Clinical characteristics of paediatric patients with Brugada syndrome with and without pathogenic (p)/likely pathogenic (LP) variant
| . | P− (n = 34) . | P+ (n = 29) . | Total (n = 63) . | P-value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 12.1 ± 1.8 | 9.7 ± 2.6 | 11.0 ± 2.1 | 0.07 |
| Gender (male) | 22 (64.7%) | 13 (44.8%) | 35 (55.6%) | 0.13 |
| Spontaneous BrS I pattern, n (%) | 3 (8.8%) | 8 (27.6%) | 11 (17.5%) | 0.09 |
| SCD family history <35 years, n (%) | 5 (14.7%) | 11 (37.9%) | 16 (25.4%) | 0.05 |
| SND, n (%) | 3 (8.8%) | 1 (3.4%) | 4 (6.3%) | 0.62 |
| History of syncope, n (%) | 10 (29.4%) | 6 (20.7%) | 16 (25.4%) | 0.56 |
| History of aborted SCD, n (%) | 2 (5.9%) | 5 (17.2%) | 7 (11.1%) | 0.23 |
| Sieira score (points) | 1.2 ± 1.7 | 1.7 ± 2.4 | 1.4 ± 2.0 | 0.36 |
| Proband, n (%) | 13 (38.2%) | 10 (34.5%) | 23 (36.5%) | 0.79 |
| ICD, n (%) | 14 (41.2%) | 11 (37.9%) | 25 (39.7%) | 1.00 |
| ECG HR (b.p.m.) | 71.9 ± 13.8 | 77.7 ± 19.6 | 74.6 ± 16.9 | 0.18 |
| ECG PQ (ms) | 145.3 ± 43.5 | 166.2 ± 42.3 | 155.3 ± 43.9 | 0.06 |
| ECG QRS (ms) | 95.2 ± 18.4 | 106.2 ± 24.5 | 100.4 ± 22.0 | 0.05 |
| ECG QTc (ms) | 440.5 ± 15.9 | 420.4 ± 19.9 | 430.5 ± 12.4 | 0.43 |
| . | P− (n = 34) . | P+ (n = 29) . | Total (n = 63) . | P-value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 12.1 ± 1.8 | 9.7 ± 2.6 | 11.0 ± 2.1 | 0.07 |
| Gender (male) | 22 (64.7%) | 13 (44.8%) | 35 (55.6%) | 0.13 |
| Spontaneous BrS I pattern, n (%) | 3 (8.8%) | 8 (27.6%) | 11 (17.5%) | 0.09 |
| SCD family history <35 years, n (%) | 5 (14.7%) | 11 (37.9%) | 16 (25.4%) | 0.05 |
| SND, n (%) | 3 (8.8%) | 1 (3.4%) | 4 (6.3%) | 0.62 |
| History of syncope, n (%) | 10 (29.4%) | 6 (20.7%) | 16 (25.4%) | 0.56 |
| History of aborted SCD, n (%) | 2 (5.9%) | 5 (17.2%) | 7 (11.1%) | 0.23 |
| Sieira score (points) | 1.2 ± 1.7 | 1.7 ± 2.4 | 1.4 ± 2.0 | 0.36 |
| Proband, n (%) | 13 (38.2%) | 10 (34.5%) | 23 (36.5%) | 0.79 |
| ICD, n (%) | 14 (41.2%) | 11 (37.9%) | 25 (39.7%) | 1.00 |
| ECG HR (b.p.m.) | 71.9 ± 13.8 | 77.7 ± 19.6 | 74.6 ± 16.9 | 0.18 |
| ECG PQ (ms) | 145.3 ± 43.5 | 166.2 ± 42.3 | 155.3 ± 43.9 | 0.06 |
| ECG QRS (ms) | 95.2 ± 18.4 | 106.2 ± 24.5 | 100.4 ± 22.0 | 0.05 |
| ECG QTc (ms) | 440.5 ± 15.9 | 420.4 ± 19.9 | 430.5 ± 12.4 | 0.43 |
BrS, Brugada syndrome; HR, heart rate; HV, His-ventricle; P−, patient without any pathogenic (P)/likely pathogenic (LP) variant; P+, patient with any 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 patients with Brugada syndrome with and without pathogenic (p)/likely pathogenic (LP) variant
