Table I.
Patient | Age (years) | Gender | Dynamic electrocardiogram/Holter | NYHA | Cardiac symptoms at onset (age, years) | Left ventricular end-diastolic diameter (mm) | Left ventricular ejection fraction (%) | LMNA p.E82K mutation |
---|---|---|---|---|---|---|---|---|
II-1 | 69 | Female | N | N | N | Negative | ||
II-2 | 50a | Female | N | 40 | N | N | – | |
II-3 | 48a | Female | N | 36 | N | N | – | |
II-4 | 45a | Male | N | 35 | N | N | – | |
II-5 | 57a | Male | Pre-pacing, abnormal Q wave in leads V1-V4, left ventricular high voltage, slow JR with sino-capture, CRBBB, ST-T change, paired PVC, sinus arrest, paroxysmal SR; post-pacing, Frequent PVC, ST-T change, AAI | III–IV | 54 | 69.6 | 45 | – |
II-6 | 54 | Female | Pre-pacing, frequent PVC (bigeminal rhythm), degree I AVB, slow JR, accelerated atrioventricular rhythm, ST-T Change, poor R-wave progression in leads V1-V4, post-pacing, paroxysmal AF, occasional PVC, AAI | II–III | 44 | 56.8 | 35 | Heterozygous |
II-7 | 52 | Female | Pre-pacing, slow AF, degree III AVB, JR, ST-T change in some leads, Poor R-wave progression in leads V1-V4, post-pacing, AF, AAI | II–III | 47 | 50.2 | 62.3 | Heterozygous |
III-3 | 41 | Male | N | I | 42.5 | 62 | Negative | |
III-7 | 35 | Male | N | II | 49.6 | 55 | Heterozygous | |
III-10 | 38 | Male | N | I | 44.3 | 72 | Negative | |
III-11 | 34 | Male | Degree I AVB | II | 51.5 | 56 | Heterozygous | |
III-13 | 30 | Male | SR, ST-T change | II | 51.5 | 56 | Heterozygous | |
IV-6 | 8 | Female | N | 30.9 | 65 | Negative | ||
IV-7 | 4 | Male | Sinus arrhythmia | 28.6 | 73 | Heterozygous |
Patients succumbed to mortality due to heart failure at ages presented (DNA samples were not obtained). AAI; paroxysmal ventricular tachycardia; AF, atrial fibrillation; AVB, atrioventricular block; CRBBB, complete right bundle branch block; JR, junctional rhythm; pacing rhythm; N, normal; NYHA, New York Heart Association; PVC, premature ventricular contraction; SR, sinus rhythm. ST-T, ST segment and T wave.