Figure 1
Transthoracic 2D speckle tracking echocardiography showing global reduced LV strain. 2D, two-dimensional; LV, left ventricular.
Figure 2
Transthoracic 2D (A) and 3D (B) echocardiography, showing dilated RV.2D, two-dimensional; 3D, three-dimensional.
Figure 3
Transthoracic 2D echocardiography, parasternal (long and short axis) view of the RV, showing dilated RV. 2D, two-dimensional; RV, right ventricular.
Figure 4
Transthoracic 2D speckle tracking echocardiography showing severely reduced RV strain. 2D, two-dimensional; RV, right ventricular.
Table 1
Diagnostic criteria fulfilled according to the 2020 International (Padua) Criteria for ACM in the present case
| Category | Finding in the present case | Classification |
|---|---|---|
| Morpho-functional RV abnormalities | Regional RV akinesia and RV dilation on echocardiography | Major |
| Repolarisation abnormalities | T-wave inversion in precordial leads in the absence of RBBB | Major |
| Ventricular arrhythmias | Sustained VT with LBBB morphology and inferior axis | Major |
| Tissue characterisation by CMR | Extensive subepicardial LV LGE in a ring-like pattern | Major |
| LV systolic dysfunction | Mildly reduced LVEF (42%) | Minor |
Figure 5
Transthoracic 2D speckle tracking echocardiography showing normal RV strain in patient’s daughter with DSP variant. 2D, two-dimensional; DSP, desmoplakin; RV, right ventricular.
Figure 6
Transthoracic 2D speckle tracking echocardiography showing normal LV strain in patient’s daughter with DSP variant. 2D, two-dimensional; DSP, desmoplakin; LV, left ventricular.