Case report: Lamin A/C gene mutation in patient with drug-induced type 1 Brugada syndrome at high arrhythmic risk

Abstract

We report the case of drug-induced type 1 Brugada syndrome at high arrhythmic risk associated with Lamin A/C gene mutation.

Syncope without prodromes during fever in type 1‐induced BrS patient: Looks can be deceiving

“…Moreover, the PVS showed a high negative predictive value among patients with type 1‐induced Brugada pattern, but a lower positive predictive value, regardless of the history of syncope 4 . According to our in‐hospital protocol, we use the 3D endocardial mapping of the right ventricle to evaluate the electroanatomic substrate of patients with type 1‐induced BrS pattern at baseline and during the PVS‐induced arrhythmias 5 …”

“…4 According to our in-hospital protocol, we use the 3D endocardial mapping of the right ventricle to evaluate the electroanatomic substrate of patients with type 1-induced BrS pattern at baseline and during the PVS-induced arrhythmias. 5 The use of an implantable loop recorder (ILR) should be considered in patients with Brugada pattern and unexplained syncope 6 ; and in BrS patients with low/intermediate risk, it can be helpful in guiding the management and ascertaining the cause of unexplained syncope. 7 Implantable cardioverter-defibrillator (ICD) implantation should be limited for patients with type 1-induced Brugada pattern with documented arrhythmic syncope 6 since the risk of lead infection and inappropriate ICD therapies is not negligible.…”

“… 4 According to our in‐hospital protocol, we use the 3D endocardial mapping of the right ventricle to evaluate the electroanatomic substrate of patients with type 1‐induced BrS pattern at baseline and during the PVS‐induced arrhythmias. 5 …”

The Prognostic Role of Programmed Ventricular Stimulation in the Risk Stratification of Sudden Cardiac Death

“…Moreover, despite a low PPV (8.9% in asymptomatic; 9.6% in symptomatic), PVS demonstrated a high NPV (95% in asymptomatic; 100% in symptomatic) for SCD risk stratification in high-risk patients with drug-induced type 1 ECG [ 61 ]. Therefore, based on current evidence, performing PVS for SCD risk stratification of BrS patients with drug-induced type 1 ECG remains controversial [ 62 ] and should be guided by non-invasive risk factors [ 63 , 64 ] such as unexplained syncope, genetic testing and family history of sudden cardiac death. Table 5 (Ref.…”