Left Ventricular Abnormal Substrate in Brugada Syndrome.
JACC Clin Electrophysiol
; 9(10): 2041-2051, 2023 10.
Article
in En
| MEDLINE
| ID: mdl-37480873
ABSTRACT
BACKGROUND:
Slow-conductive structural abnormalities located in the epicardium of the right ventricle (RV) underlie Brugada syndrome (BrS). The extent of such substrate in the left ventricle (LV) has not been investigated.OBJECTIVES:
This study sought to characterize the extent of epicardial substrate abnormalities in BrS.METHODS:
We evaluated 22 consecutive patients (mean age 46 ± 11 years, 21 male) referred for recurrent ventricular arrhythmias (mean 10 ± 13 episodes) in the setting of BrS. The patients underwent clinical investigations and wide genetic screening to identify SCN5A mutations and common risk variants. High-density biventricular epicardial mapping was performed to detect prolonged (>70 ms) fragmented electrograms, indicating abnormal substrate area.RESULTS:
All patients presented with abnormal substrate in the epicardial anterior RV (27 ± 11 cm2). Abnormal substrate was also identified on the LV epicardium in 10 patients (45%), 9 at baseline and 1 after ajmaline infusion, covering 15 ± 11 cm2. Of these, 4 had severe LV fascicular blocks. Patients with LV substrate had a longer history of arrhythmia (11.4 ± 6.7 years vs 4.3 ± 4.3 years; P = 0.003), longer PR (217 ± 24 ms vs 171 ± 14 ms; P < 0.001) and HV (60 ± 12 ms vs 46 ± 5 ms; P = 0.005) intervals, and abnormal substrate also extending into the inferior RV (100% vs 33%; P = 0.001). SCN5A mutation was present in 70% of patients with LV substrate (vs 25%; P = 0.035). SCN5A BrS patients with recurrent ventricular arrhythmias present a higher polygenic risk score compared with a nonselected BrS population (median of differences -0.86; 95% CI -1.48 to -0.27; P = 0.02).CONCLUSIONS:
A subset of patients with BrS present an abnormal substrate extending onto the LV epicardium and inferior RV that is associated with SCN5A mutations and multigenic variants.Key words
Full text:
1
Database:
MEDLINE
Main subject:
Brugada Syndrome
/
Heart Ventricles
Type of study:
Prognostic_studies
Limits:
Adult
/
Humans
/
Male
/
Middle aged
Language:
En
Year:
2023
Type:
Article