492 Role of echocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy: comparison with Magnetic Resonance imaging

Abstract

Background: The arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary heart disease of unclear etiopathogenesis, characterized by a gradual loss of myocites which are replaced by fibro-fatty tissue and consequent right ventricular (RV) dilation and dysfunction. The clinical course is characterized by arrhythmias, sudden death and heart failure. Echocardiography (ECHO) may be useful to evaluate right ventricular size and function, which are important major and minor criteria for the diagnosis of ARVC, but since structural abnormalities are slight or moderate in most cases, they can be easily overlooked. Recently several studies have investigated the role of other diagnostic techniques such as computed tomography (CT) or magnetic resonance imaging (MRI) to obtain more specific evidence of the disease. Aim of the study: To evaluate the role of ECHO in the diagnostic pathway of ARVC in a selected cohort of patients undergoing MRI. Methods: We retrospectively analyzed 78250 MRI performed in MRI lab of L’Aquila University. One hundred and fifty three patients (pts) underwent MRI for suspected ARVC. In 108 (70,5%) was performed a color Doppler ECHO. As echocardiographic findings of ARVC we considered: dilation, kinetic alterations and systolic dysfunction of the RV. According to MRI data we considered as probable diagnosis the detection of at least two of the following criteria: dilation, dyskinesis, adipose substitution. Results: MRI confirmed diagnosis in 18/46 (40%) pts who had a positive ECHO for ARVC, whereas it was positive in 6/62 pts (9%) (p<0,001) with a non significant echo. RV dilation was present in 25 (54%) pts with and in 8 (12%) without positive ECHO (p< 0,0001); adipose substitution was present in 15 (32%) pts with and in 10 (16%) without positive ECHO (p=0,07); kinetic alterations were present in 16 pts (35%) with and in 7 (11%) without positive ECHO (p<0,001). Conclusions: Echocardiography may be an useful screening method for ARVC, as it is confirmed by MRI. It can clearly distinguish slight forms of the disease from severe ones, and it can give useful informations for further diagnostic exams. In facts, only 9% of pts with a non significant ECHO had a later positive MRI, without significant clinical symptoms.