A Conversation About Sudden Unexpected Death (SUDA) in "Healthy" Adults, Adults With Known Heart Disease, Athletes, Adolescents, and Infants (SIDS)

Eric N Prystowsky, MD; George D. Lundberg, MD


December 19, 2007


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Dr. Lundberg: Good morning and welcome. I'm Dr. George Lundberg, Editor in Chief of Medscape General Medicine, and we're here in Orlando, Florida, at the annual meeting of the American Heart Association to do a Webcast video interview with Dr. Eric Prystowsky. He is a practicing cardiologist at St. Vincent Hospital in Indianapolis and a consulting professor of medicine in cardiology at Duke University, where he spent much of his time. He's also a past president of the Heart Rhythm Society. Welcome, we're very happy to have you with us today. Thank you for taking time.

Dr. Prystowsky: Thank you, George. I'm delighted to be here. Thank you.

Dr. Lundberg: Many things separate human beings from death, but how well the heart works in terms of rhythm may be the most important, at least in terms of sudden death. Is that right?

Dr. Prystowsky: That's correct and you're spot on. If you actually look at the statistics in the United States, the most frequent mode of death for adults is sudden cardiac death, over 350,000, so it's a big problem.

Dr. Lundberg: Yeah, well that immediately causes me as a pathologist to challenge those numbers because they probably come from things called death certificates. And everybody knows that the interns who sign out the patient in a hospital are going to write down cardiac failure as the first thing on a death certificate. Where do the numbers really come from?

Dr. Prystowsky: They are not hard data, you are correct in that. They come from death certificates; they come from epidemiologic studies. The CDC has put out reports that have used those numbers; the CDC has put out a report of 300,000 or 450,000. I would say it's a questionable number, but certainly I don't think anybody would say it's under 200,000 a year.

Dr. Lundberg: It's a big number.

Dr. Prystowsky: It's a big problem.

Dr. Lundberg: It's no question it's a big number.

Dr. Prystowsky: Yes.

Dr. Lundberg: Well, why don't we slice and dice this a little bit in terms of age groups. Let's talk about somebody, a man, who's 50. What's the risk for sudden unexpected cardiac death?

Dr. Prystowsky: Actually, I don't know the epidemiology data that close to tell you exactly what that person's risk is without all the other information you need, like cholesterol, hypertension, and so forth. But in the coronary-prone age group, which this person would be, their most common problem is going to be sudden death. If you look at it that way, it's going to be coronary disease. In other words, I would shift the emphasis a little bit vs epidemiology and say in that age group what's the most common cause of sudden death is clearly coronary disease, whereas in the younger age group it's a different pathophysiology.

Dr. Lundberg: Yeah, indeed. And of course mostly when we talk about this, we're talking about people who are likely to have coronary artery disease and likely to get a plaque that ruptures or something like that.

Dr. Prystowsky: That's correct.

Dr. Lundberg: Why don't we switch it down a little bit to somebody who runs marathons and is supposed to be just about as immune from this kind of disease as anything, and yet, every so often, the newspapers report, like last week --

Dr. Prystowsky: Last week, right.

Dr. Lundberg: --somebody who seemed to be an ordinary completely healthy marathon runner who dies, who drops dead. I haven't seen the autopsy report, so I don't really know what happened, but what's going on with those people?

Dr. Prystowsky: That's a really good point. It's a statistical thing in the United States, because probably it differs in different countries, but in the United States if you're under 35 it's usually hypertrophic myopathy. It's usually noncoronary. There's a whole group of things, these long QT syndromes and things like that, but the most common would be a hypertrophic myopathy. That's the typical scenario of the young football player in high school who dies on the field, and they do an autopsy and they have a big thick ventricle. That's the biggest problem there. Then there's a lot of these sort of boutique diseases, like these genetic diseases that occur very infrequently, they're in that mix. But clearly hypertrophic myopathy is the number one killer in that group.

Dr. Lundberg: Diagnosable as part of a prefootball practice physical exam or something?

