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Abstract
AimsThe 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.Methods22 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.ResultsDefinite 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.ConclusionsSignificant 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.
Highlights
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a disease of cardiomyocyte adhesion; initial myocyte slippage and loss of gap junction integrity is followed by more overt structural changes, with characteristic fibrofatty replacement of cardiomyocytes[1]
We have demonstrated significant dynamic conduction and repolarization differences between desmoplakin mutation carriers and controls, but there has been no direct comparison of the dynamic electrophysiological changes in early ARVC versus patients with outflow tract ectopy (RVOT ectopy)[6]
Absolute measurements of surface ECG inscriptions might be very sensitive to the patient-specific placement of the pacing electrode, our analysis focused on measurements of change from steady state pacing following premature stimuli, guided by the observations of significant changes in intracardiac activation and repolarization
Summary
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a disease of cardiomyocyte adhesion; initial myocyte slippage and loss of gap junction integrity is followed by more overt structural changes, with characteristic fibrofatty replacement of cardiomyocytes[1]. These early manifestations of ARVC pose an important diagnostic challenge, especially in differentiating benign outflow tract ventricular ectopy from ARVC, as sudden death may occur in the concealed phase before structural changes appear and an early manifestation may be right ventricular (RV) ectopy[2,3]. Several elegant pathogenic mechanisms have been proposed that can explain early arrhythmias in ARVC without overt structural disease
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