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Hunter syndrome

Hunter's syndrome is a mucopolysaccharide disease caused by an enzyme deficiency of iduronate-2-sulfatase (I2S). This is also called as mucopolysaccharoidosis Type II. It was first described by Scottish physician Charles A. Hunter (1873-1955) in 1917. more...

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Definition

Hunter syndrome is a hereditary disease in which the breakdown of a mucopolysaccharide (a chemical that is widely distributed in the body outside of cells) is defective. This chemical builds up and causes a characteristic facial appearance, abnormal function of multiple organs, and in severe cases, early death.

Causes, incidence, and risk factors

Hunter syndrome is inherited as an X-linked recessive disease. This means that women carry the disease and can pass it on to their sons, but are not themselves affected.

Because girls have two X chromosomes, their normal X can provide a functioning gene even if their other X is defective. But because boys have an X and a Y, there is no normal X gene to fix the problem if the X is defective.

The metabolic abnormality that causes Hunter syndrome is a lack of the enzyme iduronate-2-sulfatase. In its absence, mucopolysaccharides collect in various body tissues, causing damage.

Affected children may develop an early-onset type (severe form) shortly after age 2 that causes a large skull, coarse facial features, profound mental retardation, spasticity, aggressive behavior, joint stiffness and death before age 20. A late-onset type (mild form) causes later and less severe symptoms.

Symptoms

Juvenile form (early-onset, severe form):

  • mental deterioration
  • severe to profound mental retardation
  • aggressive behavior
  • hyperactivity
  • short stature


Late (mild form):

  • mild to no mental retardation

Both forms:

  • coarse facial features
  • large head (macrocephaly)
  • stiffening of joints
  • increased hair (hypertrichosis)
  • deafness (progressive)
  • enlargement of internal organs such as liver and spleen
  • cardiovascular problems, especially valvular dysfunction
  • abnormal retina (back of the eye)
  • carpal tunnel syndrome

Signs and tests

Signs of the disorder that the doctor might look for include:

  • hepatomegaly (enlargement of liver)
  • splenomegaly (enlargement of spleen)
  • inguinal hernia
  • spasticity
  • heart murmur and heart valve dysfunction
  • joint contractures
  • excretion of heparan sulfate and dermatan sulfate in urine
  • decreased iduronate sulfatase enzyme activity in serum or cells

Tests that may indicate this disorder is present include:

  • urine for heparan sulfate and dermatan sulfate
  • enzyme study, decreased iduronosulfate sulfatase (may be studied in serum, white blood cells and fibroblasts)
  • genetic testing may show mutation in the iduronate sulfatase gene

Read more at Wikipedia.org


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Post Magazine: "Broken Heart Syndrome"
From Washingtonpost.com, 5/31/05 by April Witt

Byline: April Witt, Ilan Wittstein and Hunter Champion

In 2004, when Karen Schillings learned that her daughter and soon-to-be son-in-law had died in the water-taxi accident in Baltimore's harbor, her strong heart threatened to stop beating. Fortunately for her, doctors at Johns Hopkins Hospital knew just what to do.

Read the story: How Do You Cure a Broken Heart?

April Witt, whose article about Schillings's experience with the malady known as "broken heart syndrome" appeared in Sunday's Washington Post Magazine, was online Tuesday, May 31, at 1 p.m. ET -- along with Dr. Ilan Wittstein and Dr. Hunter Champion -- to field questions and comments.

April Witt is a Magazine staff writer; Ilan Wittstein and Hunter Champion are cardiologists at Johns Hopkins Hospital in Baltimore.

____________________

April Witt: Good afternoon, everyone. Joining me today are Ilan Wittstein and Hunter Champion, both cardiologists with Johns Hopkins. These two doctors recently co-authored a New England Journal of Medicine article on their research into something they call broken heart syndrome. They have documented that some people who experience extreme emotions - such as grief or terror - suffer a temporary weakening of their heart that mimics a heart attack. The patients in their study had high levels of adrenaline in their bloodstream. The doctors believe that the excess adrenaline may temporarily stun the heart muscle, preventing it from contracting normally. Broken heart syndrome can be fatal. It is likely common. Their medical research strongly reinforces ancient folk wisdom that people can die from grief. So why is it that doctors haven't really begun understanding and documenting this syndrome until relatively recently? We'll let the doctors tell us. Hunter and Ilan, over to you . . .

