A precise and careful medical history is the key to achieve a correct etiologic diagnosis of syncope. Thanks to this we have already removed some possible causes of syncope (orthostatic hypotension, reflex, and neurological syncope) and epilepsy (we executed an electroencephalography which did not show any abnormality). So we focused on the most likely cardiac causes.

The patient did not show any metabolic disorder at the laboratory tests or physical signs of intoxication. He did not assume any drug as possible cause of arrhythmias.

Physical exam together with ECG and echocardiography did exclude a structural heart disease together with pulmonary embolism and acute aortic dissection.

A possible arrhythmic origin seemed to us to be the most likely cause of this syncope. During hospitalization, the patient never showed bradycardia or paroxystic atrioventricular block of different grades. At ECG, the repolarization pattern did show a typical Brugada type 1 pattern with a coved ST-segment elevation ≥2 mm and a negative T wave in V1. The febrile state during which the ECG has been recorded could have unmasked the Brugada pattern, because the previous ECGs have been described to be normal. It is well known that the typical Brugada pattern may vary according to body temperature, autonomic tone, and also drug intake. This ECG pattern is described to be associated with risks of ventricular tachyarrhythmias (torsades de pointes or ventricular fibrillation mainly), as possible cause of syncope or sudden cardiac death.

An electrophysiological study was specifically not indicated in this particular situation because the patient was “symptomatic” for unexplained syncope and there was a familiar example of sudden death at young age.

Therefore, we implanted a subcutaneous cardioverter defibrillator (S-ICD) in primary prevention. We chose a subcutaneous device instead of an intravenous one because sudden death in Brugada syndrome is due to polymorphic ventricular tachycardia (e.g., torsades de pointes) or ventricular fibrillation (VF) that did not required anti-tachycardial pacing (ATP) but only defibrillation. Moreover, our patient was young and did not require cardiac pacing. Finally, the subcutaneous device implantation is less invasive (device lead is extrathoracic), and an eventual explant (e.g., for infection) is easier compared to an intravenous device.

Quinidine has been reported as a possible pharmacological aid, but the existing data are not so numerically sufficient to be considered a lifesaving therapy.

Brugada syndrome is an autosomal dominant genetic disorder, first described in 1992, with variable expression characterized by typical patterns on the surface electrocardiogram (ECG) and an increased risk of ventricular tachyarrhythmias and sudden cardiac death [2, 3].

Brugada pattern (BrP) and Brugada syndrome (BrS) differ each other for the presence or absence of symptoms:

Initial classification considered three main ECG patterns [4], but in the last consensus, this classification was changed unifying types 2 and 3 into a unique pattern (Fig. 24.2) [5]:

Moving the right precordial chest leads (V1–V2) up to the second or third intercostal space may increase the sensitivity of detecting these abnormalities [6]. This may be crucial because those with a type 1 Brugada pattern only in high chest leads seem to have a similar rate of cardiac events during >1 year of follow-up as those with type 1 Brugada ECG in standard position [7]. With that single ECG recording, it is likely to diagnose a Brugada type 1 without referring to a drug challenge (see below) in a high percentage of patients.