Table 2 Clinical features of affected members with familial arrhythmogenic right ventricular cardiomyopathy.
Subject | Age at diagnosis (yrs.) | Sex | Clinical presentation | ECG and EPS abnormalities | RV abnormalities on imaging studies | Pathological findings on endomyocardial biopsy or autopsy | Major criteria | Minor criteria |
|---|---|---|---|---|---|---|---|---|
II:4 | 44 | F | Asymptomatic | T wave inversion V1-3 | None found on echo MRI not done | Not performed | 0 | 2 |
II:7 | 44 | F | Palpitations | T wave inversion V1-3 | Mild right ventricular dilation with wall motion change and aneurismal abnormality on MRI | Not performed | 0 | 3 |
III:2 | 16 | M | Palpitations | T wave inversion V1-3 Epsilon wave, V1 VT with LBBB morphology Inducible VT | Dilated RV with hypokinetic outflow tract | Not performed | 2 | 2 |
III:3 | 13 | F | Palpitations | VT with LBBB morphology | Dilated RV with hypokinesia | Not performed | 1 | 2 |
III:4 | 23 | M | Palpitations, chest pain | T-wave inversion V1-5 PVCs with LBBB pattern | Dilated RV on echo | Fibrofatty replacement of RV myocardium on endomyocardial biopsy | 1 | 4 |
III:6 | 21 | M | Asymptomatic | Dilated RV on echo; Evidence of fatty infiltration of RV wall on MRI | Not performed | 1 | 2 | |
III:7 | 19 | F | Syncope | Frequent PVCs (>1000/24hrs) | Dilated RV on echo and MRI; Fatty infiltration of RV wall on MRI | Not performed | 1 | 4 |
III:9 | 16 | F | Asymptomatic | None | Mild right ventricular dilation with wall motion change and aneurismal abnormality on MRI | Not performed | 0 | 2 |