_______________________

Herndon, Va.: April,

Thanks for an intriguing and very well written story. How did you come upon the idea for this story?

April Witt: The study published in the New England Journal of Medicine generated a lot of initial news stories, in part because it was published just before Valentine's Day. As soon as I read the news stories, I knew I wanted to do a long magazine piece, recreating some of the patients' experiences and trying to lift the veil a bit on the doctors process of discovery. The question I asked myself was what was different about these doctors that they saw what so many others before them missed?

_______________________

Bethesda, Md.: Dr. Wittstein, I had a similar experience not too long ago. Would you be interested in my case? If yes, how can I contact you?

Ilan Wittstein: We are always interested in hearing more about the experiences people have had. This is how we continue to learn more about this syndrome. You can contact me at iwittste@jhmi.edu or by phone at (410) 614-6258. I look forward to hearing from you.

_______________________

Southern Maryland: People who have suffered losses have known about this for centuries -- most likely since time began. Why haven't doctors known about it? Because doctors are trained to steel themselves against losses. If they went to pieces every time they lost a patient, there would be no doctors.

Lay people, however, aren't trained to build a shell around themselves. After my father died, my heart literally ached. There was a dull pressure in my chest for months after, although I was physically normal. I did catch about 6 colds during the following year -- it knocks your immune system to shreds, too. People who suffer a loss go into a deep depression. It's not new.

April Witt: You are raising some of the very questions that first drew me to this article: Why haven't doctors known about it? Doctors in Japan were among the first to document this syndrome, although they called it something different and didn't measure the adrenaline levels. But the fact that they were on to it earlier than western doctors suggests to me that worldview has a role in what doctors discover. The old saying is, "Seeing is believing." I would say, "Believing is seeing." If you are confronted with facts that don't fit your philosophical framework, I'd guess you are more likely to overlook or discount them. From what Ilan and Hunter tell me, doctors have been seeing broken heart syndrome and calling it a heart attack.

_______________________

Arcadia, Calif.: What a story, and so beautifully woven together. Are there instances in which a person is actually killed by the syndrome? And how can you tell, document, or disprove?

Kam

Hunter Champion: Kam,

While all of our patients that have been diagnosed with this syndrome have survived their hospitalization, we have certainly seen some very sick patients that would not have done very well outside the intensive care setting. It is highly likely that it could be fatal if untreated.

Thank you for the question.

Hunter

_______________________

Vienna, Va.: I wonder if several lesser stuns of adrenaline to the heart following a traumatic experience could go unnoticed -- or untreated by a person could accumulate and eventually cause a "true" heart attack?

Hunter Champion: We certain know that chronic stress makes people more susceptible to cardiovascular disease. There is a correlation between the "type A" personality and heart disease in the traditional form (coronary disease). There are adrenaline tumors (called pheochromocytomas) which are rare endocrine tumors that can cause heart failure and also increase blood pressure etc, but they are rare.

Hunter

_______________________

Springfield, Va.: May I just say, April, that your article touched my heart. Although I plan to be a pre-med student this fall, I have a strong emotional side to me and have great interest in heart-felt pieces such as this one. It will definitely be an article I plan to keep with me for the rest of my life as I plan to study medicine and discover more about what life has to offer.

I have a question for Dr. Wittstein. What advice do you have for such aspiring physicians like myself? Your investigation certainly was thought-provoking, original, and fascinating, and will surely lead to more to medical discoveries in the future.

April Witt: Thanks so much for your comment. You sound like you'll make a very caring doctor. I'll let Ilan and Hunter tackle the second part of your note.

_______________________

Oakton, Va.: Have there been any cases of men who had this syndrome?

Ilan Wittstein: It is very clear that this syndrome affects primarily women. In fact, over 95% of the cases we have seen due to emotional stress occur in women. We have seen it in only 2 men due to emotional stress, one who was very angry, and one who was the victim of an assault. So while it can be seen with men, it is far more likely to occur in women.

_______________________

Vienna, Va.: I think that I may have experienced a similar heart attack about five years ago. Would Dr. Wittstein be interested in my case?

Ilan Wittstein: We are interested in hearing about as many experiences as possible. You can contact Dr. Wittstein at iwittste@jhmi.edu. Dr. Champion can be contacted at hcc@jhmi.edu.

_______________________

Fairfax, Va.: Panic attacks cause a rush of adrenaline. Is this damaging to the heart?

