Quantitative Approach to Fragmented QRS in Arrhythmogenic Cardiomyopathy: From Disease towards Asymptomatic Carriers of Pathogenic Variants
Abstract
:1. Introduction
2. Experimental Section
2.1. Study Population
2.2. Data Collection
2.3. Total Q-fQRS
2.4. Follow-up
2.5. Statistical Methods
3. Results
3.1. Study Population
3.2. Feasibility and Reproducibility
3.3. Relation of Q-fQRS with Disease Status
3.4. Relation of Q-fQRS with Outcome
3.5. Relation of Q-fQRS with Genotype
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Overall (n = 336) | Definite ACM (n = 221) | Carriers (n = 57) | Control (n = 58) | p Value # | |
---|---|---|---|---|---|
Demographics | |||||
Age (years.) | 39 ± 15 | 42 ± 15 | 35 ± 16 * | 27 ± 6 | <0.001 |
Sex (male) | 181 (54) | 119 (54) | 29 (51) | 33 (57) | 0.811 |
Symptoms | 182 (54) | 171 (77) | 11 (19) * | 0 (0) | <0.001 |
Cardiac syncope | 52 (16) | 52 (24) | 0 (0) | 0 (0) | <0.001 |
Genetics | |||||
Pathogenic variant | 226 (67) | 169 (77) | 57 (100) | NA | <0.001 |
PKP2 | 163 (49) | 125 (57) | 38 (67) | NA | <0.001 |
DSG2 | 9 (3) | 3 (1) | 6 (11) | NA | <0.001 |
PLN | 49 (15) | 36 (16) | 13 (23) | NA | <0.001 |
ECG | |||||
PR interval (ms) | 157 ± 27 | 161 ± 28 | 145 ± 20 | 154 ± 24 | <0.001 |
QRS duration (ms) | 96 ± 15 | 97 ± 17 | 92 ± 12 | 97 ± 10 | 0.066 |
QTc interval (ms) | 419 ± 26 | 425 ± 28 | 413 ± 24 | 405 ± 14 | <0.001 |
TWI V1–2 | 136 (41) | 127 (58) | 8 (14) * | 1 (2) | <0.001 |
TWI V1–3 | 102 (30) | 102 (46) | 0 (0) | 0 (0) | <0.001 |
TWI V4–6 | 18 (5) | 18 (8) | 0 (0) | 0 (0) | 0.007 |
TAD (ms) | 56 ± 10 | 56 ± 16 | 52 ± 14 | 46 ± 9 | <0.001 |
Arrhythmia | |||||
PVC count/24 h on Holter | 851 (113–2623) | 1076 (534–3403) | 20 (2–492) | NA | <0.001 |
NSVT | 111 (33) | 101 (46) | 10 (18) | NA | <0.001 |
SVA at baseline | 81 (24) | 81 (37) | 0 (0) | NA | <0.001 |
Imaging | |||||
RVEF (%) | 48 (45–53) | 48 (41–48) | 53 (49–59) | 53 (49–56) | <0.001 |
LVEF (%) | 59 (53–62) | 62 (52–62) | 58 (53–60) | 58 (54–63) | 0.255 |
RV Volume (mL/m2) | 100 (90–118) | 100 (90–118) | 93 (83–107) | 102 (93–120) | 0.001 |
Parameters | ACM with Prior SVA (n = 81) | ACM without Prior SVA (n = 140) | Pathogenic Variant Carriers (n = 57) | Control (n = 58) | p Value |
---|---|---|---|---|---|
Q-fQRS anterior leads (V1–4) | 19 ± 8 | 17 ± 6 | 18 ± 4 | 11 ± 2 | <0.001 |
Q-fQRS inferior leads (II, III, aVF) | 16 ± 7 | 15 ± 6 | 16 ± 5 | 10 ± 3 | <0.001 |
Q–fQRS lateral leads (I, aVL, V5–6) | 18 ± 6 | 17 ± 5 | 17 ± 3 | 12 ± 2 | <0.001 |
Q-fQRS lead aVR | 4 ± 2 | 4 ± 2 | 4 ± 1 | 3 ± 1 | <0.001 |
