Table 4 shows a comparison of CMR characteristics of 16 SCN5A mutation-positive, 60 mutation-negative Brugada patients, and 30 healthy controls. Quantitative CMR analysis showed significantly larger RVESVi and RVEDVi, as well as a significantly reduced RVEF and RVCOi when comparing SCN5A mutation-positive with SCN5A mutation-negative patients (see Figure 1). The RVOT area was comparable between SCN5A mutation-positive and SCN5A mutation-negative patients. However, patients with BrS irrespective of the presence of a mutation revealed significantly larger RVOT areas when compared with healthy controls. In comparison with healthy controls, mutation-positive patients showed significantly lower RVEF, RVCOi, and increased RVEDVi and RVESVi. However, between mutation-negative patients and controls, there were only significant differences in the measurement for RVEF and RVESVi. With respect to LV measurements, patients positive for an SCN5A mutation showed a trend towards larger LV diameter and lower LVEF when compared with healthy controls, and a trend to a lower LVCOi [compared with SCN5A mutation-negative patients (Table 4)]. There were no significant differences in LV measurements between SCN5A-negative BrS patients and controls.
Table 4
Comparison of CMR findings of mutation-positive and -negative BrS patients and healthy controls
| SCN5A mutation-positive | SCN5A mutation-negative | Controls | P-value* | P-value** | P-value*** | |
|---|---|---|---|---|---|---|
| LVEF (%) | 57.9 ± 4.8 | 58.3 ± 5.4 | 60.2 ± 4.5 | 0.80 | 0.12 | 0.10 |
| EDMi (g/m²) | 64.1 ± 14.8 | 62.4 ± 14.3 | 61.5 ± 14.5 | 0.72 | 0.61 | 0.78 |
| LVEDVi (mL/m²) | 74.9 ± 14.2 | 74.4 ± 13.6 | 74.4 ± 12.0 | 0.91 | 0.90 | 1.00 |
| LVESVi (mL/m²) | 32.6 ± 7.8 | 31.2 ± 7.8 | 30.1 ± 5.7 | 0.58 | 0.27 | 0.52 |
| LVSVi (mL/m²) | 41.4 ± 8.1 | 43.4 ± 8.2 | 44.2 ± 8.8 | 0.45 | 0.34 | 0.66 |
| LVCOi (L/min m²) | 2.7 ± 0.6 | 3.0 ± 0.5 | 3.0 ± 0.7 | 0.05 | 0.05 | 0.80 |
| Septal wall thickness (mm) | 8.9 ± 2.5 | 10.2 ± 5.9 | 8.9 ± 2.4 | 0.43 | 0.96 | 0.26 |
| PWT (mm) | 7.7 ± 1.6 | 8.3 ± 1.9 | 7.6 ± 2.0 | 0.26 | 0.85 | 0.10 |
| LVEDD (mm) | 53.7 ± 4.3 | 51.3 ± 7.9 | 51.2 ± 4.4 | 0.25 | 0.07 | 0.96 |
| RVEF (%) | 51.7 ± 6.1 | 55.5 ± 5.4 | 59.8 ± 4.9 | 0.03 | <0.01 | <0.01 |
| RVEDVi (mL/m²) | 80.2 ± 5.5 | 73.6 ± 13.4 | 69.2 ± 12.7 | 0.01 | 0.02 | 0.16 |
| RVESVi (mL/m²) | 38.1 ± 3.7 | 32.7 ± 9.3 | 27.1 ± 6.9 | <0.01 | <0.01 | 0.01 |
| RVSVi (mL/m²) | 37.5 ± 7.5 | 40.9 ± 7.7 | 41.7 ± 7.8 | 0.17 | 0.13 | 0.69 |
| RVCOi (L/min m²) | 2.3 ± 0.5 | 2.9 ± 0.6 | 2.9 ± 0.6 | 0.01 | 0.01 | 0.81 |