Hunter Champion: This is a question that we have been very interested in. Certainly panic attacks can leave the patient breathless and can cause chest pain. This event would also result in a release of adrenaline. The question is if this release would cause a weakening of heart function and if it does, how long does it last. It would be very interesting to do an ultrasound of someone's heart during a panic attack to see. It is not likely that there is any long-term damage associated with it.

Hunter

_______________________

McLean, Va.: Could you explain the physiologic changes/effects that the adrenaline has on the heart? Also, what about the effects of anger and rage on the heart?

Hunter Champion: We think that there could be many effects of the adrenaline on the heart. While we do not think that arterial spasm is very likely, it is possible that this happens at the microscopic level. It is more likely that the adrenaline has a direct "toxic" effect on the heart to "stun" the heart muscle. In small doses, it is a good thing to have adrenaline. It allows for the "fight or flight" response. However, in large doses (or if your heart is "tuned" such that it is more sensitive to the adrenaline, it may prove harmful if only temporarily. I think that rage and anger operate in much the same way to increase adrenaline levels.

Hunter

_______________________

Waldorf, Md.: My son was born with a congenital heart disease; TOF, absent pul. valve, discontinuous lpa. He is 4 now and has been through 3 open heart surgeries. Is he, or a person with a compromised heart, more susceptible to broken heart syndrome?

Hunter Champion: We don't currently know if he would be more likely to have this syndrome. The vast majority of the patients that we have identified with this syndrome have had normally functioning hearts prior to the event. A recent study at a heart rhythm meeting did show that emotional stress (specifically anger) can result in irregular heart rhythm that can cause a defibrillator to fire. At this point in time, we just don't know if your son would be at a higher risk to have this syndrome.

Thank you for the question,

Hunter

_______________________

Springfield, Va.: Dr Wittstein - have you found the "broken heart" phenomenon occurs only soon after a highly emotional event, or can it occur up to months later?

The reason I'm asking is that my grandmother, who was in good health for an 80-year-old woman, suddenly died about 6 weeks after my grandfather

died. They had been married 68 years and our family has always believed that she died of a "broken heart."

Ilan Wittstein: This is a great question. We have known for a long time that people die shortly after the death of a spouse. While this is likely related to what we have written about, there are likely some important differences. In patients with the Broken Heart Syndrome, it is a sudden adrenalin rush that causes temporary stunning of the heart. The stress comes on quickly, the stunning is immediate, and the problem resolves quickly within a few days. In your grandmother's case, there were likely many of the same chemical and hormonal changes due to extreme grief, but in a much slower and more chronic nature. Grief can also cause a blunting of the immune system after a spouse dies. So while the effect of her grief was not exactly the same as what we described, a "broken heart" likely did contribute.

_______________________

College Park, Md.: Can you speculate at all about why some people seem to suffer from broken heart syndrome while others don't? When my mom died, I wanted my heart to stop beating, but it didn't.

Hunter Champion: That is a very important question. We are currently trying to study this syndrome to try to identify who will get it and who won't. We have many more questions than answers at this point. Our research is focusing on the genetics of this to find identify genes that may make you more likely to get it and also to see if this runs in families.

Thank you for the question,

Hunter

_______________________

Potomac, Md.: We are students of psychology at the Bullis School in Potomac. Our teacher read this article to our class and we were so interested that we decided to pursue this subject in our AP class this following school year. We wanted to know how we can stay up to date with any new research and how we might be able to get our hands on your published material. Your proposed methods would be valuable to our learning.

Thanks, Sophie and Zachary

Hunter Champion: Thank you for the question. Please feel free to contact myself or Dr. Wittstein via email. We also have a website www.brokenheartinfo.org that we will be updating with new material as well as have FAQs and new information about our work with the syndrome. There is an email link on the site as well.

Thanks,

Hunter

_______________________

Washington, D.C.: Hello, have you compared the effect of long term grief, like depression, and these sudden surges of grief? If so, are the effects similar?

Hunter Champion: We do know that long term grief, anxiety, or anger can predispose patients to traditional coronary disease. This is different from what we see with this syndrome.

Thanks,

Hunter

_______________________

Falls Church, Va.: So why is it that it's primarily a female syndrome? Does the heart function the same in both males and females or are there differences that can account for why this affects females primarily?

