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

• Published in: PloS one Vol. 9; no. 7; p. e99125
• Main Authors: Finlay, Malcolm C, Ahmed, Akbar K, Sugrue, Alan, Bhar-Amato, Justine, Quarta, Giovanni, Pantazis, Antonis, Ciaccio, Edward J, Syrris, Petros, Sen-Chowdhry, Srijita, Ben-Simon, Ron, Chow, Anthony W, Lowe, Martin D, Segal, Oliver R, McKenna, William J, Lambiase, Pier D
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 11-07-2014; Public Library of Science (PLoS)
• Subjects: Abnormalities; Adult; Aged; Arrhythmogenic Right Ventricular Dysplasia - diagnosis; Arrhythmogenic Right Ventricular Dysplasia - physiopathology; Benign; Biology and Life Sciences; Cardiac arrhythmia; Cardiomyopathy; Comparative analysis; Conduction; Correlation analysis; Deoxyribonucleic acid; Diagnosis, Differential; DNA; Echocardiography; EKG; Electrocardiography; Endocardium - physiopathology; Event-related potentials; Family medical history; Female; Gene mapping; Genetic testing; Genetics; Heart; Heart Conduction System - physiopathology; Heart Rate; Heart Ventricles - physiopathology; Humans; Kinetics; Male; Mapping; Medical diagnosis; Medicine and Health Sciences; Middle Aged; Mutation; Myocardial diseases; Parameter identification; Parameters; Patients; Refractory period; Science; Steady state; Tachycardia; Tachycardia, Ventricular - diagnosis; Tachycardia, Ventricular - physiopathology; Task forces; Ventricle; Ventricular Premature Complexes - diagnosis; Ventricular Premature Complexes - physiopathology; United Kingdom > UK; New York; United States > US
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0099125

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.
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.
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.
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.