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. 2019 Aug 24;105(23):1825–1831. doi: 10.1136/heartjnl-2019-315229

Table 5.

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.