Ilan Wittstein: This is a great question that we do not yet have the answer to. While we can say with certainty that women are primarily affected, we do not yet know the reason why. It may be that the female hormone estrogen is in some way protective against the effects of adrenaline. This may be why women do not seem to get this until they are post-menopausal, a time when estrogen levels are lower. People often ask us if women get this because they are more emotional. I think this is unlikely. Keep in mind that a similar stunning of the heart due to adrenaline can occur following a variety of physical stressors as well, such as stroke, brain injury, and respiratory distress, and this tends to occur primarily in women as well. Figuring out why women are primarily affected will be one of our major goals with our research.

_______________________

Washington, D.C.: What methods do you intend to use for future research? How could someone follow your continued study in depth?

Hunter Champion: Our current methods are database in nature. We are collecting blood and DNA from patients that have had this and looking at it retrospectively. If we are able to get adequate research funds, we hope to be able to do stress testing on our patients to see if they handle adrenaline differently from others. We are in the process of publishing a followup paper to the one that we had in the New England Journal. We would be happy to speak with you at length if you had a particular aspect of our work that you would like to get involved with.

Thanks,

Hunter

_______________________

Waldorf, Md.: What can someone do to prevent this if they feel they are at risk for these types of attacks?

Hunter Champion: We have been treating our patients with beta-blockers to try to counteract the effects of adrenaline on the heart. We have not yet had any recurrences that we know of. I also think that taking a stress management approach may also be of benefit.

Hunter

_______________________

Rockville, Md.: Someone else wrote in with the same symptom that I had after my son died at 17-hours-old: a "heavy" and "aching" heart that seemed to hang in my chest like a dead weight. I was acutely aware of my heart beating at times. I had trouble breathing for a couple of days. Are these symptoms of a broken heart?

Ilan Wittstein: These certainly could have been signs of a broken heart. The symptoms we typically see include shortness of breath, chest pain, irregular heart beats, and sometimes dizziness. The grief you experienced could certainly have resulted in a large surge of adrenaline that may have caused your symptoms. Fortunately, the effects on the heart typically resolve after a few days with no long-term damage. If you are still having symptoms, however, you should be seen by your doctor so that appropriate tests can be done.

_______________________

Washington, D.C.: Dr. Wittstein, has your experience with patients with Broken Heart Syndrome prompted you to take a more holistic approach to treating these patients? For example, do you find that you are more inclined to put them in contact with support groups to help patients through their grief or trauma on a longterm basis? Thank you.

Hunter Champion: I never discourage patients from going through support groups. I think that counseling can be of great benefit to many patients. Our work with this has prompted both myself and Dr. Wittstein to take a more holistic approach in that we think a great deal more about the mind-body connection and not just approach the patient from the standpoint of being a "pill dispenser".

Hunter

_______________________

McLean, Va.: Could you discuss the effect of depression on the heart? I have suffered from depression most of my life. My heart rate is slow, 55-70 range. I have always wondered if my heart rate is "depressed" too. My heart rate rarely gets much faster, even when I exercise. I can get my heart into a max 120-125 range during full tilt exercise. Fast walking up hill will bring my heart up to about 100. I wonder if exercise isn't benefiting my heart because I can't get the rate up into the "cardio" zone. Comments?

Hunter Champion: Just as anxiety and long-standing anger can predispose patients to coronary artery disease, we also know that depression is a risk factor for this disease as well. It is not likely that your slower heart rate is a function of the depression, but I understand your concern about exercise. Exercise has benefits that are not tied to getting into your "cardio zone". The endogenous chemicals that your body releases with exercise can help us sleep better and improve our outlook on life. I would recommend that you keep up the exercise and forget about the heart rate.

Hunter

_______________________

Rockville, Md.: I teach AP Psychology in a private school in Potomac and can't wait to jump into the use of this article, the original study in the New England Journal of Medicine, and the abstract as a teaser. Clearly, these students will find the mind/body connection, possibly a nature/nurture connection, and a marvelous example of primary and secondary sources. We would love to follow the research not just for the practical implications but also for what I am sure will be a paradigm of research method. How could we stay current with your work? Nancy Bluthardt, Bullis School

Ilan Wittstein: We are very pleased to hear that you find this research interesting and that it will be useful to your students. I still remember my AP classes very well. In fact, my AP biology class is one of the reasons I ended up in medicine. You can keep in touch with us by emailing me at iwittste@jhmi.edu and Dr. Champion at hcc@jhmi.edu. You can also check our website (which we will be adding to periodically) at www.brokenheartinfo.org. Thanks again for your interest.

