Value and limitations of 2-dimensional echocardiography in the identification of arrhythmogenic right ventricular dysplasia

The aim of this study was to evaluate the value and limitations of Cross-sectional Echocardiography (CSE) in the diagnosis of Arrhythmogenic right ventricular dysplasia (ARVD). Diagnosis was based on accepted clinical, electrocardiographic, electrophysiologic and angiographic criteria. CSE criteria for the diagnosis are segmental right ventricular wall motion abnormalities of unknown cause, usually associated with localized or diffuse dilatation of right ventricular (RV) chamber and with the presence of localized anomalies consisting of sacculation or bulging of RV wall. Comparison of CSE and RV angiographic findings was performed in 8 patients with ARVD (6 men and 2 women, aged 10 to 37 years, mean 28 years). CSE and angiography compared closely when diffuse RV enlargement and wall motion abnormalities were identified by both techniques. Bulging and sacculation of the RV wall at CSE predicted the presence of similar lesions at angiography, but agreement for specific location was poor and, in addition, CSE showed low sensitivity in their detection. The inherent different information provided by the two methods added to the subjectivity of the qualitative analysis probably accounts for the inconsistencies. Therefore in patients with diagnosed ARVD RV enlargement, otherwise unexplained, associated with wall motion abnormalities and localized anomalies at CSE strongly supports the diagnosis and avoids the need for angiography. By other hand, in patients with high clinical suspicion of ARVD a negative CSE study can not exclude the diagnosis and angiography should be indicated.