Arrhythmogenic Right Ventricular Cardiomyopathy & Arrhythmogenic Cardiomyopathy

• 40% live threatening situation as initial manifestation

• Major echo criteria: regional RV-dyskinesia or aneurysm and/or: PLAX RVOT ≥32mm (≥19mm/m2 BSA); PSAX RVOT ≥36mm (≥21mm/m2) or FAC ≤33%

• Minor criteria: regional RV-dyskinesia or aneurysm and/or: 

• PLAX RVOT ≥29 - <32mm (≥16 - <19mm/m2); PSAX RVOT ≥32 - <36mm (≥18 - 21mm/m2) or FAC >33-<40% 

• Strain reduction in the involved areas

• FWS is more positive than -20% as a predictor for progressing structural change

• LV-GLS more positive than -12,6% as a cut-off for worse outcome. LV-GLS is significantly reduced in normal EF

• Cut-off for RV GS -18% — signs of structural tissue change (fibrosis, fatty replacement)