Noninvasive Diagnosis of Electroanatomic Abnormalities in Arrhythmogenic Right Ventricular Cardiomyopathy

Abstract

Background-The diagnostic reliability and pathophysiologic relevance of different noninvasive diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) are undefined. We tested the association between noninvasive diagnostic criteria for ARVC and the presence of low-voltage areas (LVAs) detected at electroanatomic voltage mapping (EAM). Methods and Results-Noninvasive diagnostic criteria, including ECG, signal-averaged ECG (SAECG), and cardiac magnetic resonance (CMR) criteria, were compared with the presence and location of LVAs detected at right ventricular (RV) EAM in 17 patients (9 men) aged 50Ϯ16 years with biopsy specimen-proven ARVC. LVAs were found in 15 (88%) patients. Patients with surface ECG abnormalities showed a higher degree of RV involvement than those without ECG abnormalities (number of LVAs, 1.8Ϯ0.5 versus 0.9Ϯ0.6, respectively; PϽ0.01). A significant association was found between SAECG abnormalities and LVAs in the RV outflow tract (Pϭ0.03) but not between SAECG parameters and LVAs in other RV regions. Among CMR findings, RV delayed enhancement was more significantly associated with the distribution of LVAs (free wall, PϽ0.01; outflow tract, PϽ0.01; posteroinferior wall, Pϭ0.02). Regional RV dysfunction also showed a good correlation with LVAs, with the most significant association being found with the free wall (Pϭ0.01), whereas RV fat infiltration at CMR was not correlated with LVAs. Conclusion-In patients with ARVC, SAECG abnormalities correlate with the presence of LVAs selectively in the RV outflow tract, whereas surface ECG abnormalities are associated with a more diffuse RV involvement. Myocardial delayed enhancement is the CMR finding more strongly associated with LVAs, thus supporting the appropriateness of its inclusion among diagnostic criteria for ARVC. (Circ Arrhythm Electrophysiol. 2010;3:632-638.)

Imaging Study of Ventricular Scar in Arrhythmogenic Right Ventricular Cardiomyopathy

“…Previous studies demonstrated that a fibrofatty ventricular scar can be visualized as RV DCE in approximately one third to one half of ARVC patients. 16,31,34 Tandri et al 31 first reported RV DCE in 8 (67%) of 12 of patients with ARVC and demonstrated its relation to inducibility of sustained monomorphic VT at electrophysiological testing and fibrofatty myocardial changes at EMB. Sen-Chowdhry et al 16 reported unequivocal RV DCE in 13 (65%) of 20 patients who fulfilled task force diagnostic criteria for ARVC and were desmosomal gene mutation carriers.…”

“…Sen-Chowdhry et al 16 reported unequivocal RV DCE in 13 (65%) of 20 patients who fulfilled task force diagnostic criteria for ARVC and were desmosomal gene mutation carriers. Santangeli et al 34 found RV DCE in 8 (53%) of 15 of patients with an ARVC diagnosis based on the task force criteria. The lack of RV DCE in a significant proportion of patients fulfilling the diagnostic criteria of ARVC was explained by early disease variants with a small amount of fibrofatty tissue or even by pure fatty variants of the disease undetectable by DCE techniques.…”

Circulation: Cardiovascular Imaging Editors' Picks

“…In extending this correlation to RV regions of delayed gadolinium enhancement hyperenhancement by cardiac magnetic resonance, this work underscores the critical importance of delayed gadolinium enhancement in evaluation of the patient suspected of having ARVC and offers hope for more reliable cardiac magnetic resonance-based diagnosis. 23 …”

Arrhythmogenic Cardiomyopathy

“…In a study of 17 patients with biopsy-proven disease, abnormalities on the signal-averaged ECG correlated most closely with lowvoltage areas in the right ventricular outflow tract, whereas surface ECG abnormalities were associated with more diffuse right ventricular involvement. 29 Delayed gadolinium enhancement on magnetic resonance imaging was also highly associated with the distribution of low-voltage areas. Percutaneous epicardial mapping has defined electroanatomic correlates of pathological observations, indicating that myocardial involvement in arrhythmogenic cardiomyopathy progresses from epicardium to endocardium.…”