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Long QT Syndrome (transcript)
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Major funding for Second Opinion is provided by the Blue Cross and Blue Shield Association, an association of independent, locally operated and community-based Blue Cross/Blue Shield plans, supporting solutions that make quality, affordable health care available to all Americans.

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(Peter)
Welcome to Second Opinion, where each week our health care team solves a real medical mystery.  When we close this file in a half an hour from now, you’ll not only know the outcome of this week’s case, but you’ll be better able to take charge of your own health care and doctors will be able to listen to patients more effectively.  I’m your host, Dr. Peter Salgo; you’ve already met our special guests who are joining our primary care physician, Dr. Lisa Harris.  Lisa, good to see you again.  No one else knows, other than I, this case and we’re going to get right to it.  Let me tell you a little bit about Jared.  Jared is 60 years old, he lives in an active senior-living community.  He lives in Florida.  Jared describes himself as fit, he exercises regularly.  While playing golf one hot Florida afternoon, he faints.  His golf buddy calls 911 and Jared is rushed to the local emergency room.  You’re there, Lisa; what are you going to do?

(Lisa)  
Well, of course you want some additional history, and as usual we want to get a work-up of additional vital signs.  So we want to know if he’s had any previous episodes of passing out, has he been feeling weak or dizzy or ill…

(Peter)
There’s nothing in the chart from the emergency room that indicates that they have this kind of history.  I’ll tell you what we’ve got.  He was alert once he got to the emergency room – he actually woke up in the ambulance.   In the ER, the vital signs were 120/80 with a regular pulse; they did a neuro exam, which was normal.  His chest exam was normal; his pulse actually was 70 and regular.   So, what’s your differential?  The guy just fainted.

(Lisa)  
For someone who has an acute *syncopal episode, so he suddenly passes out –

(Peter)
Syncope means passing out.

(Lisa)  
You certainly have to worry about cardiac arrhythmias or irregularities in the heart due to the electrical system in the heart, so he should get an EKG at least for starters.

(Peter)
Jared was talking to the emergency room doctor; he said – and they recorded this in the chart because they thought it was significant – that the week before this event, he felt some fluttering from his heart in his chest, but they went away, these flutters.  What do you make of it?

(Art)   
Well, it could be a couple of things.  In his age group – he was 60, if I’m not mistaken – one could be concerned on one side that this could be atrial fibrillation, because this is frequently picked up –

(Peter)
That’s an abnormal –

(Art)   
That’s an abnormal, irregular rhythm from the upper chamber.  But it could also be a series of extra heart beats from the lower chamber, so it’s – one’s only playing the percentages based upon that information.  And as Lisa said, one really needs an electrocardiogram to see what’s going on.

(Peter)
I’m going to give you more information, but I just want to touch on one thing.  He had some fluttery stuff in his chest, was he right to ignore it?

(Lisa)  
No.  He should’ve had that checked out right away.

(Peter)
Yeah, but you have the benefit of hindsight here.

(Sam)  
Yeah.  I mean, men are going to be less likely to seek care in general, but in particular, the perception of a symptom – particularly the perception of a heart symptom seems like it would drive care but the literature is full of data that suggests many people delay care in what would be logical that they would seek care.  In fact, as we all know, there are a number of studies underway to look at why people delay with chest pain and heart flutters.

(Peter)
Let me give you some more information.  They do an cardiogram in the emergency room, and the one thing they record – and I don’t have the trace for you – is that the QT interval is 540.  All right, do you want to translate that into English?

(Art)   
Well, normally the heart is – it has an electrical trigger to the heartbeat and there is the discharge of the electrical activity that gives rise to the heart’s contraction.  And then there is the electrical recovery, and the electrical recovery has to take place before the next heartbeat.  The time interval of this electrical recovery is sometimes measured in the QT interval.  This is the time interval on the electrocardiogram, but it’s basically the recovery.  The value that you gave is very prolonged; it’s abnormally prolonged.

(Peter)
Lisa, how do you explain this to the patient?

(Lisa)  
Pretty much what Art just said. 

(Peter)
That explanation, again, is that you’ve got a lot of – what’s the name?

(Lisa)  
It’s called prolonged QT syndrome.