_______________________

Rockville, Md.: Thank you for a wonderful article and confirmation of what I knew was true! A boyfriend of mine once went to the hospital three times before they admitted he had some sort of heart failure -- he was only 32 -- but I knew he was suffering from a broken heart. I'd broken up with him shortly after his estranged wife took the kids and moved across the country. We remained close friends and he made a good recovery (though we eventually parted ways and I recently learned he suffered a fatal heart attack two years ago). Thank you for being brave and curious enough to know that our minds/spirits play into our health as much as our physical bodies.

April Witt: Thanks for your comment.

_______________________

Alexandria, Va.: Doctor Wittstein,

Reading the article in the Sunday Post magazine, I found myself reading of my own symptoms. Four years ago I had what has always been unsatisfyingly labeled a "heart attack" for which I take several heavy duty medicines (statin, ace inhibitor, beta blocker, folic acid, aspirin) daily. But tests indicated no heart damage (though considerable arterial plaque in my 61-year-old male body) and my activities since, including regular contacts sports (hockey), have been unchanged from before the event. My questions are two : first, is it possible that I fit your definition as indicated in the article; second, do you have a program at your center where people like myself can come and obtain a second opinion of their cardiac history and health? Thank you very much for your response.

Regards, Bruce Valley, Alexandria, Va.

Ilan Wittstein: While I cannot be certain without meeting you and reviewing your records, there are a couple things about your history that are atypical for the broken heart syndrome. First, it occurs almost exclusively in women. Second, most of the patients with the broken heart syndrome do not have arterial plaque. In fact, this is one of the unique things about patients with this syndrome. That being said, I would be happy to see you for a second opinion. You can contact my office at (410) 614-6258 or email me at iwittste@jhmi.edu.

_______________________

Alexandria, Va.: I found the article interesting and Dr. Wittstein's research very valuable. I have frequent feelings of adrenaline rushes for no apparent reason. This feeling can last for a short time or over an hour or more.

Is there a way to diagnose that this is what is happening?

Can this be harmful?

Can anything be done about it?

Can it be controlled?

Can drugs like Prozac help?

Where would I go to get information or a diagnosis?

Thank you.

Hunter Champion: The release of adrenaline is a normal event. It comes about from the "fight or flight response". When in small amounts, it is what keeps us motivated to work, gets us going in the morning, and allows us to catch a train. However, when there is an abnormal release (too frequently or in too great of an amount) it can be detrimental.

There is an endocrine tumor called a pheochromocytoma which results in an abnormal release of adrenaline. It is rare, but when present can give patients sudden anxiety for no apparent reason, facial flushing, wildly fluctuating blood pressures, nausea, vomiting etc. If undiagnosed a pheochromocytoma can lead to all the things that high blood pressure can lead to: stroke, heart attack, heart failure etc. If there is a tumor, the cure is surgery. Diagnosis is made by history, plasma adrenaline levels, CT or MRI, or a scan called an MIBG scan that can find this tumor. Many internists or cardiologists can do the workup for this type of tumor.

Thanks,

Hunter

_______________________

Montclair, Va.: Dr. Wittstein, the examples in the article were all of heart weakening due to a single incident of severe emotional stress. In your opinion, could a more regular pattern of emotional stress, resulting in a more chronic pumping of adrenaline, result in a totally ideopathic cardiomyopathy?

Ilan Wittstein: This is a fantastic question. While our research has shown that sudden adrenaline surges can temporarily stun the heart, we do not know whether regular daily adrenaline surges can cause a more chronic cardiomyopathy. The term idiopathic cardiomyopathy means a weakness of the heart muscle due to unexplained causes. It is certainly possible that one cause could potentially be the effects of chronic adrenaline. More research will be needed to answer this question, but it certainly seems plausible.

_______________________

Alexandria, Va.: After a traumatic experience, like the water taxi accident, should the surviving family members go immediately to their doctor for tests, etc. that may detect this problem/condition? I know this is a very difficult time for the surviving family members. However, there may be surviving children involved and they have to be cared for if only one parent survives. That parent must remain healthy to care of the children. Can a medical test detect this problem? Thank you.

