Current treatment and mortality rates in Marfan syndrome - adapted from the Journal of Cardiology, J

Date: May 15, 1995

Marfan syndrome is an autosomal dominant inherited disorder of the connective tissue. The lifespan of patients with the disorder is shortened, with most mortality resulting from cardiovascular disease. Recently, treatment has been more aggressive, including surgery for aortic and mitral regurgitation, aortic aneurysm and aortic dissection. Beta-adrenergic receptor antagonists can also delay aortic widening. Silverman and associates assessed the impact of such treatment on survival rates.

Records were reviewed for 417 patients with a definite diagnosis of Marfan syndrome who were seen at a variety of medical centers after 1970. Diagnosis was made by the presence of typical musculoskeletal, cardiovascular and ocular system evidence, as defined in the Berlin nosology.

By the time of the study, 47 deaths had occurred; cause of death was known for 42 (89 percent) of the patients. The mean age at death was 41 years. This age is significantly higher than the mean age at death reported in a 1972 retrospective study (32 years). One hundred twelve patients had at least one cardiovascular operation, and 191 patients were known to have received beta-adrenergic receptor blocking agents.

The authors conclude that the median survival for patients with Marfan syndrome has increased significantly since the early 1970s. Although the historical increase in mean life expectancy is a contributing factor, death rates from an forms of cardiovascular disease have declined significantly. Survival after cardiovascular surgery is prolonged, and an operative procedure should be recommended in patients with marked aortic enlargement or progressive valvular regurgitation resulting in diminished left ventricular function. These data also imply that the use of beta blockers decreases the rate of aortic dilatation and is beneficial. (Journal of Cardiology, January 15,1995, vol. 75, p. 157.)

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