Table 1. Clinical and echocardiography features from population of the study.
| BAG3-/DCM-N:20 | BAG3-/DCM+N: 21 | BAG 3+/DCM+N:14 | BAG3+/DCM-N:7 | |
|---|---|---|---|---|
| AGE | 40.92 ± 15.47 | 38.86 ± 14.68 | 45.21 ± 10.00 | 26.29 ± 17.43 |
| SEX (male%) | 10 | 11 | 10 | 3 |
| WEIGHT (kg) | 71.29 ± 14.66 | 74.88 ± 19.13 | 86.29 ± 15.22 | 58.00 ± 14.97 |
| HEIGHT (cm) | 168.92 ± 10.62 | 165.96 ± 29.50 | 174.21 ± 11.97 | 145 ± 53.52 |
| DYSPHNEA (+) | 0 | 30.1 | 81.2, P<0.001§ | 0 |
| PALPITATIONS (+) | 0 | 32.6 P<0.001║ | 68.8 | 28.6, P<0.001║ |
| HR (bpm) | 71.92 ± 9.50 | 87.04 ± 9.79 | 68.23 ± 11.00 | 71.33 ± 6.68 |
| SBP (mmHg) | 127.42± 17.02 | 128.63 ± 13.12 | 133.08 ± 11.41 | 121.33 ± 12.06 |
| DBP (mmHg) | 70.29 ± 12.41 | 77.04 ± 10.92 | 83.22 ± 7.39 | 76.00 ± 13.43 |
| LVEF (%) | 66.81 ± 7.17 | 45.20 ± 12.40* | 47.21 ± 12.00† | 57.71 ± 5.64 |
| TDLVD (mm) | 48.05 ± 4.68 | 55.94 ± 8.99 | 56.98 ± 8.65 | 47.98 ± 7.86 |
| TSLVD (mm) | 32.20 ± 6.17 | 1437.65 ± 10.26 | 42.96 ± 10.18 | 32.40 ± 5.97 |
| TAPSE RV (cm) | 21.58 ± 3.12 | 21.33 ± 3.23 | 21.35 ± 4.27 | 21.74 ± 1.22 |
| LA Area (cm2) | 17.26 ± 2.31 | 18.24 ± 4.32 | 19.80 ± 3.40 | 15.12 ± 4.50 |
| RA Area (cm2) | 13.67 ± 3.04 | 14.56 ± 3.97 | 16.11 ± 3.87 | 12.12 ± 2.91 |
| LA length (mm) | 48.02 ± 4.82 | 52 ± 9.07 | 60.59 ± 6.03 | 44.24 ± 4.55‡ |
| RA length (mm) | 45.54 ± 7.08 | 45.99 ± 7.06 | 52.70 ± 5.35 | 41.67 ± 5.15 |
| E wave (m/s) | 0.79 ± 0.14 | 0.79 ± 0.18 | 0.75 ± 0.23 | 0.86 ± 0.20 |
| A wave (m/s) | 0.55 ± 0.25 | 0.66 ± 0.22 | 0.70 ± 0.25 | 0.55 ± 0.14 |
| E/A | 1.34 ± 0.39 | 1.33 ± 0.45 | 1.14 ± 0.39 | 1.67 ± 0.60 |
| A septal | ||||
| Stdi (cm/s) | 7.87 ± 1.483 | 7.82±1.43 | 7.5 ± 01.50 | 8.45 ± 1.11 |
| Etdi | 8.72 ± 2.586 | 9.18±2.78 | 8.06 ± 1.64 | 11.40 ± 3.37 |
| Atdi | 7.68 ± 2.609 | 9.49±2.11 | 10.36 ± 1.85 | 7.72 ± 1.45 |
| A lateral | ||||
| Stdi | 10.40 ± 2.55 | 10.19 ± 2.46 | 9.81 ± 2.49 | 10.94 ± 2.38 |
| Etdi | 11.92 ± 4.48 | 12.90 ± 4.59 | 11.17 ± 3.23 | 16.35 ± 5.17 |
| Atdi | 9.14 ± 3.03 | 10.24 ± 2.34 | 10.18 ± 2.32 | 10.34 ± 2.58 |
| Fibrosis (+) | 0 | 26.5¶ | 62.5 | 28.6£ |
| Knock (+) | 0 | 30.5 | 43.8 | 42.9 |
BAG3 mutation carriers were divided into phenotypically negative (phenot -) or positive (phenot+) based on the presence of dysphnea and left ventricle disfunction.
Abbreviations: HR, heart rate; SBP, systolic blood pressure; DAP, dyastolic blood pressure; LVEF, left ventricle ejection fraction; TDLVD, telediastolic left ventricle diameter; TSLVD, telesistolic left ventricle diameter; TAPSE RV, tricuspid anular plane sistolic exceursion of the right ventricle; LA Area, left atrium area; RA area, right atrium area; Fibrosis +, presence of fibrosis; Knock +, presence of knock. * P<10−4 LVEF BAG3-/DCM- vs BAG3-/DCM+. † P<4x10−3 LVEF BAG3-/DCM+ vs BAG3+/DCM+. ‡ P<6x10−3 LA LENGHT BAG3-/DCM+ vs BAG3+/DCM+. § P<0.001 DYSPHNEA BAG3-/DCM+ vs BAG3+/DCM+. ║ P<0.001 PALPITATIONS BAG3-/DCM+ vs BAG3+/DCM+ AND BAG3+/DCM+ vs BAG3+/DCM-. ¶ P<0.001 PALPITATIONS BAG3-/DCM+ vs BAG3+/DCM+. £ P<0.001 PALPITATIONS BAG3+/DCM+ vs BAG3+/DCM-.