Dr. Prystowsky: You bring up a very important and controversial area, which is: what should we do as a country to prevent these things?

Dr. Lundberg: Yeah.

Dr. Prystowsky: Okay, so here's the problem, the problem is we could do what Italy does. Italy has a national program, and they've already shown they can prevent a lot of these. Well, they're picking them up, so presumably they're preventing these mortalities. But it costs a lot of money. And if you look at cost-effectiveness, on screening for school-age athletes, it's not cost-effective. These events are relatively rare, but they occur in young kids, and many of them are preventable. An EKG and an echocardiogram, when appropriate, pick up the vast majority of structural heart disease.

Dr. Lundberg: How about if it's just an EKG?

Dr. Prystowsky: It picks up the majority; the problem is it over-reads it. If you have an abnormal EKG, you must do some further evaluation. It wouldn't be fair, at that point, to stop.

Dr. Lundberg: Sensitivity high, specificity low.

Dr. Prystowsky: That's right. And I'm personally in favor of doing it, but I'll tell you, I'm in a minority when it comes to a national program. It's not cost-effective, I'll admit that, but I think there are a lot of things we do in this country that are not cost-effective.

Dr. Lundberg: A whole lot of things. Here we're talking about kids, kids who have their entire life ahead of them, that kind of thing, and it's always a tragedy.

Dr. Prystowsky: I'm in favor of it, personally, but if you look at it at a society level, it's not considered at the level of cost-effectiveness.

Dr. Lundberg: I understand that. If you're a primary care physician and you're in Indianapolis, and you do a lot of school physicals, a boy's 14, he comes in, and you say he's okay to play football. You don't do an EKG and you don't do an echo because that's not standard practice. The kid goes out, and in the first fall football practice he drops dead, are you liable?

Dr. Prystowsky: Probably not, actually.

Dr. Lundberg: In Italy, yes, but in the United States, no.

Dr. Prystowsky: In Italy, yes, probably. Well, because it's not standard of care. I think the doctor shouldn't be held liable for things that are not standard of care. If you're not required to do an EKG, I don't think you have to do one. I think the issue isn't that, as we've just said. The issue is should it be standard of care, and I think it should. If not that, at least have an AED on every available playing field so that at least you have a chance at early resuscitation.

Dr. Lundberg: Then you have to teach somebody how to use an AED.

Dr. Prystowsky: But they're very easy.

Dr. Lundberg: How many playing fields in high school football have AEDs?

Dr. Prystowsky: I don't know, but most do not. But I can tell you it's even the same in college. A study was done not too long ago looking at even at the university level; a large percentage still did not have AEDs available.

Dr. Lundberg: If it's a hypertrophic cardiomyopathy you're describing, will an AED help?

Dr. Prystowsky: It should, if you get to them quickly. You're not going to cure it if you get everybody, but if you look at the data, when you get to somebody within 1 minute when they go down, there's a fairly high salvage rate. If you don't get to them in early cardioversion, there's usually 10% survival. Even if you have 50%, it still doesn't take away the primary thesis I have: we need to start thinking about primary prevention.

Dr. Lundberg: I would see that as more important, in my personal view. Because you think about somebody who goes down on a football field, the first thing you think about now is a fractured neck and to keep them very, very still.

Dr. Prystowsky: Right.

Dr. Lundberg: In order to put on an AED, you don't keep them still. It's a tough problem. Let's move down a little bit. Sudden infant death syndrome, does arrhythmia have something to do with that?

Dr. Prystowsky: Many people think so. To be honest with you, when I talk to my pediatric EP colleagues, this is truly a grab bag. There's not one specific thing. Long QT syndrome has been identified in some of these people. As you know, noncardiac things have been identified. And, in fact, another study that I know of done also in Italy, getting EKGs within the first day or two of life has picked up, in some instances, some of these ion channelopathies. It's certainly not an area that I'm involved in, research-wise or even practically as an adult electrophysiologist. But it is a difficult area because it's not monolithic as far as a disease state. I think it's a grab bag, and there, I think, it's a different issue. If you want to go to an EKG on everybody who's 1 or 2 days old, I don't know of anyone who's advocating that at this point, but you'd almost have to, to pick up all these long QTs and EKG abnormalities.

