Distribution of baseline characteristics according to outcome: treated arrhythmia versus no arrhythmia
| FD-CIED | FD-control | |||||
| A. NSVT | ||||||
| No NSVT (n=49) | NSVT (n=41) | P value | No NSVT (n=221) | NSVT (n=5) | P value | |
| LVH (n, %) | 41 (83.7) | 37 (90.2) | 0.353 | 32/86 (37.2) | 4/5 (80) | 0.078 |
| LGE* (n, %) | 14/21 (66.7) | 15/19 (78.9) | 0.648 | 83/207 (40.1) | 2/3 (66.7) | 0.567 |
| LA dilatation* (n, %) | 28 (57.1) | 28 (68.3) | 0.815 | 12/57 (21.1) | 1/4 (25) | NS |
| PR interval (ms) | 186±42 | 163±36 | 0.020 | 145±28 | 157±35 | 0.455 |
| QRS duration (ms) | 138±34 | 133±30 | 0.520 | 99±20 | 118±32 | 0.257 |
| MSSI | 15.4±10.6 | 14.7±8.5 | 0.742 | 6.6±6.9 | 16.6±12.3 | 0.013 |
| B. AF | ||||||
| No AF (n=73) | AF (n=17) | P value | No AF (n=222) | AF (n=4) | P value | |
| LVH (n, %) | 61 (83.5) | 17 (100) | 0.575 | 32/87 (36.8) | 4/4 (100) | 0.022 |
| LGE* (n, %) | 19/29 (65.5) | 10/11 (90.9) | 0.033 | 84/207 (40.6) | 1/3 (33.3) | NS |
| LA dilatation* (n, %) | 43 (58.9) | 13 (76.4) | 0.562 | 11/5819 | 2/3 (66.7) | 0.112 |
| PR interval (ms) | 172±39 | 182±46 | 0.440 | 147±27 | 218 | 0.011 |
| QRS duration (ms) | 135±33 | 139±30 | 0.686 | 99±20 | 123±12 | 0.041 |
| MSSI | 15.4±10.4 | 13.7±5.9 | 0.504 | 7.1±7.7 | 12±7.2 | 0.110 |
| C. VT with haemodynamic compromise or VF needing defibrillation | ||||||
| No ICD shock (n=82) | ICD shock (n=8) | P value | ||||
| LVH (n, %) | 72 (87.8) | 6 (75) | 0.046 | |||
| LGE* (n, %) | 26/37 (70.2) | 3/3 (100) | 0.542 | |||
| LA dilatation* (n, %) | 52 (63.4) | 4 (50) | 0.256 | |||
| PR interval (ms) | 175±41 | 166±37 | 0.526 | |||
| QRS duration (ms) | 138±32 | 113±18 | 0.071 | |||
| MSSI | 15.2±9.3 | 13.1±13.9 | 0.586 | |||
The presence of specific clinical characteristics was evaluated in those with a treated arrhythmia in the FD-CIED and FD-control cohorts: NSVT (table 5a), AF (table 5b) and VT requiring defibrillation (table 5c, FD-CIED group only). Arrhythmia data were collected from CIED follow-up in the FD-CIED group and from Holter monitor testing in the FD-control group.
*Not all underwent CMR or transthoracic echocardiography imaging.
AF, atrial fibrillation; CMR, cardiovascular magnetic resonance; FD-CIED, Fabry disease with a cardiovascular implantable electronic device; ICD, implantable cardioverter defibrillator; LA, left atrium; LGE, late gadolinium enhancement; LVH, left ventricular hypertrophy; MSSI, Mainz Severity Score Index; NS, non-significant; NSVT, non-sustained ventricular tachycardia; VF, ventricular fibrillation; VT, ventricular tachycardia.