Brugada syndrome natural history, complications and prognosis

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Natural History

Brugada syndrome usually becomes apparent in adulthood, although signs and symptoms, including sudden death, can occur any time from early infancy to old age. The mean age of sudden death is approximately 40 years. This condition may explain some cases of sudden infant death syndrome (SIDS), which is a major cause of death in babies younger than one year. It is characterized by sudden and unexplained death, usually during sleep. Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. This condition was originally described in Southeast Asian populations, where it is a major cause of death. Researchers have determined that SUNDS and Brugada syndrome are the same disorder.

Approximately 20% of patients with Brugada syndrome develop supraventricular arrhythmias.¹² Atrial fibrillation is associated in 10% to 20% of cases. Atrioventricular (AV) nodal reentrant tachycardia and Wolff-Parkinson-White syndrome also have been described.¹³ Prolonged sinus node recovery time and sinoatrial conduction time,¹⁴ as well as slowed atrial conduction and atrial standstill, have been reported in association with the syndrome.¹⁵ A recent study reported that ventricular inducibility is positively correlated with a history of atrial arrhythmias.¹⁶ In patients with an indication for an implantable cardioverter defibrillator (ICD), the incidence of atrial arrhythmias was 27% versus 13% in patients without an indication for an ICD (P<0.05), which suggests a more advanced disease process in patients with Brugada syndrome and spontaneous atrial arrhythmias. Inappropriate shocks from atrial arrhythmia episodes were observed in 14% of cases, highlighting the need for careful programming of the ICD.¹⁵

Complications

The following arrhythmias may occur in the patient with Brugada syndrome:

• Polymorphic VT resembling a rapid Torsade de Pointes (TdP)
• Monomorphic VT is observed infrequently
• VT/VF often terminates spontaneously in patients with the Brugada syndrome which may explain why patients wake up at night after episodes of agonal respiration caused by the arrhythmia.

Prognosis

Patients who are symptomatic with unexplained [[syncope], ventricular tachycardia or aborted sudden cardiac death may have a symptom recurrence risk of 2% to 10% per year. In these patients an AICD implant is advisable.