Hunter Champion: Great question. Certainly all of the cases that we have seen in this particular paper have come about from the patient feeling bad enough to go to the hospital. The main symptoms have been chest pain an/or shortness of breath. My recommendation would be based on symptoms. If the survivors did not feel bad, then there is probably no need to seek medical attention. However, if they experience chest pain and/or shortness of breath, I would seek medical attention immediately. It is much better to be told that you have had the "broken heart syndrome" and not a true heart attack rather than the alternative.

Thank you.

Hunter

_______________________

Annandale, Va.: My father died on November 16th, 2005, at the age of 74 after a long hospitalization for an aortic aneurysm. My mother, 69, died a little over two weeks later, just two days after his memorial service. He and my mom were very close, having been married for 46 years. She had chronic emphysema, but there was no reason to think she would pass away so quickly. Obviously everyone has had their comment to me about "broken heart syndrome," but your article truly pointed out that this was almost definitely the case. It has obviously been tremendously tough for me (I am an only child at 44) but I was truly amazed that "broken heart syndrome" now seems to truly be the cause of her death. She was found lying on her bed, with the phone in her hand, having been discovered about four or five hours after she passed.

I just had to touch base and say thank you for an incredibly eye-opening article, and I commend you Dr. Wittstein on your defining of this mysterious yet very real syndrome.

Steve Houk

Ilan Wittstein: I am sorry to hear about the loss of both of your parents in such a short period of time. Sadly,we have heard from many people who have reported similar stories. Thank you for sharing your experience with us. Hearing from you, and others, will allow us to better understand the complex relationship between the mind and the heart.

_______________________

Alexandria, Va.: Thank you for a wonderful article. Have you found a link between "broken heart syndrome," mitral valve prolapse and panic attacks? I am 56yo/wf with MVP and developed what was finally diagnosed as panic attacks after several personal crises. Physical and/or mental exhaustion exacerbates the likelihood of experiencing another panic attack, the precursors to which simulate female heart attack symptoms.

Ilan Wittstein: Your question is a very good one because it gets to important question: Who is at risk to get the broken heart syndrome? Certainly the people at greatest risk are women, typically middle age and elderly women who are post-menopausal. We have not seen an association with mitral valve prolapse, but we are seeing an association with people who have depression and/or anxiety. It is possible that people who have an anxiety disorder produce more adrenaline in response to emotional stress, but we do not yet understand the mechanism entirely. It does appear, however, that anxiety/depression may turn out to be a risk factor for this problem. Further research will be necessary and is on-going.

_______________________

Falls Church, Va.: I realize this is a human study that you are conducting on Broken Heart Syndrome and you've had several people write in telling of how one parent passed away shortly after losing a spouse. I've also heard that certain animals also can be afflicted with this. For example, large parrots often bond with a single person (and typically live upwards of 70 years) but if separated from that person the parrot can die of a broken heart. Also, recently there was a report of an elephant in one of the zoos that was separated from its long time companion. The elephant became severely depressed and the zoo decided to rejoin the 2 elephants which solved the problem. Do you believe that animals can be afflicted by this as well?

Ilan Wittstein: I definitely believe this can happen in animals. It is well described that certain animals die suddenly when they are trapped and cannot escape from dangerous situations. This has also been shown experimentally. Subjecting animals to various stressors can cause the same type of changes in the heart that have been described in humans.

_______________________

Washington, D.C.: As a child who recently lost her father to cancer, I read your story with great interest. Dr. Wittstein, has your experience been that Broken Heart Syndrome occurs only once in patients immediately after they experience a sudden trauma or loss, or can it continue to recur when the patient revisits the traumatic events that caused the first attack? Also, now that you are sensitized to this syndrome and can more easily identify patients suffering from it, I wondered whether you take steps to put these patients in contact with grief counselors or a social worker to help them overcome their trauma and avoid a recurrence. Thank you.

Ilan Wittstein: Thank you for this excellent question. One of the things people want to know is whether this syndrome can recur. It is important for people to know that while this syndrome can recur, recurrence is actually fairly uncommon. In the 5 years that I have been following patients with this syndrome, I have only seen 1 patient with recurrence. This patient actually had a severe pain syndrome and the heart stunning occurred with episodes of severe pain. None of the other patients had recurrence of the full-blown syndrome, even when exposed to other forms of stress. Some patients did have recurrence of chest pain with stress, but none had recurrence of heart muscle weakness. Certainly assistance from grief counselors or social workers might be very helpful for some people, but at this point we do not know if this would in any way help to reduce the risk of recurrence. More experience and research with this syndrome will be necessary to better answer this question.

