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(Circulation. 2005;111:3042-3050.)
© 2005 American Heart Association, Inc.

Arrhythmia/Electrophysiology

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

Domenico Corrado, MD, PhD; Cristina Basso, MD, PhD; Loira Leoni, MD; Barbara Tokajuk, MD; Barbara Bauce, MD, PhD; Gianfranco Frigo, MD; Giuseppe Tarantini, MD; Massimo Napodano, MD; Pietro Turrini, MD, PhD; Angelo Ramondo, MD; Luciano Daliento, MD; Andrea Nava, MD; Gianfranco Buja, MD; Sabino Iliceto, MD; Gaetano Thiene, MD

From the Division of Cardiology (D.C., L.L., B.T., B.B., G.T., M.N., G.F., P.T., A.R., L.D., A.N., G.B., S.I.) and Institute of Pathological Anatomy (C.B., G.T.), University of Padua Medical School, Padua, Italy.

Correspondence to Gaetano Thiene, MD, Istituto di Anatomia Patologica, Via A. Gabelli, 61-35121 Padova, Italy. E-mail cardpath{at}unipd.it

Received June 23, 2004; revision received February 14, 2005; accepted March 2, 2005.

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.

Key Words: cardiomyopathy • death, sudden • electrophysiology • mapping • myocarditis

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