Clinical details on selected members of the pedigree; individuals can be identified from fig 1
| Individual | Age (years) | MYO6 | Sensorineural hearing loss | ECG | ECHO | |
|---|---|---|---|---|---|---|
| QTc (ms) | Other ECG abnormalities | |||||
| *II-3 was considered a double phenocopy with presbycusis and cardiac abnormalities consistent with valvar and ischaemic disease. | ||||||
| Prolonged QTc durations are in bold type. | ||||||
| AI, aortic valve incompetence; ECHO, echocardiographic findings; LAH, left atrial hypertrophy; LBBB, left bundle branch block; LVH, left ventricular hypertrophy; MR, mitral valve regurgitation; RAD, right axis deviation; SAM, systolic anterior motion of the mitral valve; 1° HB, first degree atrioventricular block. | ||||||
| II-1 | 75 | Mutant | Yes | 581 | q wave in lead AVL; LVH; LAH; LBBB | Apical LVH, SAM, hyperdynamic non-dilated |
| II-2 | 89 | Mutant | Yes | 529 | Borderline 1° HB | Normal for age |
| II-3* | 74 | wild-type | No | 572 | q in lead AVL; LVH; LAH; wide QRS complex | Mild AI, mod MR, inferior hypokinesia |
| III-1 | 39 | Mutant | Yes | 468 | q in leads III, AVF | Apical LVH |
| III-2 | 43 | Mutant | Yes | 505 | q in leads II, III, AVR; post hemi block; biphasic t waves | Apical LVH, trivial SAM |
| III-3 | 36 | Mutant | Yes | 584 | q in lead AVL; LAD; LAH; biphasic t waves | Septal LVH |
| III-4 | 50 | Mutant | Yes | 435 | Normal | Normal |
| III-5 | 47 | Mutant | Yes | 459 | Borderline 1° HB; LAH | Normal |
| III-6 | 50 | Mutant | Yes | 510 | None | Not available |
| IV-1 | 12 | Mutant | No | 509 | Borderline QT elevation | Normal |
| IV-3 | 10 | Mutant | Yes | 447 | LAH; RAD possibly normal for age | Normal |
| IV-6 | 5 | Mutant | Yes | 424 | None | Normal |
| IV-10 | 28 | Mutant | No | 425 | None | Normal |