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April Witt: Ilan and Hunter, I have a question for you. Has your research into broken heart syndrome changed how you practice medicine overall? I wonder if it makes you view the mind-body connection dramatically differently than you did just 10 years ago.

Ilan Wittstein: The experience with this syndrome has indeed had an effect on how we practice medicine. We are much more sensitive to the symptoms people describe, and we listen very carefully to the stories people tell. We have learned from our experience with this syndrome that there are many things out there in medicine that have yet to be explained, and that listening carefully and keeping an open mind is critical to being an effective physician.

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Charleston, S.C.: In the article it mentions that post-menopausal women are at the greatest risk for broken heart syndrome, but in your research did you find any cases of men with this condition?

This was a fantastic article, you did a wonderful job of capturing the terror Karen must have felt during the accident and the sorrow felt by all the families after this terrible tragedy. How are the families doing now, are they returning to a semblance of normal life or is the grief still too recent? I hope they are doing better, I'm sure everyone who read this article is touched by their story and will keep them in their hearts.

April Witt: I'm glad you liked the article. I'm sure the families appreciate your good wishes. The grieving parents have told me that while time has somewhat eased their suffering, they don't expect it ever to be erased. Losing a child is a sorrow that lasts a lifetime. Karen and Denny Schillings, for example, both described to me feeling hours or days of relative relief - then being hit with a new wave of grieving when they come across any small reminder of their late daughter, Corinne.

Regarding your question about men experiencing broken heart syndrome, I'll let the doctors weigh in with answers to that.

Ilan Wittstein: We have seen only 2 men with this syndrome due to emotional stress. Over 95% of the cases have been women. If you look through reported cases in the literature, the same pattern is observed...the vast majority are women.

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Rockville, Md.: When I had my first panic attack, I ended up at a hospital thinking I was having a heart attack, like so many people do. Since then, after more panic and anxiety over the years, I've always felt that it is actually adrenaline-out-of-control. It's such a consistent feature that I've wondered if I have a faulty adrenal gland -- it seems more physical than only brain chemistry. What do you think? Is anyone looking into that aspect??? Thanks, Molly

Ilan Wittstein: You ask a very good question here. Adrenal gland pathology can certainly lead to states of adrenaline excess. We have seen one patient with this syndrome who 2 years later was diagnosed with an adrenal tumor, but so far, the vast majority of our patients have had no adrenal disease.

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Arlington, Va.: I was delighted to read the article and to note the research by Dr. Wittstein. I experienced what I think may have been a similar episode on Sept. 22, 2001, immediately after giving a eulogy for a friend who had died on the plane that crashed into the Pentagon. My heart beat wildly; I had chest pain; was admitted to the hospital. The EKG changes were enough to warrant a cardiac cath, which was negative. Subsequent EKGs have been normal.

I was told I had experienced a heart spasm.

I have a history of PAT and thought that that was what was happening. But I have always been able to interrupt the PAT by holding my breath or bearing down. But those maneuvers were not effective.

My questions concern the future. How does one prevent "broken heart syndrome"? Or how does one prepare oneself to cope with future episodes? Does an increased sensitivity to epinephrine or psuedoephredrine increase the likelihood of an attack?

Ilan Wittstein: Thank you for sharing this story with us. We have heard from several people who developed similar symptoms while giving a eulogy, a time when emotions are extremely intense. Fortunately, recurrence has not been a big part of this syndrome. We have seen only 1 patient who had this syndrome more than once. We do not yet know if relaxation or stress management techniques can help prevent this syndrome, or whether medicines that help block the effects of adrenaline such as beta blockers can prevent it either. These will be focused areas of research in the future.

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April Witt: Thanks all for taking the time to read this article on a holiday weekend, and for joining us today. Thank you Ilan and Hunter for graciously agreeing to answer questions.

April Witt: We'll close now. Goodbye.

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Ilan Wittstein: Dr. Champion and I would like to thank you for your thoughtful questions and for taking the time to share your personal experiences with us. We apologize that we were unable to answer every question. If we did not answer your question, please feel free to contact us directly. I can be reached at iwittste@jhmi.edu. Dr. Champion can be reached at hcc@jhmi.edu. We also invite you to check our website at www.brokenheartinfo.org which we will continue to update on a regular basis. We look forward to hearing from you.

Ilan Wittstein

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