Table 1. Cardiac manifestations in affected subjects from a single extended family with LMNA R225X mutation.
| Cardiac Manifestations (age of diagnosis in years) | |||||||
|---|---|---|---|---|---|---|---|
| Affected subjects | Sex | AV block | AF | VT/SCD | Cardiomyopathy | AICD/Pacemaker | Age of death |
| I-1 | M | ? | ? | + (59) | ? | - | (59) |
| II-2 | F | CHB (43) | + (46) | + (53) | + (43) | + (43) | (53) |
| II-4 | F | 2° HB (53) | - | - | - | - | - |
| II-5 | F | CHB (51) | + (52) | - | - | + (51) | - |
| II-7 | M | CHB (49) | + (49) | + (50) | + (51) | + (52) | - |
| II-9 | F | CHB (48) | + (48) | - | - | + (48) | - |
| III-2 | F | 2° HB, (43) | - | + (44) | - | + (44) | - |
| III-3 | F | 1° HB, PR: 360ms (43) | - | - | - | - | - |
| III-4 | M | 1° HB, PR: 320ms (39) | - | + (39) | + (39) | - | - |
Abbreviations: 1° HB: first degree heart block; 2° HB: second degree heart block; AF: Atrial fibrillation; AICD: automatic implantable cardioverter defibrillator; AV block: atrio-ventricular block; CHB: complete heart block; PR: P-R interval; SCD: sudden cardiac death; VT: ventricular tachyarrhythmia.