Brugada syndrome

By Islam khedr

Background
Brugada syndrome is a disorder characterized by sudden death associated with one of several ECG patterns characterized by incomplete right bundle branch block and ST elevations in the anterior precordial leads.

Background
Three types of ST-segment elevation in Brugada syndrome, as shown in the precordial leads on ECG in the same patient at different times Type 1 ECG pattern with pronounced elevation of the J point (arrow), a coved-type ST segment, and an inverted T wave in V1 and V2. Type 2 pattern with a saddleback ST-segment elevated by >1 mm Type 3 pattern in which the ST segment is elevated <1 mm. Type I is diagnostic when combined with the right clinical picture. Types II and III raise suspicion for Brugada but they are only diagnositic if they can be converted to Type I during challenge with a sodium channel blocker. These patterns are dynamic and inducible.

Background
Subtle structural abnormalities in the right ventricular outflow tract have been reported. Patients with Brugada syndrome are prone to develop ventricular tachyarrhythmias that may lead to syncope, cardiac arrest, or sudden cardiac death. Brugada syndrome is genetically determined and has an autosomal dominant pattern of transmission in about 50% of familial cases (SCN5A gene) . About 5% of survivors of cardiac arrest have no clinically identified cardiac abnormality, and about half of these cases are thought to be due to Brugada syndrome.

Diagnostic criteria of The brugada syndrome

Pathophysiology
Brugada syndrome is an example of a channelopathy Mutations in the SCN5A gene, which encodes the cardiac voltage-gated sodium channel reduce the sodium current (INa) available during the phases 0 (upstroke) and 1 (early repolarization) of the cardiac action potential affect the right ventricular endocardium differently from the epicardium It is thought that loss of the action potential dome in the right ventricular epicardium, but not in the endocardium, results in the persistent ST segment elevation. Heterogeneous loss of the action potential dome in the right ventricular epicardium leads to propagation of the dome from sites where it is maintained to sites at which it is lost (phase 2 reentry), resulting in ventricular arrhythmias. Among several agents that can reproduce this electrocardiographic phenomenon are sodium channel blockers. They can expose latent electrocardiographic forms of the syndrome and have been proposed as a provocative test.

Pathophysiology

Clinical picture
The typical patient with Brugada syndrome is young, male, and otherwise healthy, with normal general medical and cardiovascular physical examinations. Syncope and cardiac arrest are the most common clinical manifestations leading to the diagnosis of Brugada syndrome. some patients remain asymptomatic, and the diagnosis of Brugada syndrome is suggested by a routine ECG showing ST-segment elevation in leads V1 through V3. A family history of sudden cardiac death is common, though not universal, as the syndrome can occur sporadically. In about 20% of patients, atrial fibrillation is an associated arrhythmia.

Workup
serum potassium and calcium levels creatine kinase-MB (CK-MB) and troponin Patients with high likelihood of the disease may be genetically tested for a mutation in SCN5A Echocardiography and/or MRI ECG and exercise stress testing EPS to determine the inducibility of arrhythmias in an effort to risk-stratify patients with Brugada syndrome (debated). Challenge with sodium channel blockers.

Treatment
At present, implantation of an automatic implantable cardiac defibrillator (ICD) is the only treatment proven effective in treating ventricular tachycardia and fibrillation and preventing sudden death in patients with Brugada syndrome. No pharmacologic therapy has been proven to reduce the occurrence of ventricular arrhythmias or sudden death.

Indications for ICD implantation in patients with Brugada syndrome

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