I.1 | II.2 | II.3 | II.6 | II.7 | III.3 | III.5 | |
Age/age at death | Sudden death aged 60 | 29 | 45 | Sudden death aged 30 | 37 | 8 | Sudden death aged 17 |
Symptoms | Exertional dyspnoea, NYHA II, dizzy spells | Dizzy spells | |||||
Signs | ESM from age 12 | ESM | ESM | ||||
ECG | QRS axis +180°, ST/T changes SLI 33 | QRS axis −60° SLI 33 | SLI 36.5 | ||||
Max LV wall thickness (mm) | Heart weight 540 g | 15.4 | 17.2 | 25.4 | 10.51-150 | ||
SAM/gradient | Incomplete SAM | Incomplete SAM |
↵1-150 >99th centile for age/body weight/body surface area.
SLI = Sokolov-Lyon Index; ESM = ejection systolic murmur; SAM = systolic anterior movement of mitral valve.