BRUGADA SYNDROME: DIAGNOSIS, ORIGIN, RELATION WITH OTHER SYNDROMES
Sri Lakshmi Narasimha College of Pharmacy, Palluru,
Chittoor District, Andhra Pradesh-517132, India.
Brugada syndrome is a genetic cause of sudden cardiac arrest characterized by abnormal electrocardiographic (ECG) pattern in the right precordial leads either at rest or after provocation. In this condition, sudden death may occur due to polymorphic ventricular tachycardia or ventricular fibrillation. In approximately 30% of patients, sudden cardiac arrest is the initial clinical manifestation of Brugada syndrome.
Brugada syndrome (BrS) is a genetic cause of sudden cardiac arrest (SCA). In this condition, SCA occurs due to the development of either polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF). In approximately 30% of patients, SCA is the initial clinical manifestation of BrS. When VT or VF occurs, it tends to be in the evening hours. This is thought to occur because of circadian rhythm. Patients with BrS do not have structural heart disease on echocardiogram, stress testing, or cardiac catheterization. However, a standard electrocardiogram (ECG) usually shows the characteristic abnormality.
Reference Id: PHARMATUTOR-ART-1604
The syndrome of right bundle branch block, persistent ST segment elevation and sudden death was described in 1992 as a new entity causing unexpected sudden cardiac death (SCD). The original description of eight patients was followed by case reports and subsequently by numerous publications focusing on the clinical characteristics of greater populations of patients, and publications defining the genetic, molecular and cellular aspects of this disease that became known as Brugada syndrome. The number of scientific publications dealing with the syndrome has increased substantially in the last years, and continues to do so , reaching an average of 20 publications per month in the first semester of 2009. The description of this syndrome became one of the landmarks in the integration of cardiology, rhythmology, electrophysiology, genetics, epidemiology and many other medical disciplines in the fight against cardiac diseases, and in particular sudden unexpected cardiac death in the young. This paper reviews the past and current knowledge concerning the clinical, genetic and molecular features of Brugada syndrome.
What is Brugada syndrome?
This is an inherited arrhythmia that causes the bottom chambers of the heart (the ventricles) to beat so fast that they can prevent the blood from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset. While this is a disease that usually affects people in their 30's, it has actually been described at all ages. So it is important to screen everybody in a family. Not everybody who has the disease will have arrhythmias. However, we cannot know yet who will be OK and who will have problems. If you have had fainting spells related to Brugada syndrome, our experience indicates that you are at very high risk of having them again.
How common is Brugada syndrome?
It is very difficult to know because it was only described 10 years ago. It is found all over the world and in some countries in Asia is the most common cause of death in young people after car accidents.
Is there any medication for Brugada syndrome?
Not yet. Brugada syndrome was discovered 10 years ago, and there is no medication to prevent the black outs yet.
Is there anything that resets the heart when it beats too fast?
Yes, there is a special pacemaker, called defibrillator, that when used in patients that have Brugada syndrome and black outs, jump-starts the heart every time it malfunctions. All the patients who have a defibrillator are doing well.
If one of my parents or siblings has Brugada syndrome what do I need to do:
Brugada syndrome may be inherited, that means passed from parents to children. So it is important that you follow some important steps:
· Contact your physician who will probably perform a physical exam and an electrocardiogram. Brugada syndrome can be seen in few occasions right away in the electrocardiogram, but sometimes, to see it, it requires the infusion of a medication.
· If the electrocardiogram shows Brugada syndrome or if you had some signs that your heart malfunctions, like loss of consciousness, fainting, passing out, your physician may advise you to undergo an EP test. This is a test that checks your heart's electrical activity and whether there is any malfunction.
· Because Brugada syndrome is inherited, the physician may request that you give a blood sample to perform genetic analysis in you and your family.
Talking to your physician is the first thing to do and he will advise you.
The diagnosis of the syndrome is easily obtained by electrocardiography as long as the patient presents the typical electrocardiographic pattern and there is a history of aborted sudden death or syncope caused by a polymorphic ventricular tachycardia. It is difficult to forget such a typical electrocardiogram. The ST segment elevation in V1 to V3 with the right bundle branch block pattern is characteristic. The ST changes are different from the ones observed in acute septal ischemia, pericarditis, and ventricular aneurysm and in some normal variants like early repolarization. There are though, electrocardiograms which are not as characteristic (picture 6), and they are only recognized by a physician who is thinking of the syndrome. There are also many patients with a normal electrocardiogram in whom the syndrome can only be recognized a posteriori when the typical pattern appears in a follow-up electrocardiogram or after the administration of ajmaline, procainamide or flecainide (picture 2).
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