Living With Marfans

EMERGENCY ALERT INFORMATION

PATIENTS WITH MARFAN SYNDROME  OR A RELATED DISORDER ARE AT 250 TIMES GREATER RISK OF AORTIC DISSECTION THAN THE GENERAL POPULATION.

Symptoms of aortic dissection can be variable, relatively minor, and nonspecific. CHEST PAIN IS THE MOST COMMON SYMPTOM, BUT PAIN CAN ALSO OCCUR IN THE BACK AND/OR ABDOMEN.  The pain maybe described as SEVERE OR VAGUE, CONSTANT OR INTERMITTENT, MIGRATORY, TEARING, TIGHTNESS OR FULLNESS.  Other signs and symptoms can include CARDIOVASCULAR INSTABILITY, PULSENESS, PARATHESIAE, PARALYSIS, SYNCOPE OR A SENSE THAT SOMETHING IS TERRIBLY WRONG.

The most definitive tests for Aortic Dissection are:

CT SCAN - TRANSESOPHAGEAL ECHOCARDIOGRAM - MRI

Choose the one that is most readily available, and expertly performed and interpreted.

Keep in mind that a normal x-ray does NOT rule out the possibility of Aortic Dissection.

50% OF PATIENTS WITH UNDIAGNOSED AORTIC DISSECTION DIE WITHIN 48 HOURS.

PLEASE DO NOT discount aortic dissection until it has been definitively ruled out.

---The above information is provided as part of the Marfan Foundation's Emergency Medical  Campaign  and  Dr. Hal Dietz, Chair NMF Professional Advisory Board, John Hopkins Hospital ---

(You can receive an Emergency Alert Card  by contacting the Marfan Foundation at www.marfan.org )

PLEASE NOTE: There are a lot of people who do not know they have Marfans (or possible one of the many similar conditions such as Loeys-Dietz Syndrome, MASS Phenotype, Ehlers Danlos Syndrome, Marfan Hypermobility Syndrome, etc. to name a few) and it is important for all ER staff to be alert to the symptoms listed above. It it also important to be aware that not all Marfan syndrome patients have the classic body type of tall, thin, etc. There are many patients who have been diagnosed with Marfans that are not tall and/or thin.
- Jon R. -