Fig. 2

a Echocardiography of index patient shows dilatation of the right ventricle. RVD1 (basal right ventricle): 42.5 mm (Mean value (95 % CI) 33 (31–35 mm)). RVD2 (mid right ventricle) 32.7 mm (Mean value (95 % CI) 28 (23–33 mm)). RVD3 (base to apex length) 58.6 mm (Mean value (95 % CI) 71 (67–75 mm)). Mean values (95 % CI) according to Rudski et al. [9]. b Cardiac MRI of the index patient reveals moderate enlargement of the right ventricle with an enddiastolic volume of 186 ml (standard value female 140 ± 37 ml) and (c) endsystolic volume of 135 ml (standard value female 52 ± 22 ml) resulting in a highly reduced ejection fraction of 27 % (standard value female 64 ± 7 %) Furthermore, the apical posterior wall of the right ventricle showed akinesia and (d) late gadolinium enhancement identified apical fibrosis (yellow arrows) in the 4-chamber view. Standard values according to Hudsmith et al. [10]