(Peter)
Prolonged QT syndrome, which is an electrical –

(Lisa)  
That’s probably not what I would tell the patient.  I would explain to them that their heart has an electrical rhythm just like the wiring in your house and if it short-circuits, bad things can happen to you.

(Peter)
Is it fair to divide heart problems into plumbing and electricity?  Plumbing is heart attacks and heart muscle death and electricity is how the heart muscle signals itself to beat.

(Raymond)     
That’s one of the real problems; people use ‘heart attack’ pretty generally.  As you said, when the plumbing – the blood vessels to the heart – have a blockage, that’s what I call a real heart attack.  An arrhythmia is when the heart rhythm is abnormal – either it stops or it goes too fast or too slow.

(Art)   
We tend to think of either-or; it’s electrical or it’s mechanical.  Sometimes it can be both.  With a heart attack, with the blockage of a blood vessel, you can have damage to the mechanical function and that damage can trigger an electrical disorder.

(Lisa)  
I go back to the house; if your plumbing gets blocked, if you have an ice flow blockage in your plumbing and your plumbing ruptures and your house floods, all your electricity is going to short-circuit too.

(Peter)
What causes this prolonged QT syndrome?  Aren’t you just born with it?

(Art)   
You can be born with it. There is an inherited, hereditary form of the disorder.  We usually see it in younger patients, but those who survive to the older age can have it.   In his age group, one would be more concerned about an acquired form, possibly drug-related, possibly due to certain viral disorders or what have you…

(Peter)
So if he had a predisposition for this, medication could bring this out?

(Raymond)     
That’s very often the case.  When you look at people who’ve had drugs that induce arrhythmias, you go back and you find out that they had a silent situation where their genes – everything seemed perfectly normal, but the drug brought out the genetic problem.  But you can also just have it without the genetic component.

(Peter)
How bad is prolonged QT, or how bad can it be?  Can it kill you?

(Mary Jo)      
Absolutely.

(Peter)
How do you know this?  You’ve got it, but you’re still here.

(Mary Jo)      
I do and I’m still here.

(Peter)
Tell me a bit about your story.

(Mary Jo)      
In 1979, my 17-year old sister suffered cardiac arrest.  She had for 3 years prior to that been having *syncopal episodes.  When she would have these fainting, *syncopal episodes, she would always look like she was seizing a bit but she would come out of it.   So it was assumed that she had some type of seizure disorder and she was treated for that.

(Peter)
For epilepsy?

(Mary Jo)      
For epilepsy, right.  And just involved in a minor car accident, the startle effect of being involved in that accident was enough to cause the event that caused her cardiac arrest…  It was at that time my family learned that we had long QT syndrome. 

(Peter)
That was back in 1979 when it wasn’t very well known.

(Sam)  
It’s still not very well known.

(Peter)
It will be after today.

(Mary Jo)      
And I had been treated – and treatments can be as simple as a beta blocker that people normally take to lower their blood pressure.  And for the type of long QT that we have, it’s very effective.

(Peter)
So at this point you know the risks first-hand.  Let me tell you about Jared, though, because Jared doesn’t know any of these risks and Jared says in the chart here, ‘I had a cardiogram a year ago.  It was normal.  And now you’re telling me that I have an abnormal cardiogram, this long QT stuff and it’s dangerous.  How come they didn’t pick this up a year ago?’

(Lisa)  
What’s he taking?

(Peter)
Not in the chart.

(Raymond)     
I would also ask, let’s make sure it was normal.  We found about 5 to 10 percent of the machines miss it and a lot of the physicians miss it.  It’s encouraging that it was called normal, but I would verify that.

(Mary Jo)      
The thing we know about the ECG is that with long QT, it can change.  I had a number of ECG’s done in my early adulthood and in my 40’s and it never showed up; I never had a prolonged QT interval.   Then I did have one that showed it was prolonged, so it can change.

(Peter)
I have more information for you about Jared.  Would you like some more? 

(multiple voices) Yes

(Peter)
I thought you would.  Jared is admitted – they don’t send him home – and the doctor gets more of a history.  Jared says he recently retired to Florida from where he calls ‘up North’.  And he developed allergies – here it comes – when he moved to Florida.  His allergies are being treated with a daily prescription antihistamine.  Now, he’s not on any other medication; does that ring any bells to anybody?

