The Role of Right Ventricular Angiography in ARVCD

The structural abnormalities of the RV myocardium are the basis for the main clinical and diagnostic features of ARVC/D. Apart from the ventricular tach-yarrhythmias that are frequently the first clinical manifestation of ARVC/D, they also result in characteristic features in the ECG and imaging. However, the clinical diagnosis of ARVC/D may be difficult because there is no easily obtained single test or finding that is definitely diagnostic. Therefore, the diagnosis of ARVC/D usually requires an integrated approach with assessment of electrical, anatomical, and functional abnormalities [11] and the various diagnostic (imaging) modalities are complementary rather than competing.

Different imaging techniques can be used to detect and characterize abnormalities of RV wall structure and motion. In addition to invasive contrast cine angiocardiography, which was the first imaging modality to be used for the diagnosis of ARVC/D, several noninvasive techniques have become avail

Fig. 16.2 • "Triangle of dysplasia"in ARVC/D. Predilection areas for structural and functional abnormalities of the right ventricle in ARVC/D. These are located in the outflow tract (RVOT),the apex,and the inferobasal (subtricuspid) area of the RV free walls (leftpanel).The RV angiogram (30° RAO view, end systole) demonstrates characteristic wall motion abnormalities in the "triangle of dysplasia"with a large subtricuspid aneurysm,an apical akinesia,and a localized dyskinesia and bulging in the outflow tract of the enlarged RV (arrows) (rightpanel). RVOT, right ventricular outflow tract; LV, left ventricle

Fig. 16.2 • "Triangle of dysplasia"in ARVC/D. Predilection areas for structural and functional abnormalities of the right ventricle in ARVC/D. These are located in the outflow tract (RVOT),the apex,and the inferobasal (subtricuspid) area of the RV free walls (leftpanel).The RV angiogram (30° RAO view, end systole) demonstrates characteristic wall motion abnormalities in the "triangle of dysplasia"with a large subtricuspid aneurysm,an apical akinesia,and a localized dyskinesia and bulging in the outflow tract of the enlarged RV (arrows) (rightpanel). RVOT, right ventricular outflow tract; LV, left ventricle able. Among these are echocardiography, radionuclide angiography, magnetic resonance imaging, and multislice computed tomography. However, none of these techniques are ideal, because they all have their individual advantages and limitations in the diagnosis and characterization of ARVC/D.

Despite its invasiveness, RV angiography still remains the reference imaging technique in the diagnosis of ARVC/D. This is mainly because selective cine angiography displays not only the entire cavity but also all the contours of the RV better than other techniques. However, due to its complex shape and geometry, the angiographic definition of the normal RV is complex since different morphological and functional findings produce a large range of normality.

Angiographic features of RV structure and function in ARVC/D include global and regional dilatation or aneurysms with abnormalities of wall motion, contrast evacuation, or trabecular size and structure. Although many of these angiographic signs and features are compatible with ARVC/D, only few are specific for this diagnosis. In addition, such features are dependent on the subjective interpretation of an experienced investigator because objective criteria for evaluation are not available. In addition, specificity of RV angiography requires further definition in relation to normal subjects and patients with other diseases affecting the RV. It is therefore important that cine an-giography of the RV is performed under optimal conditions according to a standardized protocol.

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