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Old 05-22-2011
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Question Most common cause of death in Marfan's Syndrome

Hi Friends

Goljan said the most common cause of sudden death in Marfan's syndrome is due to mitral valve prolapse... in other sources it's aortic dissection (which makes sense to me).

any thoughts? what should I answer if I get this kind of question!!
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Old 05-22-2011
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"Aortic disease is the main cause of death among patients with Marfan syndrome. "

ref:
Nature Reviews Cardiology 7, 266-276 (May 2010)
http://www.nature.com/nrcardio/journ...o.2010.31.html

"Individuals with Marfan syndrome often present to forensic facilities because of the risk of early and sudden death, most often due to aortic dissection with haemothorax and haemopericardium, with cardiac tamponade and/or coronary artery compromise. Other cardiovascular causes of premature demise include aneurysm rupture, mitral valve prolapse , cardiac failure and arrhythmias and endocarditis associated with valve replacement. Fatal or near-fatal events due to these conditions may be precipitated by exercise, trauma, stimulant usage and pregnancy, and may occur at very early ages including infancy.5 Deaths may also result from atlanto-occipital instability or from post-operative complications following cardiovascular surgery. Lethal mechanisms associated with Marfan syndrome are discussed in greater detail elsewhere."

REF:
Marfan syndrome and sudden death within a family – Aetiologic, molecular and diagnostic issues at autopsy
Journal of Forensic and Legal Medicine
Volume 15, Issue 4, May 2008, Pages 205-209
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Old 05-25-2011
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Just to Clarify,

Dr. Goljan just say, If you found a Marfan Patient who got SUDDEN DEATH is not to Aortic Dissection, is due to Mitral Valve Prolapse... Aortic Dissection is painful...

So bottom line,

- Aortic Dissection is the common Cause of "Death" on Marfan Patient...

- Mitral Valve Prolapse is the common Cause of Sudden death to Marfan, due a defect of abnormality conduction.

In my opinion be aware of the same structural question relate to Enhles danlos, because is common to see mitral valve prolapse on enhles danlos also... but not the dissection ( just to clarify )
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Old 05-25-2011
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Good point Rulz!
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Old 05-25-2011
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"Aortic rupture or dissection is the most common cause of premature death among persons with Marfan syndrome," said Duke E. Cameron, MD, Professor of Surgery, James T. Dresher Sr. Professor, Director of Paediatric Cardiac Surgery, and Co-Director of The Dana and Albert "Cubby" Broccoli Centre for Aortic Diseases, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.
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Old 05-25-2011
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Quote:
Originally Posted by rulz View Post
Just to Clarify,

Dr. Goljan just say, If you found a Marfan Patient who got SUDDEN DEATH is not to Aortic Dissection, is due to Mitral Valve Prolapse... Aortic Dissection is painful...

So bottom line,

- Aortic Dissection is the common Cause of "Death" on Marfan Patient...

- Mitral Valve Prolapse is the common Cause of Sudden death to Marfan, due a defect of abnormality conduction.

In my opinion be aware of the same structural question relate to Enhles danlos, because is common to see mitral valve prolapse on enhles danlos also... but not the dissection ( just to clarify )
Curr Sports Med Rep. 2002 Apr;1(2):67-74.
Marfan syndrome: screening for sudden death in athletes.
Glorioso J Jr, Reeves M.

"Marfan syndrome is a common, preventable cause of sudden cardiac death in the athlete. It is an autosomal-dominant disorder of connective tissue with variable penetration that affects multiple organ systems. Aortic root aneurysm rupture or dissection is the most common cause of sudden death. A directed family and personal history, in addition to a search for characteristic physical stigmata, can optimize the screening of athletes during the preparticipation evaluation. Athletes who have pertinent findings on the preparticipation evaluation should undergo further diagnostic evaluation. Echocardiography is essential to rule out cardiovascular involvement in those suspected of having Marfan syndrome, and should be mandated when positive pertinent family or personal history is elicited or when cardiac abnormalities are detected. Fortunately, due to characteristic historic and clinical findings, Marfan syndrome can be detected early, allowing appropriate treatment and ultimately prevention of sudden death in affected athletes."
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Old 05-25-2011
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i dont think so,, maybe is beyond of us right now, but Sudden Death should not be relate to aortic dissection because it should be PAINFUL and a death which is painful is not sudden ... at least you gotta show sign of pain...

