Greetings. I have arrhythmia for so many years but I can’t say that I saw that this disease is getting worse or better. It was always the same – at least I was thinking that it was the same. I went to see my doctor last week, to do some ordinary tests and analyses. Today he called me and he told me that I have, I quote “inherited arrhythmia syndromes”. I don’t know what this means and I don’t know did I get this like I should, but I want to know what are the diagnosis and management of patients with inherited arrhythmia syndromes?
Hey there Jake. Look, inherited arrhythmia disorders that are associated with structurally normal heart can cause nearly of 9 or 10 percent of almost 1 million of sudden deaths in Europe and in the USA. I found this lately and that I started to read a little bit about this, especially since my maid of honor’s mom was diagnosed with it.
Inherited arrhythmia disorders can cause a loss of consciousness or even the dangerous heart rhythms. There are a variety of inherited arrhythmia disorders and they are inherited form your mother or your father.
Diagnosis and management still depends on the type of arrhythmia.
Hey all. Here is what I know about this topic. Inherited arrhythmic disorders is actually a group of syndromes. All these syndromes have different, unique genetic abnormalities and presentations. So they are different by nature, but they have very similar clinical outcomes. And also the complications that could occur as a result of these syndromes are very similar.
I read a lot about this because my very close friend has Brugada syndrome and this is one of the syndromes that go under inherited arrhythmic disorders. That’s why I know all these things. There is also Long QT syndrome, but I don’t know much about it.
These disorders include: long and short QT syndrome, early repolarization syndrome, Brugada syndrome, idiopathic ventricular fibrillation and catecholaminergic polymorphic ventricular tachycardia.
You asked about diagnosis. Well they use some genetic tests but not always. I think that the majority of patients have echocardiography and then they do Hotler and stress test.
Sometimes magnetic resonance imaging is recommended for the diagnostic workup of Brugada Syndrome, early repolarization syndrome and idiopathic ventricular fibrillation. It is more often recommended for this group than for more often than for long and short QT syndrome and catecholaminergic polymorphic ventricular tachycardia.
For some patience it is good to have coronary angiography, for example in the work-up of idiopathic ventricular fibrillation.
I read that the most common therapy in Long QT Syndrom is drugs. Great majority of patients is treated that way, probably over 75 percent. Around 12 percent of people take drugs and have implantable cardioverter-defibrillator.
And for Short QT Syndrome, in almost 50 % of the patients implantable cardioverter-defibrillator is used. Drugs take the second place with approximately 20 percent of people, and then there is around 15 percent that use both implantable cardioverter-defibrillator and drugs.
I can't really recall the statistics for Brugada syndrom but I will find it, I have it somewhere in PDF and I’ll post it here.
Well I need to tell you that I don’t have a clue about it at all so I am glad that I found this topic. Why? Because a couple of days ago, one lady that practically raised my brother because my mom and my dad were working a lot and I was at the college out of my city, was diagnosed with this disease. Since that she is struggling to find the proper treatment, she wants to know how to manage this disease properly and so far she didn’t find the way. I am glad because I found it and I am going to tell her all this right now.
Here it goes, I found it.
In patients with Brugada Syndrom the first treatment option is implantable cardioverter-defibrillator, around half of all the people with this syndrome use it. And the other half uses several different options. Some of them use only drugs, or to be precise eleven percent. Eight percent of them use both implantable cardioverter-defibrillator and drugs. And 30 percent of the patients don’t use anything.
And for the early repolarization syndrome, 29 percent use implantable cardioverter-defibrillator. Sixteen percent use drugs. Three percent use both ICD and drugs and believe it or not more than 50 of people don’t use anything.
Adria, thank you for this amazing explanation. I was always wondering what this is and what is the best management for the patients with inherited arrhythmia syndrome. I was pretty sure that I don’t know anyone who had this diagnose, but that I remember that a little girl, a daughter from my friend. (she is 8 years old) was diagnosed with this disease.
Since then she is always at some therapy treatments and I don’t even want to ask my friend how they are managing with it. I am sure that it is pretty hard.
I hope that she will be fine.
Hello everyone. Jake, those are irregular heartbeats like others already told you. There are two patterns of inherited arrhythmia disorders. There are almost 50 percent chance that your parent can transmit this gene to you. This is a first form.
The second form of inherited arrhythmia disorders is autosomal recessive and in this type you inherit one copy of the abnormal gene from booth – your father and your mother. Having those genes does not confirm that you will have any symptoms of this condition.
That is the diagnosis and the management depends on the stage and type of the disease.