This syndrome has a very poor prognosis when left untreated:

 

One third of patients having suffered from syncopal episodes or resuscitated from near-sudden death develops a new episode of polymorphic ventricular tachycardia within 2 years. Unfortunately, prognosis of asymptomatic individuals with typical electrocardiogram is also poor. In spite of not having any previous symptoms, also one third of these individuals presents a first polymorphic ventricular tachycardia or ventricular fibrillation within 2 years of follow-up (picture 16).

 

These data are of extreme importance for the delineation of treatment policies of these patients. Because antiarrhythmic drugs (amiodarone or beta-blockers) do not protect against sudden cardiac death, the only available treatment is the ICD, implantable cardioverter-defibrillator (picture 16). This device effectively recognizes and treats the ventricular arrhythmias.

 

When provided with the implantable defibrillator total mortality in patients with Brugada syndrome has been 0% with up to 10 years follow-up. These results are not surprising.

 

These patients are young and usually devoid from other diseases. Because the heart is structurally normal, and there is no coronary artery disease, these patients do not die from heart failure or complications of ischemic events. Thus, they are the most ideal candidates for treatment with an implantable defibrillator. Symptomatic patients must receive this device.

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Picture 16
Survival of symptomatic and asymptomatic patients with Brugada syndrome and survival of patients with Brugada syndrome according to treatment. Only the implantable cardioverter-defibrillator protects against sudden cardiac death.

On the other hand, major concerns arise in the treatment of asymptomatic individuals. In our previous report six asymptomatic patients died suddenly during follow-up. Four patients were members of affected families, but two were sporadic cases. Data from electrophysiologic investigations did not help us to predict prognosis at that time. Recent data support the fact that this may have been caused by a type II error (not sufficient number of patients to prove a statistically significant difference). At present, we believe three different groups of patients can be distinguished:

 

1.- Symptomatic individuals with the disease who require an implantable cardioverter-defibrillator. Patients with transient normalization of the electrocardiogram during follow-up have the same prognosis as compared to patients with a permanently abnormal electrocardiogram.

 

2.- Asymptomatic patients with a spontaneous abnormal electrocardiogram, and inducible polymorphic ventricular tachycardia or ventricular fibrillation who also require an implantable defibrillator.

 

3.- Asymptomatic individuals with an abnormal electrocardiogram only after drug chanllenge and no inducible ventricular arrhythmias who should not be treated but followed-up carefully for development of symptoms suggesting arrhythmias (particularly syncope). One has to realize, however, that these recommendations may rapidly change depending upon the availability of new data.