Clinical characteristics of paediatric and adult patients with Brugada syndrome
| . | Age >16 years (N = 437) . | Age ≤16 years (N = 63) . | Total (N = 500) . | P–value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 42.9 ± 13.9 | 11.0 ± 2.1 | 38.9 ± 16.9 | <0.001 |
| Gender (male) | 210 (48.1%) | 35 (55.6%) | 245 (49.0%) | 0.28 |
| Spontaneous BrS I pattern, n (%) | 76 (17.4%) | 11 (17.5%) | 87 (17.4%) | 1.00 |
| SCD family history <35 years, n (%) | 59 (13.5%) | 16 (25.4%) | 75 (15.0%) | 0.022 |
| SND, n (%) | 20 (4.6%) | 4 (6.3%) | 24 (4.8%) | 0.53 |
| History of syncope, n (%) | 143 (32.7%) | 16 (25.4%) | 159 (31.8%) | 0.31 |
| History of aborted SCD, n (%) | 22 (5.0%) | 7 (11.1%) | 29 (5.8%) | 0.07 |
| Sieira score (points) | 1.3 ± 1.7 | 1.4 ± 2.0 | 1.3 ± 1.7 | 0.67 |
| Proband, n (%) | 252 (57.7%) | 23 (36.5%) | 275 (55%) | 0.004 |
| ICD, n (%) | 172 (39.4%) | 25 (39.7%) | 197 (39.4%) | 1.00 |
| ECG HR (b.p.m.) | 71.4 ± 13.2 | 74.6 ± 16.9 | 71.8 ± 13.7 | 0.047 |
| ECG PQ (ms) | 166.3 ± 31.4 | 155.3 ± 43.9 | 165.0 ± 33.4 | 0.015 |
| ECG QRS (ms) | 100.7 ± 19.2 | 100.4 ± 22.0 | 100.7 ± 19.5 | 0.90 |
| ECG QTc (ms) | 413.7 ± 33.8 | 430.5 ± 12.4 | 420.4 ± 157.9 | 0.02 |
| EPS HV (ms) | 44.5 ± 8.1 | 43.4 ± 10.2 | 44.4 ± 8.3 | 0.49 |
| cSNRT (ms) | 321.8 ± 124.3 | 274.2 ± 116.7 | 317.1 ± 124.1 | 0.13 |
| VA inducibility at EPS, n (%) | 36 (8.2%) | 0 (0.0%) | 36 (7.2%) | 0.015 |
| SCN5A P/LP variant | 75 (17.2%) | 29 (46.0%) | 104 (20.8%) | <0.001 |
| . | Age >16 years (N = 437) . | Age ≤16 years (N = 63) . | Total (N = 500) . | P–value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 42.9 ± 13.9 | 11.0 ± 2.1 | 38.9 ± 16.9 | <0.001 |
| Gender (male) | 210 (48.1%) | 35 (55.6%) | 245 (49.0%) | 0.28 |
| Spontaneous BrS I pattern, n (%) | 76 (17.4%) | 11 (17.5%) | 87 (17.4%) | 1.00 |
| SCD family history <35 years, n (%) | 59 (13.5%) | 16 (25.4%) | 75 (15.0%) | 0.022 |
| SND, n (%) | 20 (4.6%) | 4 (6.3%) | 24 (4.8%) | 0.53 |
| History of syncope, n (%) | 143 (32.7%) | 16 (25.4%) | 159 (31.8%) | 0.31 |
| History of aborted SCD, n (%) | 22 (5.0%) | 7 (11.1%) | 29 (5.8%) | 0.07 |
| Sieira score (points) | 1.3 ± 1.7 | 1.4 ± 2.0 | 1.3 ± 1.7 | 0.67 |
| Proband, n (%) | 252 (57.7%) | 23 (36.5%) | 275 (55%) | 0.004 |
| ICD, n (%) | 172 (39.4%) | 25 (39.7%) | 197 (39.4%) | 1.00 |
| ECG HR (b.p.m.) | 71.4 ± 13.2 | 74.6 ± 16.9 | 71.8 ± 13.7 | 0.047 |
| ECG PQ (ms) | 166.3 ± 31.4 | 155.3 ± 43.9 | 165.0 ± 33.4 | 0.015 |
| ECG QRS (ms) | 100.7 ± 19.2 | 100.4 ± 22.0 | 100.7 ± 19.5 | 0.90 |
| ECG QTc (ms) | 413.7 ± 33.8 | 430.5 ± 12.4 | 420.4 ± 157.9 | 0.02 |
| EPS HV (ms) | 44.5 ± 8.1 | 43.4 ± 10.2 | 44.4 ± 8.3 | 0.49 |
| cSNRT (ms) | 321.8 ± 124.3 | 274.2 ± 116.7 | 317.1 ± 124.1 | 0.13 |
| VA inducibility at EPS, n (%) | 36 (8.2%) | 0 (0.0%) | 36 (7.2%) | 0.015 |
| SCN5A P/LP variant | 75 (17.2%) | 29 (46.0%) | 104 (20.8%) | <0.001 |
BrS, Brugada syndrome; cSNRT, corrected sinus node recovery time; EPS, electrophysiological study; HR, heart rate; HV, His-ventricle; P/LP, pathogenic (P)/likely pathogenic (LP) variant; RBBB, right bundle branch block; SCD, sudden cardiac death; SND, sinus node dysfunction; VA, ventricular arrhythmia.