(Art)   
It should for Ray, because Ray is the expert on this.

(Peter)
Well, tell us, Ray.  I think they just put you on the hot seat.

(Raymond)     
It’s a difficult story because antihistamines were, for many years, considered like food; people would take them anytime they wanted.

(Peter)
They were over the counter.

(Raymond)     
They were over the counter, but then there came Seldane – the first non-sedating antihistamine – and people started dropping dead. They were often women; far more than men, actually.  And we became aware that there were several antihistamines on the market that were causing prolonged QT and sudden death.  Most of those have been taken off the market, though.

(Peter)
What are the real offenders here, real or otherwise?  What drugs can cause the QT to get long?

(Raymond)     
More often, it’s the drugs for the heart – the drugs you take to improve heart rhythm can actually prolong the QT.  A little bit may be okay, but some of them have very high instances of prolonged QT.   So the heart drugs are always the first you think about.   Antibiotics are often a problem – some, but not all – and antihistamines for a while were a major contributor to this.  Anti-depressants, anti-psychotics – every drug class that we use today, even drugs for cancer, has within it one or two drugs that can do this. We maintain a website where we list probably 30 drugs that are known to cause prolonged QT and sudden death.  We have another list of about 30 drugs that prolong the QT; we don’t know if they cause sudden death.  So this is a constant area of research. 

(Art)   
But there are also drugs we don’t know that could do it that aren’t on the list, too.  You have to be suspicious.

(Raymond)     
That’s true too.  It’s even more complicated because of additional factors like gender.  Women are far more sensitive – it’s actually the other way around.  Men are resistant, their heart is insensitive, my wife tells me.

(Peter)
I’m sure she’s speaking *cardiologically…

(Male)
No, she’s talking about me!  {laugh} But the heart is hormonally regulated and that’s something we always thought of as a muscle, but it’s actually affected by hormones also.  So women are more at risk.

(Peter)
What puts you at risk, in terms of all comers, for prolonged QT and sudden cardiac death?

(Art)   
Well, if you’re talking about this prolongation of the electrical recovery, if it’s in a younger patient that’s not on medication you would think of an inherited disorder.  That would come to mind first.  If you’re talking about a woman in middle life, they are more susceptible to certain types of medications that are commonly used and can be associated with this long QT inherited disorder that may not manifest itself until the 30’s or 40’s.  If you’re talking about somebody in the 60 age group, you’re talking probably multiple causes – maybe drugs, maybe underlying heart disease that puts them at increased risk, any number of things, so… you really have to be thinking in age and gender-specific aspects of this disorder.  I think that’s helpful.

(Peter)
What do you do if somebody – Lisa sends you a cardiogram, sends you the patent, you’re going to see this patient; what is the cardiologist going to do?

(Art)   
Well, hopefully the big question is for he or she, what’s the cause of this and how you go about identifying the cause will require – you might get the information just from the electrocardiogram but if you don’t, you might want to make sure there’s not some mechanical disorder so you might do an echocardiogram. 
In view of the palpations, you might want to get a more prolonged recording; that’s referred to as a halter recording, and you might pick up some palpations that would give you an idea of what’s going on.   There is a whole diagnostic work-up that you would start with the simplest and move to the more complex.

(Peter)
What do they tell you?  What is it like living with a disease that at any moment may reach out and bite you and kill you?

(Sam)  
Well, there’s no question that it’s the uncertainties that go with the unknowns.  I think all of us benefit in a way from – we convince ourselves that we’re invulnerable to lots of bad things that can happen to us.  When families are faced with this, they’re faced with breaking down that denial system that keeps us all kind of healthy in a way.  And then they have to deal with it straightforwardly.   I think the other thing we have to work with our patients on and talk with our patients about is survivorship.   You know, overcoming these challenges despite the fact you know they exist.

(Art)   
Just hearing Mary Jo’s response, I got the feeling that you’ve kind of controlled your emotions.  I wonder if this is just your natural personality, or is this something that developed because you have this condition in which emotions can play a role?

