Arrythmogenic cardiomyopathy: Severe right ventricular disease
The following example shows the typical findings of RV deformation imaging in severe arrhythmogenic cardiomyopathy (or ARVC)
The case features a 55-year-old male who was diagnosed with ARVC fifteen years ago. The genetic substrate was found to be a pathogenic desmosomal mutation (DSG2). His exercise tolerance is slowly decreasing over the last couple of years.
RVOT is severely dilated (RVOT PLAX = 55 mm). RV peak annular velocity = 6 cm/s indicating poor systolic function. The basal and mid region of the RV free wall are akinetic. Signs of LV involvement is also present
Deformation abnormalities are seen in all RV free wall segments. Systolic peak strain is reduced in all RV regions. The onset of shortening is prolonged in the RV basal and mid region. Large post-systolic shortening is present in the basal region.
This combination of severe deformation abnormalities in the basal region (or subtricuspid area) as shown in this example is also known as the Type-III deformation pattern.