Clinical characteristics of paediatric and adult patients with Brugada syndrome
| . | Age >16 years (N = 437) . | Age ≤16 years (N = 63) . | Total (N = 500) . | P–value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 42.9 ± 13.9 | 11.0 ± 2.1 | 38.9 ± 16.9 | <0.001 |
| Gender (male) | 210 (48.1%) | 35 (55.6%) | 245 (49.0%) | 0.28 |
| Spontaneous BrS I pattern, n (%) | 76 (17.4%) | 11 (17.5%) | 87 (17.4%) | 1.00 |
| SCD family history <35 years, n (%) | 59 (13.5%) | 16 (25.4%) | 75 (15.0%) | 0.022 |
| SND, n (%) | 20 (4.6%) | 4 (6.3%) | 24 (4.8%) | 0.53 |
| History of syncope, n (%) | 143 (32.7%) | 16 (25.4%) | 159 (31.8%) | 0.31 |
| History of aborted SCD, n (%) | 22 (5.0%) | 7 (11.1%) | 29 (5.8%) | 0.07 |
| Sieira score (points) | 1.3 ± 1.7 | 1.4 ± 2.0 | 1.3 ± 1.7 | 0.67 |
| Proband, n (%) | 252 (57.7%) | 23 (36.5%) | 275 (55%) | 0.004 |
| ICD, n (%) | 172 (39.4%) | 25 (39.7%) | 197 (39.4%) | 1.00 |
| ECG HR (b.p.m.) | 71.4 ± 13.2 | 74.6 ± 16.9 | 71.8 ± 13.7 | 0.047 |
| ECG PQ (ms) | 166.3 ± 31.4 | 155.3 ± 43.9 | 165.0 ± 33.4 | 0.015 |
| ECG QRS (ms) | 100.7 ± 19.2 | 100.4 ± 22.0 | 100.7 ± 19.5 | 0.90 |
| ECG QTc (ms) | 413.7 ± 33.8 | 430.5 ± 12.4 | 420.4 ± 157.9 | 0.02 |
| EPS HV (ms) | 44.5 ± 8.1 | 43.4 ± 10.2 | 44.4 ± 8.3 | 0.49 |
| cSNRT (ms) | 321.8 ± 124.3 | 274.2 ± 116.7 | 317.1 ± 124.1 | 0.13 |
| VA inducibility at EPS, n (%) | 36 (8.2%) | 0 (0.0%) | 36 (7.2%) | 0.015 |
| SCN5A P/LP variant | 75 (17.2%) | 29 (46.0%) | 104 (20.8%) | <0.001 |
| . | Age >16 years (N = 437) . | Age ≤16 years (N = 63) . | Total (N = 500) . | P–value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 42.9 ± 13.9 | 11.0 ± 2.1 | 38.9 ± 16.9 | <0.001 |
| Gender (male) | 210 (48.1%) | 35 (55.6%) | 245 (49.0%) | 0.28 |
| Spontaneous BrS I pattern, n (%) | 76 (17.4%) | 11 (17.5%) | 87 (17.4%) | 1.00 |
| SCD family history <35 years, n (%) | 59 (13.5%) | 16 (25.4%) | 75 (15.0%) | 0.022 |
| SND, n (%) | 20 (4.6%) | 4 (6.3%) | 24 (4.8%) | 0.53 |
| History of syncope, n (%) | 143 (32.7%) | 16 (25.4%) | 159 (31.8%) | 0.31 |
| History of aborted SCD, n (%) | 22 (5.0%) | 7 (11.1%) | 29 (5.8%) | 0.07 |
| Sieira score (points) | 1.3 ± 1.7 | 1.4 ± 2.0 | 1.3 ± 1.7 | 0.67 |
| Proband, n (%) | 252 (57.7%) | 23 (36.5%) | 275 (55%) | 0.004 |
| ICD, n (%) | 172 (39.4%) | 25 (39.7%) | 197 (39.4%) | 1.00 |
| ECG HR (b.p.m.) | 71.4 ± 13.2 | 74.6 ± 16.9 | 71.8 ± 13.7 | 0.047 |
| ECG PQ (ms) | 166.3 ± 31.4 | 155.3 ± 43.9 | 165.0 ± 33.4 | 0.015 |
| ECG QRS (ms) | 100.7 ± 19.2 | 100.4 ± 22.0 | 100.7 ± 19.5 | 0.90 |
| ECG QTc (ms) | 413.7 ± 33.8 | 430.5 ± 12.4 | 420.4 ± 157.9 | 0.02 |
| EPS HV (ms) | 44.5 ± 8.1 | 43.4 ± 10.2 | 44.4 ± 8.3 | 0.49 |
| cSNRT (ms) | 321.8 ± 124.3 | 274.2 ± 116.7 | 317.1 ± 124.1 | 0.13 |
| VA inducibility at EPS, n (%) | 36 (8.2%) | 0 (0.0%) | 36 (7.2%) | 0.015 |
| SCN5A P/LP variant | 75 (17.2%) | 29 (46.0%) | 104 (20.8%) | <0.001 |
BrS, Brugada syndrome; cSNRT, corrected sinus node recovery time; EPS, electrophysiological study; HR, heart rate; HV, His-ventricle; P/LP, pathogenic (P)/likely pathogenic (LP) variant; RBBB, right bundle branch block; SCD, sudden cardiac death; SND, sinus node dysfunction; VA, ventricular arrhythmia.
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