eme physical characteristics. For aortic root diameter 5 cm, elective aortic root replacement is recommended; the operation risk is equal to the risk of spontaneous dissection at this measurement. Optimally the aortic root replacement would take place prior to 5 cm. Beta-blocker therapy has been shown to be effective in slowing the rate of dilation of the aortic root.
The life span is shortened in Marfan patients and most deaths are due to cardiovascular problems. Median survival was 53 years for men and 72 years for women in one study from 1970 to 1990. Lifespan has increased 25% since 1972. Silverman et al. (1995) conducted a study of 417 Marfan syndrome patients, comparing results of life expectancy with data regarding patients who died prior to 1970. Findings were that 47 of the patients died but the mean age at death (41 +/- 18 years) was significantly increased from the mean age at death for 1972 (32 +/- 16 years). It is concluded that this increase in life span may be due to: an overall population life expectancy improvement, cardiovascular surgery benefits, a greater proportion of milder cases because of increased frequency of diagnosis, and medical therapy, including beta-blockers (OMIM, 2002; Silverman et al., 1995).
Clinical Manifestations, Signs, & Symptoms
Marfan, the French pediatrician after which the condition was named, described a five-year-old female child as having: disproportionately long and thin arms, legs, fingers, and toes; poor muscle development; and an abnormally curved spine. The most obvious characteristic is found in the skeletal system, with patients tending to be tall and slim with a long and narrow face. Clinical manifestations are found in the bones and ligaments, the eyes, the heart and blood vessels, the skin, the lungs, the nervous systems, and other systems (OMIM, 2002; Pyeritz & Casner, 1999).
The main signs are found in the skeletal, ocular, and cardiovascular systems. ...