Feasibility and Variability of Three Dimensional Echocardiography in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

• Published in: The American journal of cardiology 2006-03, Vol.97 (5), p.703-709
• Main Authors: Prakasa, Kalpana R., Dalal, Darshan, Wang, Jianwen, Bomma, Chandra, Tandri, Harikrishna, Dong, Jun, James, Cynthia, Tichnell, Crystal, Russell, Stuart D., Spevak, Philip, Corretti, Mary, Bluemke, David A., Calkins, Hugh, Abraham, Theodore P.
• Format: Article
• Language: English
• Subjects: Adult; Arrhythmogenic Right Ventricular Dysplasia - diagnostic imaging; Arrhythmogenic Right Ventricular Dysplasia - physiopathology; Biological and medical sciences; Cardiac dysrhythmias; Cardiology; Cardiology. Vascular system; Cardiovascular disease; Case-Control Studies; Echocardiography, Three-Dimensional; Feasibility Studies; Female; Heart; Humans; Image Interpretation, Computer-Assisted; Image Processing, Computer-Assisted; Magnetic Resonance Imaging, Cine; Male; Medical diagnosis; Medical imaging; Medical sciences; Middle Aged; Observer Variation; Prospective Studies; Radiography; Stroke Volume
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2005.11.020

Arrhythmogenic right ventricular dysplasia (ARVD/C) is a genetic cardiomyopathy characterized by fibrous fatty replacement of the right ventricular (RV) myocardium, leading to progressive RV failure and ventricular arrhythmias in young athletes. This study evaluated whether transthoracic, real-time, 3-dimensional echocardiography (3DE) can adequately assess RV morphology and function in ARVD/C by comparing 3DE with cardiac magnetic resonance (CMR), the current reference standard. Three-dimensional echocardiography was prospectively performed in 58 patients (23 with ARVD/C, 20 first-degree relatives with no ARVD/C, 8 with idiopathic ventricular tachycardia with no ARVD/C, and 7 healthy volunteers). All patients, except 15 patients with ARVD/C with implanted defibrillators, also underwent CMR. Three-dimensional echocardiography and CMR-derived RV volumes and ejection fractions were obtained by offline data analysis by blinded, independent observers. The mean age of the study group was 37 ± 11 years (30 men). The feasibility of 3DE was high, and analyzable images were obtained in all subjects. Three-dimensional echocardiography revealed a wide variety of RV morphologic abnormalities in ARVD/C. There was a good correlation between 3DE and CMR for RV end-systolic volume (r = 0.72, p = 0.0001), RV end-diastolic volume (r = 0.50, p = 0.0001), and the RV ejection fraction (r = 0.88, p = 0.001). We found high intraobserver and moderate interobserver correlations for 3DE estimations of volumes and ejection fractions. In conclusion, 3DE measurements of RV volumes and ejection fractions closely correlate with CMR values and may be useful in the follow-up of patients with ARVD/C.