Contact:
Eileen Masciale
Consulting Director of Communications
631-665-2163
publicity@marfan.org


MARFAN SYNDROME AND AORTIC DISSECTION
Facts & Figures

• Approximately 200,000 people in the United States have Marfan syndrome or a related disorder. It is estimated that half of those with Marfan syndrome are not diagnosed. 
• People with Marfan syndrome are frequently taller than other members of their family and are often nearsighted. Typically, they have long arms, legs, fingers and toes, and are loose-jointed. In most cases, the aorta, the large artery that carries blood away from the heart, is weakened and prone to enlargement.
• With an early diagnosis and proper medical treatment, people with Marfan syndrome and related disorders can live a normal life span.
• Nearly 10,000 people in the U.S. suffer from an aortic dissection each year. However, experts believe this estimate is low because aortic dissection is not a reportable condition. In addition, dissections that do not result in death or cardiovascular surgery may be recorded under a different diagnosis, such as chest or abdominal pain.
• According to a study in the New England Journal of Medicine (April 29, 1999), elective — rather than emergent — aortic surgery is critical for the survival of Marfan syndrome patients. Among the study’s key findings is that elective aortic root replacement has a low operative mortality (1.5% among the 455 patients in the study). In contrast, emergent aortic repair has a mortality rate eight times that of elective root replacement (12% vs. 1.5%).
• Other groups at high risk for aortic dissection are people who have known enlargement of the aorta (familial aortic aneurysm), bicuspid aortic valves, Loeys-Dietz syndrome, Turner syndrome, Ehlers-Danlos Type IV, or family history of aortic disease. High blood pressure can also lead to aortic dissection.