(Mary Jo)
Well, I like to say that because I take a beta blocker that slows down my heart and also maintains my blood pressure that I’m fairly calm all the time.  But I’ve had 30 years since my sister’s cardiac arrest to learn about this.  I focused my career on learning about heart rhythm disorders, specifically long QT.  And because I work with patients and families, I need to be calm; I need to be a calming effect for them.

(Peter)
But the import of your question, I suspect, was emotional trauma, emotional stress; sudden shocks can precipitate arrhythmias.  Have you trained yourself to maintain an even keel so as to not have that happen?

(Mary Jo)      
I have.  There are moments, but I have.  I know it’s important for me to remain calm.

(Peter)
So the answer’s yes.  Let’s pause here for just a moment.  I’d like to sort of sum up a little bit of what we’ve been discussing; it’s been wide-ranging stuff – interesting stuff.   Long QT is a syndrome; it’s an electrical problem in the heart.  It’s a complex problem that can be affected by medications as well.  It can lead to sudden cardiac death and if you are, or may be at risk, you need to be monitored.  A cardiologist is one way to do that; a primary care physician can help.  Lisa, correct?

(Lisa)  
Absolutely.  I think we had started to talk a little bit about that before the summary.  It’s important how you craft the message and the relationship you have with your patients because you want to make sure, as you were saying, they understand that it’s serious but that doesn’t mean you need to wrap yourself in a cocoon and end your life.   How do we help you achieve some normalcy to your life, understanding that you have a serious condition?

(Raymond)     
As a physician, I’ve found it really hard because you want them to know it’s serious so they can adapt their life the way you have, but at the same time, not paralyze them.

(Mary Jo)      
You don’t want – and I’ve heard this from families; their child now becomes disabled.

(Raymond)     
Right.

(Peter)
We know that Jared has drug-associated long QT syndrome.  At least that’s what his doctors think they know.  What do you want to do?

(Art)   
Well, if you really feel it’s the drug and there is good evidence to incriminate a specific drug he may have been taking, the simplest thing is to take him off the drug and see what happens to his electrocardiogram – whether that electrical recovery, that QT interval normalizes, and if it does, then you have a very good cause and effect relationship.  A lot depends upon what took place.

(Peter)
Supposing he maintains his long QT after the antihistamines are stopped?

(Art)   
Then he has an underlying cause and you would be a little bit more concerned now that this could be a genetic disorder.  There are many different ways one can handle this; one can ask him if he has any siblings or children and one could take an electrocardiogram on them so if his son or daughter had the same pattern on the electrocardiogram you could make a family diagnosis.

(Peter)
You mentioned you were taking a beta blocker.  Why did they put you on a beta blocker?

(Mary Jo)      
The beta blocker keeps my heart rate from going too high.  If my heart rate becomes too fast, too high, I can have that classic type of ventricular tachycardia called Torsades de pointes.

(Peter)
You, however, had an unavoidable stressful moment.

(Mary Jo)      
I was having a cerebral angiogram and the perfect storm happened in the recovery room.  My potassium was low because I had been off of fluids and food since the night before, the wound site bled, and because of the manual pressure applied by the nurses there was this ‘startle effect’, pain effect that can actually cause, trigger the arrhythmia.  I lost consciousness and arrested.

(Peter)
So you had to be resuscitated?

(Mary Jo)      
Yes.

(Peter)
Then what did they do?

(Mary Jo)      
They gave me an implanted cardio-defibrillator.  The implanted cardio-defibrillator, also known as an ICD, is a generator.  It’s a small device that’s implanted just under the skin in my chest –

(Peter)
Just like a pacemaker.

(Mary Jo)      
Just like a pacemaker, and it in fact has a pace-making function in it so my heart is paced if it gets too slow.  There are wires or leads that go into my heart; one to pace the upper portion of my heart and one in the lower portion, if I were to then have another cardiac arrest.

(Peter)
If your heart stops, it shocks your heart.   And by the way, I heard you murmur that it’s changed your life.

(Mary Jo)      
It’s a life-changer.

(Peter)
How?

(Mary Jo)      
I finally, now after all these years of being afraid of long QT syndrome, seeing what happened to my family, I’m no longer afraid that I’m going to have cardiac arrest and die.

(Peter)
You have a safety net.

