What Brugada Syndrome patients should do to help prevent a cardiac event

A British organization, Cardiac Risk in the Young, was established in 1995 to raise awareness of conditions that can lead to young sudden cardiac death. In the video found on this page, posted earlier this year, a consulting physician does an excellent job explaining Brugada Syndrome. Near the end, he discusses some things that patients should be aware of and avoid, in order to lessen the probability of a cardiac event.

He mentions the family of tricyclic antidepressants as being dangerous to the Brugada Syndrome patient and also warns patients to monitor their body temperature, as fevers are associated with increased probability of having a cardiac event if one has Brugada Syndrome.

Most patients with Brugada Syndrome know that it's primarily a passive condition, with no symptoms. Other than the inconvenience of having and maintaining an ICD, the typical healthy patient leads a normal life. It's easy to forgot how serious this condition is!

So, the wise patient keeps up with the literature on the syndrome. Since Brugada Syndrome is so rare, progress seems to move at a snail's pace. But every once in a while, an article, video, interview or event is discovered to help patients learn more about it, and this helps them manage their own lives and feel more in control of their own health and futures.

 

Brugada Syndrome and show business?

Last evening on the television show, House, starring Hugh Laurie, Brugada Syndrome was suggested as a possible explanation for the collapse of this week's diagnostically challenging patient.  Once in a while there are references to Brugada Syndrome in movies or television.

Wes Craven

Wes Craven first came up with a basic idea for the movie Nightmare On Elm Street after reading about the mysterious deaths of some young, healthy Cambodian refugees who had come to America to escape the reign of Pol Pot. It's been well documented that this phenomenon, now known as Brugada Syndrome, is seen more frequently among the Asian population and statistically strikes young males in every population more often than females. I've never seen the movie but have seen Brugada Syndrome mentioned as one of Craven's influences often enough to believe this little bit of trivia is probably true.



If you'd like to see a movie starring Brugada Syndrome, this modeling of the presence of Brugada Syndrome, from Elizabeth Cherry, of Cornell University's College of Veterinary Medicine, is mesmerizing. But this video that models a Brugada Syndrome event through stimulation of the heart muscle from the left is hauntingly beautiful even as it represents a potentially dreadful outcome.

In With The New, Out With The Old

My husband had his Medtronic ICD removed last week, and a new St. Jude ICD called Fortify was implanted in its place.


Unfortunately just prior to surgery, the surgeon discovered my husband was in atrial fibrillation.  What was hoped to be a routine procedure turned into more. Now he has to be on blood thinners for six weeks, go through a tranesophageal echocardiogram to check for clots, be treated for the a-fib (a shock was mentioned) and then go back under to have the new ICD and his old lead tested so the surgeon can sign off on it.


The surgeon couldn't test the unit upon implantation, because he has no idea how long my husband has been in a-fib or what the clot situation is.

My husband's primary concern before the surgery was the lead - it had been recalled a few years ago but many cardiologists, including his, agreed it should stay in place. But since a new device was being installed, the surgeon was going to closely inspect the old lead and replace it if necessary.


There was another surprise - my husband's heart rate dropped to 30 when they disconnected the old ICD. He'll follow up with his regular cardiologist on all this next week.

In What Circumstances Should An ICD Be Removed?

My husband and son had their Medtronic ICDs implanted in the fall of 2004. In the meantime, both models had the leads recalled but on the recommendation of their health care providers, they opted not to have them replaced.

Now my husband's ICD battery is running low. His cardiologist tells him that he has to have the unit replaced within a few months. He doesn't want it replaced. He wants it removed, once and for all.

The ICD has never misfired. That's not the problem. It's twofold. First of all, my husband was diagnosed with Brugada Syndrome within a week of our son's diagnosis. While our son's diagnosis was strong based on many factors, my husband's was not. Brugada Syndrome is statistically more likely to strike males between the ages of 25 and 45. My husband is 60. And his VF was not as easily induced as our son's was during the EP study. Finally, I'm reading now that the earlier studies that determined a relationship between EP study results and the likelihood of future cardiac sudden death has been challenged through later studies. So perhaps if this had happened to my family in 2011 rather than 2004, he would not have been implanted with an ICD.

In addition, he just absolutely hates it. He complains constantly about the fact that he cannot sleep through the night because of the ICD. He awakens many mornings with great soreness at the side, because he's a lifelong left-side sleeper and he can't seem to break himself of the habit of rolling over onto that side during the night, only to be awakened by discomfort and fall fitfully back to sleep on his right side.

When he explained this to his cardiologist, he was basically told that he had no choice. The ICD had to be replaced. Period.

I guess I'm not exactly sure what to think. Is it truly that rare to remove the ICD? What happens to the leads if the ICD is removed? It probably doesn't make sense to remove them, since by this time I'm sure they've been embedded into the surrounding tissue by now.

One Mother's Story About Brugada Syndrome

There is a temptation to focus on the technical advances that help protect individuals with Brugada Syndrome from experiencing sudden cardiac death. New types of ICDs, studies about quinine or quinidine treatments as an alternative to ICD implantation in certain cases and so on.

What we can't forget is that Brugada Syndrome is a real disease with terrifyingly real consequences for people who are struck down before they are diagnosed. Since Brugada Syndrome was identified in recent years, the medical community is not completely up to speed on the condition, within the United States and throughout the world.

Consider the heartbreaking story told by this mum in the United Kingdom, and her years-long struggle to confirm the diagnosis of Brugada Syndrome for her young son. From the National Genetics Education and Development Centre.

Twiddler Syndrome - No Fooling

I'm serious. Such a condition exists and it has to do with ICDs. Wait for it...Twiddler Syndrome is caused by the patient twiddling with his or her newly implanted ICD to the point where the leads dislodge, rendering the ICD ineffective.

You can read more about Twiddler Syndrome and other conditions that might be encountered by an Emergency Technician here in Emergency Medicine.

Xray photo from article.

Medtronic introduces Protecta line of ICDs in United States

Found this posting on medgadget blog, describing a new ICD by Medtronic. Introduced in Europe in summer of 2010 it is now cleared for use in the United States.

The Protecta line of ICDs should allow patients who fear inappropriate shocks to breathe a sigh of relief. "Findings from the Virtual ICD study, based on a statistical model, shows that 98 percent of patients with SmartShock Technology will be free of inappropriate shocks one year after implant and 92 percent will be free of inappropriate shocks five years after implant."

Might be worth a discussion with your health care team if your ICD, like my son's and my husband's, is near replacement time!

ICD Wearers: Do you realize how valuable you really are? Technically, I mean...

This article was posted on Facebook today.

It's entitled, "Oops...ICDs Don't Bounce" and is a rather unique look at the value of that little box of magic called an ICD.

(The doctor's blog is worth checking out, by the way!)