TABLE 1.
Cardiovascular manifestations
| Total (n = 122) | Michigan (n = 83) | Turkey (n = 39) | P value | |
|---|---|---|---|---|
| Age when cardiac examination, y | 46 (34–55) | 46 (35–57) | 44 (30–53) | 0.284 |
| Sex, Females | 101 (83) | 68 (82) | 33 (85) | 0.802 |
| BMI, kg/m2 | 27.2 (22.6–32.4) | 30 (24.1–34.4) | 24.6 (20.6–26.2) | <0.001 |
| LMNA variant | 60 (49) | 33 (40) | 27 (69) | 0.011* |
| Any cardiac issue | 95 (78) | 69 (83) | 26 (67) | 0.060 |
| Ischaemic heart disease | 30 (25) | 19 (23) | 11 (28) | 0.653 |
| Heart catheterization | 35 (29) | 23 (28) | 12 (31) | 0.830 |
| Balloon angioplasty | 15 (12) | 7 (8) | 8 (21) | 0.077 |
| Cardiac stent | 16 (13) | 8 (10) | 8 (21) | 0.148 |
| Myocardial infarction | 16 (13) | 13 (16) | 3 (8) | 0.383 |
| CABG | 6 (5) | 5 (6) | 1 (3) | 0.663 |
| Stroke | 11 (9) | 9 (11) | 2 (5) | 0.500 |
| PR interval, ms | 156 (142–168) | 158 (146–168) | 153 (136–180) | 0.234 |
| QRS duration, ms | 88 (82–98) | 88 (82–98) | 88 (80–102) | 0.761 |
| QTC, ms | 442 (421–462) | 445 (430–468) | 429 (400–455) | 0.001 |
| Arrhythmia | 45 (37) | 36 (43) | 9 (23) | 0.044 |
| Atrial fib/flutter | 12 (10) | 6 (7) | 6 (15) | 0.196 |
| PVC | 10 (8) | 10 (12) | 0 | 0.030 |
| PAC/SVPC | 7 (6) | 5 (6) | 2 (5) | 1.000 |
| Sinus arrhythmia | 21 (17) | 18 (22) | 3( 8) | 0.072 |
| Conduction abnormality | 20 (16) | 17 (20) | 3 (8) | 0.114 |
| Prolonged QT | 24 (20) | 19 (23) | 5 (13) | 0.229 |
| Axis deviation | 9 (7) | 6 (7) | 3 (8) | 1.000 |
| Cardiomyopathy | 13 (11) | 9 (11) | 4 (10) | 1.000 |
| Congestive heart failure | 18 (15) | 15 (18) | 3 (8) | 0.175 |
| LV hypertrophy† | 29 (32) | 21 (40) | 8 (21) | 0.068 |
| Diastolic dysfunction† | 21 (23) | 14 (27) | 7 (18) | 0.451 |
| Valvular heart disease† | 27 (30) | 13 (25) | 14 (36) | 0.354 |
| LVEF†, % | 60 (60–65) | 63 (57–65) | 60 (60–65) | 0.369 |
Note: Values are median (interquartile range) or n (%).
CABG indicates coronary artery bypass graft surgery; PVC, premature ventricular complex; PAC, premature atrial complex; SVPC, supraventricular premature complex; LVEF, left ventricular ejection fraction.
†
Echocardiogram is available in 91 patients (52 Michigan and 39 Turkey).
*
Seven patients with no complete gene sequencing for LMNA are excluded.