Lets face it... just think it.

For me is going to stay as that... Thanks for the "quotation" anyways...
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Old 05-25-2011
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Quote:
Originally Posted by rulz View Post
i dont think so,, maybe is beyond of us right now, but Sudden Death should not be relate to aortic dissection because it should be PAINFUL and a death which is painful is not sudden ... at least you gotta show sign of pain...

Lets face it... just think it.

For me is going to stay as that... Thanks for the "quotation" anyways...

"Sudden cardiac death (SCD) is an unexpected death due to cardiac causes occurring in a short time period (generally within 1 h of symptom onset) in a person with known or unknown cardiac disease"...
ref:
http://emedicine.medscape.com/

other:
"The Framingham definition of "sudden death" is a death that occurs within one hour of the onset of symptoms, and this definition is the most commonly used..."

pain or without pain...that's not the point here
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nice discussion!!!
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Old 05-25-2011
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I can see that it's also confusing for you guys
thank you all for the great explanation
I'll go for most common cause of death : dissection
most common cause of sudden death: mitral prolapse

and I'm crossing y fingers lol
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Old 05-25-2011
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Hey Everyone, Great Discussion.

Just wanted to point out something I found on UptoDate:

It has been estimated that the overall risk of serious complications in MVP is about 1 percent per year in patients with clinically and echocardiographically diagnosed disease; this is a not a cumulative risk since some patients have multiple complications

If a question comes up about which is more common cause of death, I would go with aortic dissection, both sudden or otherwise. But I highly doubt they will ask a question like that.

If they do ask a question, something in the history would surely help us differentiate between the two.

Severe Chest Pain + Diminished Pulses = Aortic Dissection
Palpitations, etc = Arrhythmia caused by MVP
Sudden onset of dyspnea, signs of Mitral Regurgitation = Chordae tendinae rupture caused by MVP
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Old 04-29-2012
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Question Most common cause of death in Marfan Syndrome?

mcc of death in marfans?
in audio goljan says it is arrythmia due to mvp in book it is aortic dissection
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Old 04-29-2012
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Quote:
Originally Posted by tyagee View Post
mcc of death in marfans?
in audio goljan says it is arrythmia due to mvp in book it is aortic dissection
i would go dissection
although i don't hv third source to pint out
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Old 04-29-2012
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Default take a look..

take a look at the discussion here:

http://forums.studentdoctor.net/arch.../t-513050.html
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Old 04-29-2012
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As i know mvp is more common in marfan syndrome compared to aortic dissection.
i think that sudden death in marfan syndrome will be due to mvp - arrythmia, but overall most common couse of death will be aortic problems i.e. dissection.
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Old 04-29-2012
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I thought the MCC of death in Marfan's was the rupture of a dissecting aortic aneurysm.
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Quote:
Originally Posted by Hope2Pass View Post
I thought the MCC of death in Marfan's was the rupture of a dissecting aortic aneurysm.
Yes it is. but the most common cause of sudden death in marfan patient in MVP.
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Old 10-17-2012
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Quote:
Originally Posted by rulz View Post
Just to Clarify,

Dr. Goljan just say, If you found a Marfan Patient who got SUDDEN DEATH is not to Aortic Dissection, is due to Mitral Valve Prolapse... Aortic Dissection is painful...

So bottom line,

- Aortic Dissection is the common Cause of "Death" on Marfan Patient...

- Mitral Valve Prolapse is the common Cause of Sudden death to Marfan, due a defect of abnormality conduction.

In my opinion be aware of the same structural question relate to Enhles danlos, because is common to see mitral valve prolapse on enhles danlos also... but not the dissection ( just to clarify )
You are correct, but you can also see aortic dissection in Ehlers-Danlos. Aortic dissection can also occur in Turner Syndrome and Syphilis.
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