Three-Dimensional Electroanatomic Voltage Mapping Increases Accuracy of Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Abstract

Background— Three-dimensional electroanatomic voltage mapping offers the potential to identify low-voltage areas that correspond to regions of right ventricular (RV) myocardial loss and fibrofatty replacement in patients with arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). Methods and Results— Thirty-one consecutive patients (22 men and 9 women; mean age, 30.8±7 years) who fulfilled the criteria of the Task Force of the European Society of Cardiology and International Society and Federation of Cardiology (ESC/ISFC) for ARVC/D diagnosis after noninvasive clinical evaluation underwent further invasive study including RV electroanatomic voltage mapping and endomyocardial biopsy (EMB) to validate the diagnosis. Multiple RV endocardial, bipolar electrograms (175±23) were sampled during sinus rhythm. Twenty patients (group A; 65%) had an abnormal RV electroanatomic voltage mapping showing ≥1 area (mean 2.25±0.7) with low-voltage values (bipolar electrogram amplitude <0.5 mV), surrounded by a border zone (0.5 to 1.5 mV) that transitioned into normal myocardium (>1.5 mV). Low-voltage electrograms appeared fractionated with significantly prolonged duration and delayed activation. In 11 patients (group B; 35%), electroanatomic voltage mapping was normal, with preserved electrogram voltage (4.4±0.7 mV) and duration (37.2±0.9 ms) throughout the RV. Low-voltage areas in patients from group A corresponded to echocardiographic/angiographic RV wall motion abnormalities and were significantly associated with myocyte loss and fibrofatty replacement at EMB ( P <0.0001) and familial ARVC/D ( P <0.0001). Patients from group B had sporadic disease and histopathological evidence of inflammatory cardiomyopathy ( P <0.0001). During the time interval from onset of symptoms to the invasive study, 11 patients (55%) with electroanatomic low-voltage regions received an implantable cardioverter/defibrillator because of life-threatening ventricular arrhythmias, whereas all but 1 patient with a normal voltage map remained stable on antiarrhythmic drug therapy ( P =0.02). Conclusions— Three-dimensional electroanatomic voltage mapping enhanced accuracy for diagnosing ARVC/D (1) by demonstrating low-voltage areas that were associated with fibrofatty myocardial replacement and (2) by identifying a subset of patients who fulfilled ESC/ISFC Task Force diagnostic criteria but showed a preserved electrogram voltage, an inflammatory cardiomyopathy mimicking ARVC/D, and a better arrhythmic outcome.

Clinical utility gene card for: arrhythmogenic right ventricular cardiomyopathy (ARVC)

“…22 Among the new imaging tools, endocardial voltage mapping has the ability to identify low-amplitude electrical signals, that is, electroanatomic scar areas, which reflect myocardial replaced tissue in the ventricular walls. 23 Contrast-enhancement-cardiac magnetic resonance is less sensitive to identify right ventricular scar lesion but is the only tool able to detect early subepicardial LV involvement that can be the first manifestation of the disease in otherwise functionally normal hearts. 24 3.1.3 How is the cost effectiveness of alternative diagnostic methods to be judged?…”

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia: Lessons Learned

“…A detailed RV endocardial voltage map is created in sinus rhythm using the standard 0.5-1.5 mV voltage cutoffs to define the endocardial substrate as previously discussed [13,22] . Special attention is focused on the periannular area and any identified low-voltage areas to ensure adequate sampling has occurred [23][24][25] . Occasionally, it can be technically challenging to perform catheter manipulation in the periannular tricuspid valve region.…”

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Risk Stratification and Therapy

“…Ideally, the diagnosis should be based on the results of multiple non invasive/invasive tests utilizing the major and minor criteria stipulated by the international task force (35). Recently, electrocardiographic depolarization/repolarization abnormalities (36) and electroanatomic voltage mapping (37) have been reported to have a high diagnostic value.…”