(Mary Jo)      
I have a safety net.  I have patients that say this device is their angel on their shoulder and I now feel that I can go places by myself, I can exercise, I have freedom and before I was always worried, could I do things that I did before?  And now I can.

 

(Peter)
Sam, you work with people who have these implanted defibrillators.  What is it like?  Does it have a psychological impact on people that have them?

(Sam)  
Absolutely.  This is my life’s work – how to help people deal with heart rhythm disorders and then how they deal with defibrillators and then go live life with them.   Our research has been focused on how patients are able to acknowledge the potential threats of the disease, and also then come to some peace with the treatment, which is a defibrillator that’s going to keep them safe.

(Peter)
Let me tell you some more about Jared.  It’s two months later; I presume he’s out of the hospital, Lisa.  He’s been off the antihistamines, no other fainting spells or heart issues.  How common is this? He’s back to what he considers his baseline.

(Art)   
If it’s drug-related, that’s the right course of action. 

(Raymond)     
The challenge is staying away from those medicines because they’re out there.  There’s another message I try to emphasize and that is that patients have to become informed.  They shouldn’t ignore this; they should immediately start studying this.  This is true for all patients but the other part of it is, we often say you are the most important member of your healthcare team but it has to be a team.  There has to be communication amongst doctors.  I’ve seen patients who would go have this exact case, go to a new doctor or go back to their internist who hadn’t been informed of everything and get this exact same medicine again.   Because there are 15 names for most medicines and how do patients know?

(Peter)
Let me pause for a moment and sum up where we are; as frightening as long QT syndrome can be, it’s important to know there are long-term effective treatments.  The critical thing to know is that you might be at risk and then you’ve got to ask for help because otherwise you’re not going to get it.  Speaking of that, ((Art)), I think it’s time to point out that you’re not just your average cardiologist with an interest in this; you set up a national registry for this.  Tell us about that.

(Art)   
Well, we did set up a national registry and it’s become an international registries.  We have over 1,000 families, 5,000 patients – we’re now into the third generation of patients with this inherited disorder. But then we’ve also realized that this disorder can be acquired, and this is where all the work that Ray has done has come in.  It’s become a model for other types of inherited disorders, and there is a whole spectrum of them; long QT syndrome is only one of several inherited disorders that can cause life-threatening heart rhythm disorders.

(Raymond)     
And it’s not just a registry where people learn about the disease.  The fact that beta blockers work is only because of that registry.  You couldn’t do a study – the disorder is so rare that most doctors don’t see enough to do a study.  It was only through that registry that we’re now saving lives by using beta blockers.

(Sam)  
I think one of the things that patients often don’t know about us who take care of them, we have tremendous admiration and respect for their commitment to life, their ability to tolerate the unknown.  What’s neat about this is that, as serious as these disorders are, you see tremendous courage.  We have a window of courage on peoples’ lives. It makes you speechless.

(Peter)
We just want to tell you that reading your story, and listening to you, folks may feel you’ve been dealt a terrible hand – a pair of deuces maybe – but you’ve made something remarkable out of it.

(Mary Jo)      
The best thing that happened in my life was to be able to be involved with this because we know that every day and every time we talk to a family, we’re helping them.  We’re saving lives by helping them, and helping them have a full life.

(Peter)
Congratulations, and thank you so much for sharing your experiences.  Thank all of you for being here; it’s been a great broadcast but it’s time to sum up what we’ve been talking about and let our viewers move on.  So long QT syndrome is an electrical problem in the heart. It can be associated with medications as well; it’s a bit more complicated as well, but it can lead to sudden cardiac arrest.  If you are, or may be, at risk you need to be monitored by a cardiologist – certainly somebody who knows what he or she is doing.  As frightening as long QT syndrome can be, it is important to know that there are effective treatments.  The critical thing to know is if you might be at risk, the first thing is to ask for help.  That’s the first step.  And our final message is this:  Taking charge of your health means being informed and having honest communication with your doctor.  I’m Dr. Peter ((Salgo)) and I’ll see you next time, on another ‘Second Opinion’.
  
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Major funding for Second Opinion is provided by the Blue Cross and Blue Shield Association, an association of independent, locally operated and community-based Blue Cross/Blue Shield plans, supporting solutions that make quality, affordable health care available to all Americans.  Additional funding provided by…