Dr. Lundberg: By creating an acronym SIDS for sudden infant death syndrome, it was made a public health issue.

Dr. Prystowsky: Yes.

Dr. Lundberg: And many interventions have been possible that have had good value. When I was doing a sabbatical in Sweden about 30 years ago, I created an acronym called SUDA, sudden unexpected death in adults. I studied personally, by autopsy, 100 consecutive adults in the country of Sweden in a forensic medicine setting who were found with sudden unexpected death. Nobody knew why they died and they couldn't figure out. They came to our medical examiner, or a coroner-type system in Sweden, and I autopsied 100 consecutive. I found that in the United States in most medical examiner's offices, these people would be signed out as a cardiac arrest of some kind, most likely because there are no external signs of violence or other kinds of foul play indications. I found that 50% did die from heart disease that I could tell at autopsy and 50% did not, they had a grab bag of other conditions. As far as I'm concerned, in the United States, if you have no history of any kind of heart disease or any kind of disease, and you're found suddenly dead, unexpectedly, it's a coin flip: 50% have heart disease and 50% of them did not die of it. That study was published in JAMA a long time ago, probably before you even were reading.

Dr. Prystowsky: To be fair about it, though, it's not the only study.

Dr. Lundberg: No, it's just mine.

Dr. Prystowsky: I certainly respect your research. There have been other studies done, especially the Seattle Heart Watch program, and when they have evaluated people resuscitated from a cardiac arrest, it's a different issue. Eighty percent of those people have coronary disease.

Dr. Lundberg: Right.

Dr. Prystowsky: It depends on how you look at that. I think the big problem for me, right now, is there has been almost a malaise out there trying to find people who we know are at risk for cardiac arrest. I'll take your thesis, George, I think it's a reasonable thing, I would never doubt your data. But let's say, "How about other data?" If we know someone is at risk for sudden death, do we not have an obligation after all the randomized trials to find that person and appropriately try to prevent it? See, that's where I'm at. In the school-age kids, I'm very much for everyone getting at least an EKG. And the adults, if they've had a previous myocardial infarction, or they have congestive heart failure, there are multiple trials now that show that you can prevent a lot of these deaths with an implantable defibrillator. Yet very few people are being approached like that. That's a little different spin. You're talking about a person where you don't know anything, and they die, and you have to figure it out. And I agree, there it's much more of a question. But what about the person that's got defined heart disease to a significant level, we know they're at risk of sudden death, and as a group of doctors in the United States, we're not doing a real good job of preventing it.

Dr. Lundberg: Well, that's where you've worked a lot, and I laud you for the kind of efforts you've put in to this. One final thing, since we talk about selection bias to a large extent as part of this interview, your 80% number in terms of the Seattle study having serious coronary artery disease, presumed serious, in people who suddenly arrest, if those same people had been shot with a gun and autopsied, a large number would have also had coronary artery --

Dr. Prystowsky: They wouldn't lose it because of a gun shot. I'll grant you that.

Dr. Lundberg: Yeah, but again, studies of young American soldiers dead in the Korean War showed that many killed by gunshot also had significant coronary artery disease.

Dr. Prystowsky: [interposing] Plaques in the young soldiers.

Dr. Lundberg: -- about 1950, 1951, showed that coronary disease starts earlier.

Dr. Prystowsky: Yes.

Dr. Lundberg: Well, thank you very much Dr. Prystowsky for being with us today. And thank you all for watching. We've been talking with Eric Prystowsky, a practicing cardiologist at St. Vincent Hospital in Indianapolis and a professor of medicine in cardiology at Duke University in Durham, North Carolina. Signing off from the American Heart Association in Orlando, Florida, I'm Dr. George Lundberg, Editor in Chief of Medscape General Medicine.



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