Background
Methods
Population
Electrocardiogram
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- X-axis: the S-wave deviation only in V5 (ignoring the R-wave in V5, even if it has a greater deviation from baseline than the S-wave);
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- Y-axis: the R or S maximum deviation from lead II;
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- Z-axis: the negative one half of the deviation of the R in lead V1.
Statistics
Results
Population
ARVC Total (N = 155) | ECG-positive ARVC (N = 89) | ECG-negative ARVC (N = 66) |
p-value ECG-positive vs ECG-negative | |
---|---|---|---|---|
Age | 42.1 ± 17.3 | 42.0 ± 17.4 | 41.7 ± 17.6 | 0.861 |
Sex (% male) | 106 (68.4%) | 63 (70.8%) | 43 (65.2%) | 0.488 |
Proband (%) | 111 (71.6%) | 71 (79.8%) | 30 (45.5%) | 0.012 |
I. Imaging | 155 (100%) | 89 (100%) | 66 (100%) | 1.000 |
major, % | 111 (71.6%) | 68 (76.4%) | 43 (65.2%) | 0.082 |
minor, % | 89 (57.4%) | 60 (67.4%) | 29 (43.9%) | 0.005 |
II. Tissue characterization of the wall, biopsies performed (% of patients) | 70 (45.2%) | 39 (43.8%) | 31 (47.0%) | 0.745 |
major, % | 53 (75.7%) | 31 (79.1%) | 22 (71.0%) | 0.576 |
minor, % | 63 (90.0%) | 36 (92.3%) | 27 (87.1%) | 0.454 |
III. Repolarization abnormality | ||||
major, % | 54 (34.8%) | 54 (60.7%) | 0 (0.0%) | <0.001 |
minor, % | 20 (12.9%) | 20 (22.5%) | 0 (0.0%) | <0.001 |
IV. Depolarization abnormality | ||||
major, % | 13 (8.4%) | 13 (14.6%) | 0 (0.0%) | <0.001 |
minor, % | 17 (11.0%) | 17 (19.1%) | 0 (0.0%) | <0.001 |
V. Arrhythmia | ||||
major, % | 44 (28.4%) | 26 (29.2%) | 18 (27.3%) | 0.858 |
minor, % | 64 (41.3%) | 42 (47.2%) | 22 (33.3%) | 0.1000 |
VI. Family history | ||||
major, % | 84 (54.2%) | 48 (53.9%) | 35 (53.0%) | 1.000 |
VII. Genotype positive | 48 (31.0%) | 29 (32.6%) | 19 (28.8%) | 0.726 |
PLN (% genotype positive) | 1 (2.1%) | 0 (0.0%) | 1 (5.3%) | 0.396 |
TTN (% genotype positive) | 11 (22.9%) | 8 (27.6%) | 3 (15.8%) | 0.488 |
PKP2 (% genotype positive) | 23 (47.9%) | 17 (58.6%) | 6 (31.2%) | 0.083 |
DSC2 (% genotype positive) | 2 (4.2%) | 1 (3.5%) | 1 (5.3%) | 1.000 |
DSG2 (% genotype positive) | 12 (25.0%) | 6 (20.7%) | 6 (31.5%) | 0.501 |
DSG3 (% genotype positive) | 2 (4.2%) | 1 (3.5%) | 1 (5.30%) | 1.000 |
DSP (% genotype positive) | 8 (16.7%) | 6 (20.7%) | 2 (10.56%) | 0.4510 |
ARVD/C (n = 155) | ARVD/C ECG-positive (N = 84) | ARVD/C ECG-negative (N = 66) |
P-value ECG positive/ negative | |
---|---|---|---|---|
ECG: Depolarization | ||||
- Epsilon waves | 13 (8.4%) | 13 (15.5%) | 0 (0.0%) | <0.001 |
- upslope S-wave ≥55 ms V1,V2 or V3 | 17 (11.0%) | 17 (20.2%) | 0 (0.0%) | <0.001 |
Bundle branch blocks | 22 (14.1%) | 17 (20.2%) | 0 (0.0%) | 0.035 |
SAECG performed (% total) | 63 (40.7%) | 32 (38.1%) | 31 (41.7%) | 0.515 |
- fQRSd ≥114 ms (% of SAECG) | 33 (52.4%) | 17 (53.1%) | 16 (51.6%) | 1.000 |
- LAS40 ≥ 38 ms (% of SAECG) | 31 (49.2%) | 18 (56.3%) | 13 (41.9%) | 0.317 |
- RMS40 ≤ 20 μV (% of SAECG) | 28 (44.4%) | 13 (40.6%) | 15 (48.4%) | 0.616 |
ECG: Repolarization | ||||
- T-wave inversions V1-V3 > 14 years no RBBB | 54 (34.8%) | 54 (64.3%) | 0 (0.0%) | <0.001 |
