People with Marfan syndrome are at up to 250 times greater risk of aortic dissection (a tear or rupture between layers of the aortic wall) than the general population. That’s why it’s important to know the signs of an aortic dissection and what to do.
Knowing that you have Marfan syndrome puts you at an advantage in the event that you experience unexplained chest, back, or abdominal pain. A person who does not know they have the disorder may not be treated with the same urgency as those who know they are at increased risk of aortic dissection.
Although aortic dissection is uncommon, it is not rare. When it is does occur, it is commonly catastrophic. Based on available medical information, there are probably 5,000-10,000 dissections a year in the United States. However, the number may be underreported since dissections that do not result in death or cardiovascular surgery may be recorded under a different diagnosis, such as chest or abdominal pain. The number of dissections may be also underreported because so few autopsies are conducted, and many deaths are attributed to "heart attack" or "sudden death," when the cause is actually a dissection.
Since emergency departments may be very busy and knowledge about Marfan syndrome may vary greatly, it is possible that you may need to advocate for yourself should you experience these symptoms. Do not be shy about telling the doctors and nurses at the hospital what you know about your condition and the information about aortic dissection diagnosis treatment.
Symptoms of aortic dissection
The pain may be severe, sharp, or tearing, and it may travel from the chest to the back and/or stomach. Sometimes, the pain is less severe, but a person still has a feeling that “something is very wrong.” If a dissection is suspected, a person needs to go to a hospital emergency department right away.
What you can do
Unless someone has a known diagnosis of Marfan syndrome or very obvious physical characteristics that would indicate Marfan syndrome or a related condition, reports of chest pain often do not automatically raise the possibility of aortic dissection in the emergency room.
Therefore, be sure that you are prepared to:
- Advocate for yourself by telling emergency department staff that you have Marfan syndrome or a related condition.
- Communicate effectively with doctors and nurses in the emergency department.
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