Total Q-fQRS | 57 ± 20 | 53 ± 16 | 55 ± 10 | 35 ± 5 | <0.001 |
Univariable | Multivariable | ||||
---|---|---|---|---|---|
Hazard Ratio (95% CI) | p Value | Hazard Ratio (95% CI) | p Value | ||
Age | 0.98 (0.96–1.00) | 0.071 | Age | 0.96 (0.93–0.99) | 0.008 |
Sex | 2.19 (1.08–4.44) | 0.030 | Sex | 2.01 (0.92–4.41) | 0.080 |
Symptoms | 3.05 (1.17–7.93) | 0.022 | Symptoms | 4.91 (1.43–16.84) | 0.011 |
PR interval | 0.99 (0.98–1.01) | 0.206 | |||
QRS duration | 0.99 (0.97–1.02) | 0.529 | |||
QTc interval | 1.00 (0.99–1.01) | 0.856 | |||
RVEF | 0.96 (0.92–0.99) | 0.013 | RVEF | 0.97 (0.93–1.00) | 0.064 |
LVEF | 0.99 (0.94–1.03) | 0.536 | |||
Total Q-fQRS | 0.99 (0.96–1.02) | 0.335 |
Univariable | Multivariable | ||||
---|---|---|---|---|---|
Hazard Ratio (95% CI) | p Value | Hazard Ratio (95% CI) | p Value | ||
Age | 0.99 (0.96–1.02) | 0.591 | Age | 0.96 (0.92–0.99) | 0.044 |
Sex | 2.32 (1.01–5.30) | 0.046 | Sex | 3.71 (1.25–11.03) | 0.018 |
Symptoms | 3.03 (1.03–8.93) | 0.044 | Symptoms | 4.93 (1.05–23.05) | 0.042 |
PR interval | 0.99 (0.98–1.01) | 0.476 | |||
QRS duration | 0.98 (0.95–1.01) | 0.312 | |||
QTc interval | 1.00 (0.99–1.01) | 0.897 | |||
RVEF | 0.93 (0.89–0.97) | 0.002 | RVEF | 0.93 (0.88–0.98) | 0.010 |
LVEF | 0.98 (0.93–1.03) | 0.372 | |||
Total Q-fQRS | 0.99 (0.96–1.03) | 0.693 |
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Roudijk, R.W.; Bosman, L.P.; van der Heijden, J.F.; de Bakker, J.M.T.; Hauer, R.N.W.; van Tintelen, J.P.; Asselbergs, F.W.; te Riele, A.S.J.M.; Loh, P. Quantitative Approach to Fragmented QRS in Arrhythmogenic Cardiomyopathy: From Disease towards Asymptomatic Carriers of Pathogenic Variants. J. Clin. Med. 2020, 9, 545. https://doi.org/10.3390/jcm9020545
Roudijk RW, Bosman LP, van der Heijden JF, de Bakker JMT, Hauer RNW, van Tintelen JP, Asselbergs FW, te Riele ASJM, Loh P. Quantitative Approach to Fragmented QRS in Arrhythmogenic Cardiomyopathy: From Disease towards Asymptomatic Carriers of Pathogenic Variants. Journal of Clinical Medicine. 2020; 9(2):545. https://doi.org/10.3390/jcm9020545
Chicago/Turabian StyleRoudijk, Rob W., Laurens P. Bosman, Jeroen F. van der Heijden, Jacques M. T. de Bakker, Richard N. W. Hauer, J. Peter van Tintelen, Folkert W. Asselbergs, Anneline S. J. M. te Riele, and Peter Loh. 2020. "Quantitative Approach to Fragmented QRS in Arrhythmogenic Cardiomyopathy: From Disease towards Asymptomatic Carriers of Pathogenic Variants" Journal of Clinical Medicine 9, no. 2: 545. https://doi.org/10.3390/jcm9020545