- T-wave inversions V1-V4 with RBBB | 12 (7.7%) | 12 (14.3%) | 0 (0.0%) | <0.001 |
- T-wave inversions V1 and V2 or in V4,V5,V6 | 8 (5.2%) | 8 (9.5%) | 0 (0.0%) | 0.008 |
Imaging | ||||
Echocardiograms performed (% total) | 155 (100.0%) | 84 (100.0%) | 66 (100.0%) | 1.000 |
- Regional akinesia/dyskinesia/aneurysm (% echo) | 111 (71.6%) | 65 (77.4%) | 43 (65.2%) | 0.108 |
MRI’s performed (% total) | 124 (80.0%) | 72 (85.7%) | 52 (73.2%) | 0.070 |
- Regional akinesia/dyskinesia/aneurysm (% MRI) | 89 (71.8%) | 58 (80.6%) | 31 (59.6%) | 0.272 |
- RVEDV ≥110 ml/m2 (M), 100 ml/m2 (F) (%MRI) | 65 (52.4%) | 49 (68.1%) | 16 (30.8%) | <0.001 |
- RVEDV ≥100 ml/m2 but <110 ml/m2 (M), ≥90 ml/m2 but <100 ml/m2 (F) (%MRI) | 24 (19.4%) | 10 (11.9%) | 14 (26.9%) | 0.106 |
QTc and QRSd
Parameter | QRSd (ms),[p-value] | QTc (ms),[p-value] | SPQRS-T angle | RPD angle | RtRMS-QRS |
---|---|---|---|---|---|
Controls (N = 66) | 91.5 (85.5 to 99.0) | 405.0 (387.5 to 430.2) | 24.1 (13.5 to 42.1) | 54.4 (48.9 to 61.5) | 1.54 (1.17 to 1.90) |
ARVD/C ECG-positive (N = 89) | 104.0 (94.0 to 122.0), [<0.001]* | 425.0 (403.0 to 449.0), [0.022]* | 43.8 (23.6 to 72.9), [0.228] | 74.8 (58.4 to 94.7), [0.971] | 0.81 (0.63 to 1.13), [0.371] |
ARVD/C ECG- and no BBB (N = 66) | 98.0 (86.0 to 104.0), [0.052]s | 412.0 (399.0 to 430.0), [0.061] | 33.6 (16.7 to 54.2), [0.004] | 76.2 (62.3 to 92.9), [<0.001] | 0.81 (0.64 to 1.15), [<0.001] |
ARVD/C ECG-, no BBB, SAECG-negative (N = 20) | 93.0 (85.5 to 100.0), [0.947] | 420.5 (397.5 to 430.0), [0.057] | 40.9 (22.3 to 55.7), [0.081] | 71.2 (60.4 to 84.7) [<0.001] | 0.77 (0.67 to 1.18), [<0.001] |
Parameter | Optimum cut-off | AUC |
p-value | Sensitivity (%) | Specificity (%) | PPV (%) | NPV (%) | Odds ratio |
---|---|---|---|---|---|---|---|---|
QRSd | 99.0 ms | 0.64 | 0.026 | 48.5 | 83.3 | 74.4 | 61.8 | 4.7 (2.1 to 10.6) |
QTc | 451.0 ms | 0.56 | 0.289 | 12.1 | 100.0 | 100.0 | 53.2 | 19.3 (1.1 to 342.1) |
SPQRS-T angle | 50.8° | 0.68 | <0.001 | 30.0 | 94.0 | 53.6 | 50.9 | 1.2 (0.5 to 2.7) |
RPD angle | 70.2° | 0.86 | <0.001 | 72.7 | 94.0 | 91.7 | 80.5 | 41.3 (13.1 to 130.2) |
RtRMS-QRS | 0.81 mV | 0.85 | <0.001 | 51.5 | 92.4 | 92.3 | 77.5 | 13.0 (4.6 to 36.4) |
Both right parameters | N/A | N/A | N/A | 81.8 | 90.9 | 90.0 | 83.3 | 45.0 (15.8 to 128.2) |
Spatial peaks QRS-T angles
Right root mean square QRS voltage
Combined right-precordial directed parameters
ECG-negative proband versus ECG-negative non-proband
ECG-based 2010 taskforce criteria and their relationship to the right-precordial ECG parameters
SPQRS-T angle (degrees) | RPD angle (degrees) | RtRMS-QRS (millivolts) | |
---|---|---|---|
Epsilon-wave +, n = 13 | 54.4 ± 31.3 | 100.5 ± 42.5 | 1.0 ± 0.8 |
Epsilon-wave -, n = 142 | 54.6 ± 42.0 | 81.2 ± 31.2 | 0.9 ± 0.4 |
TAD > = 55 ms, n = 17 | 67.0 ± 41.1 | 92.9 ± 40.7 | 0.7 ± 0.3a
|
TAD < 55 ms, n = 138 | 53.1 ± 41.1 | 81.7 ± 31.4 | 0.9 ± 0.5a
|
No T-wave inversion/only in V1, n = 26 | 56.7 ± 31.0 | 83.8 ± 33.8 | 1.0 ± 0.6 |
T-wave inversion V1-V2, n = 56 | 35.0 ± 28.0a
| 87.5 ± 31.8 | 0.9 ± 0.4 |
T-wave inversion V1-V3 or beyond, n = 73 | 67.7 ± 46.8 | 78.1 ± 32.4 | 0.8 ± 0.4 |