| RVEDD (mm) | 42.0 ± 4.4 | 42.5 ± 7.6 | 42.0 ± 5.1 | 0.81 | 0.98 | 0.74 |
| Right atrial diameter (mm) | 45.3 ± 5.4 | 44.2 ± 7.5 | 43.6 ± 5.5 | 0.58 | 0.33 | 0.73 |
| RVOTi | 5.7 ± 1.3 | 5.3 ± 1.1 | 4.6 ± 0.8 | 0.25 | 0.01 | 0.03 |
| SCN5A mutation-positive | SCN5A mutation-negative | Controls | P-value* | P-value** | P-value*** | |
|---|---|---|---|---|---|---|
| LVEF (%) | 57.9 ± 4.8 | 58.3 ± 5.4 | 60.2 ± 4.5 | 0.80 | 0.12 | 0.10 |
| EDMi (g/m²) | 64.1 ± 14.8 | 62.4 ± 14.3 | 61.5 ± 14.5 | 0.72 | 0.61 | 0.78 |
| LVEDVi (mL/m²) | 74.9 ± 14.2 | 74.4 ± 13.6 | 74.4 ± 12.0 | 0.91 | 0.90 | 1.00 |
| LVESVi (mL/m²) | 32.6 ± 7.8 | 31.2 ± 7.8 | 30.1 ± 5.7 | 0.58 | 0.27 | 0.52 |
| LVSVi (mL/m²) | 41.4 ± 8.1 | 43.4 ± 8.2 | 44.2 ± 8.8 | 0.45 | 0.34 | 0.66 |
| LVCOi (L/min m²) | 2.7 ± 0.6 | 3.0 ± 0.5 | 3.0 ± 0.7 | 0.05 | 0.05 | 0.80 |
| Septal wall thickness (mm) | 8.9 ± 2.5 | 10.2 ± 5.9 | 8.9 ± 2.4 | 0.43 | 0.96 | 0.26 |
| PWT (mm) | 7.7 ± 1.6 | 8.3 ± 1.9 | 7.6 ± 2.0 | 0.26 | 0.85 | 0.10 |
| LVEDD (mm) | 53.7 ± 4.3 | 51.3 ± 7.9 | 51.2 ± 4.4 | 0.25 | 0.07 | 0.96 |
| RVEF (%) | 51.7 ± 6.1 | 55.5 ± 5.4 | 59.8 ± 4.9 | 0.03 | <0.01 | <0.01 |
| RVEDVi (mL/m²) | 80.2 ± 5.5 | 73.6 ± 13.4 | 69.2 ± 12.7 | 0.01 | 0.02 | 0.16 |
| RVESVi (mL/m²) | 38.1 ± 3.7 | 32.7 ± 9.3 | 27.1 ± 6.9 | <0.01 | <0.01 | 0.01 |
| RVSVi (mL/m²) | 37.5 ± 7.5 | 40.9 ± 7.7 | 41.7 ± 7.8 | 0.17 | 0.13 | 0.69 |
| RVCOi (L/min m²) | 2.3 ± 0.5 | 2.9 ± 0.6 | 2.9 ± 0.6 | 0.01 | 0.01 | 0.81 |
| RVEDD (mm) | 42.0 ± 4.4 | 42.5 ± 7.6 | 42.0 ± 5.1 | 0.81 | 0.98 | 0.74 |
| Right atrial diameter (mm) | 45.3 ± 5.4 | 44.2 ± 7.5 | 43.6 ± 5.5 | 0.58 | 0.33 | 0.73 |
| RVOTi | 5.7 ± 1.3 | 5.3 ± 1.1 | 4.6 ± 0.8 | 0.25 | 0.01 | 0.03 |
*SCN5A positive vs. negative.
**SCN5A positive vs. controls.
***SCN5A negative vs. controls.
Table 4
Comparison of CMR findings of mutation-positive and -negative BrS patients and healthy controls
| SCN5A mutation-positive | SCN5A mutation-negative | Controls | P-value* | P-value** | P-value*** | |
|---|---|---|---|---|---|---|
| LVEF (%) | 57.9 ± 4.8 | 58.3 ± 5.4 | 60.2 ± 4.5 | 0.80 | 0.12 | 0.10 |
| EDMi (g/m²) | 64.1 ± 14.8 | 62.4 ± 14.3 | 61.5 ± 14.5 | 0.72 | 0.61 | 0.78 |
| LVEDVi (mL/m²) | 74.9 ± 14.2 | 74.4 ± 13.6 | 74.4 ± 12.0 | 0.91 | 0.90 | 1.00 |
| LVESVi (mL/m²) | 32.6 ± 7.8 | 31.2 ± 7.8 | 30.1 ± 5.7 | 0.58 | 0.27 | 0.52 |
| LVSVi (mL/m²) | 41.4 ± 8.1 | 43.4 ± 8.2 | 44.2 ± 8.8 | 0.45 | 0.34 | 0.66 |
| LVCOi (L/min m²) | 2.7 ± 0.6 | 3.0 ± 0.5 | 3.0 ± 0.7 | 0.05 | 0.05 | 0.80 |
| Septal wall thickness (mm) | 8.9 ± 2.5 | 10.2 ± 5.9 | 8.9 ± 2.4 | 0.43 | 0.96 | 0.26 |
