I’ve been to various gatherings where the conversation has turned into a family member dying “before their time.” One theme I kept hearing about was, “My uncle dropped dead of a heart attack while grilling some burgers and he was only 45 years old. Never smoked or drank in his life. He ran five miles each day!” As a medical student I was curious; “Why would a seemingly healthy person just drop dead? Of a heart attack no less? Without a family history?” That question was answered when I read an article by Dr. Ramon Brugada. He discussed a form of heart disease that was so silent and so deadly that the first symptom was death!
Brugada Syndrome, an eponym for the acclaimed physician, is a very rare form of heart disease, where the electrical signal of the heart goes haywire, causing it to beat erratically. This chaos can ultimately lead to death. You may be saying “well, there have to be some symptoms, some way I can tell that there’s a problem.” Not really. You see, often the symptom are vague. A couple of weird heart beats that you may feel in your chest or throat. You may even get a little light headed or dizzy (from the lack of blood flow to your head). Unfortunately, a common symptom is death.
In medical jargon, this sudden “dropping dead” is also known as “Sudden Cardiac Death Syndrome.” The lack of symptoms is compounded by several facts. Structural abnormalities, such valve or wall problems, are uncommon. There’s rarely any gunk (plaque) built-up in the arteries. The electrocardiogram (ECG/EKG) you get at your doctors may show very subtle changes that he or she may not pick up.
Hold your horses! Before you run to the doctor and tell them you read about some sort of heart problem that has to do with electricity and death and, “Oh my God, why didn’t you tell me I’m going to die tomorrow?!” Slow down, take a deep breath and continue reading.
Brugada Syndrome is relatively rare. Although no solid numbers on the incidence are available, there are approximation of 0.16%-15%.
There is a silver lining. First, this disease is genetic. The gene responsible has been found and the “why” of the disease is pretty well understood. Even more good news; if your doctor knows what to look for, they can see it on an ECG. This disease is treatable, but is not curable. One way to treat it is to put in an implantable cardioverter defibrillator (ICD). An ICD is a little device which is placed under your skin, below your shoulder, with one or two prongs in your heart. The ICD will give you a little shock if the worst should ever happen, a deadly, chaotic heart rhythm. Medications are available, but the jury is still out on those. Not all doctors agree that medications really work. You know the saying, “when you get 13 doctors in one room, you’ll get 13 different opinions.” The bad news for us men is that we are nine more times more apt to have this than the ladies.
A great start is to make sure that if you’ve had a relative that “dropped dead of a heart attack” when they were young, tell your doctor. When you’re at the doctors office, tell them the truth and every detail of your family history. You never know what can save your life.