Age (M/F) | Genotype | QRS (ms) | BBB/eps | Repolarization abnormalities | SAECG | Ventricular arrhythmias | LVEDD & EF | CMR | Dilated RV | Fulfills 2010 criteria | Clinically examined | Other | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Family 1 | |||||||||||||
I.1 | M | (A/G) | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | - | No | Deceased |
I.2 | 86F | (A/G) | 142 | LBBB | No | n.a. | No | n.a. | n.a. | n.a. | No | No | Deceased Heart failure AFa CADb |
II.1 | 45 M | A/G | 102 | No | No | n.a. | No | n.a. | n.a. | n.a. | No | Yes | |
II.2 | 50 M | A/G | 108 | No | No | n.a. | No | 58 mm50% | n.a. | No | No | Yes | |
II.3 | 52 M | G/G | 102 | pRBBB | TI V1-V4 | n.a. | Monomorphic VT of RVOT origin, VES 2800 | 61 mm 53% | RVEDV 322 ml RVEF 39% | Yes | Yes 2 major 1 minor | Yes | Right ventricular scar in EPSc ICDd |
II.4 | 53 M | G/G | 92 | No | TI V1-V3 | LP 3/3 | VES 1700 | 53 mm51% | RVEDVI 104 ml/m2 RVEF 37% | Yes | Yes 2 major 2 minor | Yes | Micro-aneurysms |
II.5 | 54F | G/G | 86 | No | TI V1-V2 | n.a. | Monomorphic VT of RVOT origin, VF | 45 mm 52% | n.a. | Yes | Yes 1 major 2 minor | Yes | Myocardial infarction RV scar in EPS ICD |
II.6 | 55F | A/G | 88 | No | No | n.a. | No | 48 mm 53% | n.a. | No | No | Yes | |
II.7 | 56F | A/A | 82 | No | No | n.a. | No | 48 mm 70% | n.a. | No | No | Yes | |
III.1 | 20 M | A/G | 94 | No | No | n.a. | No | 43 mm 73% | n.a. | No | No | Yes | |
III.2 | 22 M | A/G | 88 | No | No | n.a. | No | 47 mm 66% | RVEDVI 76 ml/m2 RVEF n.a. | No | No | Yes | Collapse |
III.3 | 23F | A/G | 86 | No | No | n.a. | No | 46 mm 69% | RVEDVI 82 ml/m2 RVEF n.a. | No | No | Yes | |
Family 2 | |||||||||||||
I.1 | 82 M | (G/a) | 115 | No | No | n.a. | VF | 57 mm 40% | n.a. | No | No | No | Deceased Stroke Myocardial infarction |
I.2 | 76F | (G/a) | 102 | pRBBB | No | n.a. | No | n.a. | n.a. | n.a. | No | No | Deceased CAD COPDe |
II.1 | 56 M | G/G | 108 | Epsilon wave | TI II, III, aVF | LP 3/3 | Monomorphic VT | 54 mm 52% | RVEDVI 151 ml/m2 RVEF 37% | Yes | Yes 3 major 1 minor | Yes | |
II.2 | 56F | A/G | 102 | No | No | n.a. | No | 43 mm 78% | RVEDVI 69 ml/m2 RVEF 67% | No | No | Yes | |
II.3 | 57 M | ? | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | n.a. | - | No | Deceased Pulm. Ca |
II.4 | 58F | A/G | 104 | No | No | n.a. | No | 48 mm 80% | n.a. | No | No | Yes | |
II.5 | 59 M | G/G | 108 | No | No | LP 3/3 | VES 1000, VF | 53 mm 56% | RVEDVI 135 ml/m2 RVEF 37% | Yes | Yes 2 major 2 minor | Yes | CAD Micro-aneurysms RVOTf -aneurysm |
II.6 | 61 M | A/G | 122 | No | No | n.a. | No | 54 mm 62% | RVEDVI 97 ml/m2 RVEF 64% | No | No | Yes |