Autosomal dominant transmission. Prevalence 1/2,000 to 5,000 in Europa. Cause of syncope and sudden death at rest by ventricular fibrillation in young adults. Strong male predominance (10:1) after puberty; it seems that estrogen could have an antiarrhythmic role in women. This syndrome is common among young men in Japan and Southeast Asia. It may present as atrial fibrillation in children.
It is a channelopathy of of the Na (types 1, 2, 5 and 7), K (type 6, 9) or Ca (types 3 and 4) cardiac epicardial channels, especially at the level of the ejection pathway of the right ventricle.
From a genetic point of view, a distinction is made between:
- type 1 [MIM 601 144] (20 -30 %): mutation of the SCN5A gene (3p21); it can also produce the LQT3 type long QT syndrome
- type 2 [MIM 611 777]: mutation of the GPD1L gene (3p22)
- type 3 [MIM 611 875]: mutation of the CACNA1C gene (12p13) and type 4 [MIM 611 876]: mutation of the CACNB2 gene (10p12). They may also present as a short QT syndrome
- type 5 [MIM 612 838]: mutation of the SCN1B gene (19q13)
- type 6 [MIM 613 119]: mutation of the KCNE3 gene (11q13)
- type 7 [MIM 613 120]: mutation of the SCNB3 gene (11q24)
- type 8 [MIM 613 123]: mutation of the HCN4 gene (15q24)
- type 9 [MIM 616 399]: mutation of the KCND3 gene (1p13)
Three types of ECG tracings (non-ischemic modifications in V1 - V3) are described: the 3 types present a wide QRS similar to a right bundle branch block followed with:
Type 1 (kown as typical): a dome-shaped elevation ('coved') of the segment ST >= 2 mm (especially in V1) the ascending part of which is short: it is followed by a symmetrical reverse T-wave in V1 and V2
Type 2 (known as atypical ): "saddle" shaped ST-segment elevation of point J >= 2 mm and elevation of the ST > 1 mm, followed by a positive or biphasic T wave
Type 3 (known as atypical): looks like type 2, but is more flattened with an elevation of J point <= 1 mm
Some cardiologists believe that Brugada syndrome and idiopathic ventricular fibrillation with a high J wave and elevation of the ST-segment are part of spectrum of phenotypic expression called the 'J-wave syndromes'
These recordings are sometimes intermittent and unmasqued by fever, vagal stimulation, or sleep, and disappear during an effort or following the administration of isoprenaline. All types can be present in a successive way in the same patient. Because the type 2 and 3 ECG patterns are less specific, pharmacological tests have to be performed to confirm the diagnosis: either with ajmaline (1 mg/kg in 5 minutes) or flecainide (2 mg/kg in 10 minutes).
The term of Brugada syndrome is used when an ECG type 1 tracing is associated with an episode of polymorphic ventricular tachycardia or ventricular fibrillation, with a family history of SIDS under the age of 45 years or with unexplained syncope .
Treatment with hydroquinidine seems effective. An implantable defibrillator is inserted in case of episodes of arrhythmias associated with an abnormal ECG at rest; it can sometimes be associated with a pacemaker in case of sick sinus syndrome.
Useful reference : www.brugadadrugs.org
Sometimes ones refers to a " acquired Brugada syndrome " when a typical ECG pattern appears under the influence of a pharmacological treatment (mostly continuous infusion of propofol where this change may announce a propofol infusion syndrome), with or without fever, in a patient whose ECG was previously normal.
Anesthetic implications:
If an internal defibrillator is implanted, turn it off before the surgical procedure because the electrocautery may trigger defibrillation; defibrillation patches should be placed on the chest to defibrillate whenever necessary during the procedure. Monitoring is adapted to the importance of surgery: it is prudent to use a 5-lead ECG and do not hesitate to insert an arterial catheter.
In case of intraoperative ST segment elevation, it is useful to start an infusion of isoproterenol.
Avoid:
- any vagal stimulation and beta -blockers;
- sympathetic stimulation: a perioperative analgesia of quality is necessary
- neostigmine which increases the elevation of ST segment in this context;
- hyperthermia;
Sevoflurane is the halogenated of choice. All intravenous anesthetics have been used without problem, including propofol in a single dose or as continuous infusion. However several reported cases of PRIS (Propofol Infusion Syndrome) presented with an ECG similar to a Brugada syndrome shortly before the occurrence of rhythm disorders and, conversely a few cases of arrhythmias following the administration of propofol have been reported in patients with Brugada syndrome.
Decreased doses of local anesthetics (which block Na+ channels ) especially bupivacaine (except for spinal anesthesia).
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Case Reports in Anesthesiology 2022: 3220486, doi 10.1155/2022/3220486
Updated: September 2022