| . | P− (n = 34) . | P+ (n = 29) . | Total (n = 63) . | P-value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 12.1 ± 1.8 | 9.7 ± 2.6 | 11.0 ± 2.1 | 0.07 |
| Gender (male) | 22 (64.7%) | 13 (44.8%) | 35 (55.6%) | 0.13 |
| Spontaneous BrS I pattern, n (%) | 3 (8.8%) | 8 (27.6%) | 11 (17.5%) | 0.09 |
| SCD family history <35 years, n (%) | 5 (14.7%) | 11 (37.9%) | 16 (25.4%) | 0.05 |
| SND, n (%) | 3 (8.8%) | 1 (3.4%) | 4 (6.3%) | 0.62 |
| History of syncope, n (%) | 10 (29.4%) | 6 (20.7%) | 16 (25.4%) | 0.56 |
| History of aborted SCD, n (%) | 2 (5.9%) | 5 (17.2%) | 7 (11.1%) | 0.23 |
| Sieira score (points) | 1.2 ± 1.7 | 1.7 ± 2.4 | 1.4 ± 2.0 | 0.36 |
| Proband, n (%) | 13 (38.2%) | 10 (34.5%) | 23 (36.5%) | 0.79 |
| ICD, n (%) | 14 (41.2%) | 11 (37.9%) | 25 (39.7%) | 1.00 |
| ECG HR (b.p.m.) | 71.9 ± 13.8 | 77.7 ± 19.6 | 74.6 ± 16.9 | 0.18 |
| ECG PQ (ms) | 145.3 ± 43.5 | 166.2 ± 42.3 | 155.3 ± 43.9 | 0.06 |
| ECG QRS (ms) | 95.2 ± 18.4 | 106.2 ± 24.5 | 100.4 ± 22.0 | 0.05 |
| ECG QTc (ms) | 440.5 ± 15.9 | 420.4 ± 19.9 | 430.5 ± 12.4 | 0.43 |
| . | P− (n = 34) . | P+ (n = 29) . | Total (n = 63) . | P-value . |
|---|---|---|---|---|
| Age at diagnosis (years) | 12.1 ± 1.8 | 9.7 ± 2.6 | 11.0 ± 2.1 | 0.07 |
| Gender (male) | 22 (64.7%) | 13 (44.8%) | 35 (55.6%) | 0.13 |
| Spontaneous BrS I pattern, n (%) | 3 (8.8%) | 8 (27.6%) | 11 (17.5%) | 0.09 |
| SCD family history <35 years, n (%) | 5 (14.7%) | 11 (37.9%) | 16 (25.4%) | 0.05 |
| SND, n (%) | 3 (8.8%) | 1 (3.4%) | 4 (6.3%) | 0.62 |
| History of syncope, n (%) | 10 (29.4%) | 6 (20.7%) | 16 (25.4%) | 0.56 |
| History of aborted SCD, n (%) | 2 (5.9%) | 5 (17.2%) | 7 (11.1%) | 0.23 |
| Sieira score (points) | 1.2 ± 1.7 | 1.7 ± 2.4 | 1.4 ± 2.0 | 0.36 |
| Proband, n (%) | 13 (38.2%) | 10 (34.5%) | 23 (36.5%) | 0.79 |
| ICD, n (%) | 14 (41.2%) | 11 (37.9%) | 25 (39.7%) | 1.00 |
| ECG HR (b.p.m.) | 71.9 ± 13.8 | 77.7 ± 19.6 | 74.6 ± 16.9 | 0.18 |
| ECG PQ (ms) | 145.3 ± 43.5 | 166.2 ± 42.3 | 155.3 ± 43.9 | 0.06 |
| ECG QRS (ms) | 95.2 ± 18.4 | 106.2 ± 24.5 | 100.4 ± 22.0 | 0.05 |
| ECG QTc (ms) | 440.5 ± 15.9 | 420.4 ± 19.9 | 430.5 ± 12.4 | 0.43 |
BrS, Brugada syndrome; HR, heart rate; HV, His-ventricle; P−, patient without any pathogenic (P)/likely pathogenic (LP) variant; P+, patient with any 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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