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.
