals just above the ventricular effective refractory period (VERP) increased in definite ARVC (43 ± 20 ms) more than RVOT ectopy patients (36 ± 14 ms, p = 0.03) or Normals (25 ± 16 ms, p = 0.008) and a progressive separation of the repolarisation time curves between groups existed. Repolarization time increases in the RVOT were also greatest in ARVC (definite ARVC: 18 ± 20 ms; RVOT ectopy: 5 ± 14, Normal: 1 ± 18, p<0.05). Surface ECG correlates of these intracardiac measurements demonstrated an increase of greater than 48 ms in stimulus to surface ECG J-point pre-ERP versus steady state, with an 88% specificity and 68% sensitivity in distinguishing definite ARVC from the other groups. This technique could not distinguish patients with genetic predisposition to ARVC only (probable ARVC) from controls.<h4>Conclusions</h4>Significant changes in dynamic conduction and repolarization are apparent in early ARVC before detectable RV structural abnormalities, and were present to a lesser degree in probable ARVC patients. Investigation of dynamic electrophysiological parameters may be useful to identify concealed ARVC in patients without disease pedigrees by using endocardial electrogram or paced ECG parameters.https://doi.org/10.1371/journal.pone.0099125
spellingShingle Malcolm C Finlay
Akbar K Ahmed
Alan Sugrue
Justine Bhar-Amato
Giovanni Quarta
Antonis Pantazis
Edward J Ciaccio
Petros Syrris
Srijita Sen-Chowdhry
Ron Ben-Simon
Anthony W Chow
Martin D Lowe
Oliver R Segal
William J McKenna
Pier D Lambiase
Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
PLoS ONE
title Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
title_full Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
title_fullStr Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
title_full_unstemmed Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
title_short Dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping &amp; paced surface ECG analysis.
title_sort dynamic conduction and repolarisation changes in early arrhythmogenic right ventricular cardiomyopathy versus benign outflow tract ectopy demonstrated by high density mapping amp paced surface ecg analysis
url https://doi.org/10.1371/journal.pone.0099125
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