| PWT (mm) | 7.7 ± 1.6 | 8.3 ± 1.9 | 7.6 ± 2.0 | 0.26 | 0.85 | 0.10 |
| LVEDD (mm) | 53.7 ± 4.3 | 51.3 ± 7.9 | 51.2 ± 4.4 | 0.25 | 0.07 | 0.96 |
| RVEF (%) | 51.7 ± 6.1 | 55.5 ± 5.4 | 59.8 ± 4.9 | 0.03 | <0.01 | <0.01 |
| RVEDVi (mL/m²) | 80.2 ± 5.5 | 73.6 ± 13.4 | 69.2 ± 12.7 | 0.01 | 0.02 | 0.16 |
| RVESVi (mL/m²) | 38.1 ± 3.7 | 32.7 ± 9.3 | 27.1 ± 6.9 | <0.01 | <0.01 | 0.01 |
| RVSVi (mL/m²) | 37.5 ± 7.5 | 40.9 ± 7.7 | 41.7 ± 7.8 | 0.17 | 0.13 | 0.69 |
| RVCOi (L/min m²) | 2.3 ± 0.5 | 2.9 ± 0.6 | 2.9 ± 0.6 | 0.01 | 0.01 | 0.81 |
| RVEDD (mm) | 42.0 ± 4.4 | 42.5 ± 7.6 | 42.0 ± 5.1 | 0.81 | 0.98 | 0.74 |
| Right atrial diameter (mm) | 45.3 ± 5.4 | 44.2 ± 7.5 | 43.6 ± 5.5 | 0.58 | 0.33 | 0.73 |
| RVOTi | 5.7 ± 1.3 | 5.3 ± 1.1 | 4.6 ± 0.8 | 0.25 | 0.01 | 0.03 |
| SCN5A mutation-positive | SCN5A mutation-negative | Controls | P-value* | P-value** | P-value*** | |
|---|---|---|---|---|---|---|
| LVEF (%) | 57.9 ± 4.8 | 58.3 ± 5.4 | 60.2 ± 4.5 | 0.80 | 0.12 | 0.10 |
| EDMi (g/m²) | 64.1 ± 14.8 | 62.4 ± 14.3 | 61.5 ± 14.5 | 0.72 | 0.61 | 0.78 |
| LVEDVi (mL/m²) | 74.9 ± 14.2 | 74.4 ± 13.6 | 74.4 ± 12.0 | 0.91 | 0.90 | 1.00 |
| LVESVi (mL/m²) | 32.6 ± 7.8 | 31.2 ± 7.8 | 30.1 ± 5.7 | 0.58 | 0.27 | 0.52 |
| LVSVi (mL/m²) | 41.4 ± 8.1 | 43.4 ± 8.2 | 44.2 ± 8.8 | 0.45 | 0.34 | 0.66 |
| LVCOi (L/min m²) | 2.7 ± 0.6 | 3.0 ± 0.5 | 3.0 ± 0.7 | 0.05 | 0.05 | 0.80 |
| Septal wall thickness (mm) | 8.9 ± 2.5 | 10.2 ± 5.9 | 8.9 ± 2.4 | 0.43 | 0.96 | 0.26 |
| PWT (mm) | 7.7 ± 1.6 | 8.3 ± 1.9 | 7.6 ± 2.0 | 0.26 | 0.85 | 0.10 |
| LVEDD (mm) | 53.7 ± 4.3 | 51.3 ± 7.9 | 51.2 ± 4.4 | 0.25 | 0.07 | 0.96 |
| RVEF (%) | 51.7 ± 6.1 | 55.5 ± 5.4 | 59.8 ± 4.9 | 0.03 | <0.01 | <0.01 |
| RVEDVi (mL/m²) | 80.2 ± 5.5 | 73.6 ± 13.4 | 69.2 ± 12.7 | 0.01 | 0.02 | 0.16 |
| RVESVi (mL/m²) | 38.1 ± 3.7 | 32.7 ± 9.3 | 27.1 ± 6.9 | <0.01 | <0.01 | 0.01 |
| RVSVi (mL/m²) | 37.5 ± 7.5 | 40.9 ± 7.7 | 41.7 ± 7.8 | 0.17 | 0.13 | 0.69 |
| RVCOi (L/min m²) | 2.3 ± 0.5 | 2.9 ± 0.6 | 2.9 ± 0.6 | 0.01 | 0.01 | 0.81 |
| RVEDD (mm) | 42.0 ± 4.4 | 42.5 ± 7.6 | 42.0 ± 5.1 | 0.81 | 0.98 | 0.74 |
| Right atrial diameter (mm) | 45.3 ± 5.4 | 44.2 ± 7.5 | 43.6 ± 5.5 | 0.58 | 0.33 | 0.73 |
| RVOTi | 5.7 ± 1.3 | 5.3 ± 1.1 | 4.6 ± 0.8 | 0.25 | 0.01 | 0.03 |
*SCN5A positive vs. negative.
**SCN5A positive vs. controls.
***SCN5A negative vs. controls.
Figure 1
Comparison of RV CMR measurements between SCN5A mutation-positive and -negative BrS patients. RVEF, right ventricular ejection fraction; RVEDVi, indexed right ventricular end-diastolic volume; RVESVi, indexed right ventricular end-systolic volume; RVCOi, indexed right ventricular cardiac output.
