Echocardiometric evaluation of cardiovascular abnormalities in Marfan syndrome

Marfan syndrome is an inherited disorder of connective tissue with manifestations in various organ-systems including cardiovascular system. The aim of this study was to characterize and determine the frequency of cardiovascular alterations by echocardiography in 2 age cohorts of Mexican patients with Marfan syndrome and their comparisons with control groups.

Sixty six with Marfan syndrome and 33 control patients were evaluated by echocardiography. Segments of the aorta and pulmonary artery were measured at different levels, cardiac valves were examined for prolapse and the interatrial septum was assessed for septal aneurysm. Numeric values were corrected for the body surface area and compared with the control group.

Mean significant values between group I (children) and Group II (adults) were as follows: aortic annulus 16.62 +/- 4.57 mm/m2 vs 12.81 +/- 1.95 (p < 0.001), aortic root 23.30 +/- 7.49 mm/m2 vs 18.36 +/- 2.97 (p < 0.001), sinuses of Valsalva 24.14 +/- 7.29 mm/m2 vs 19.84 +/- 3.59 (p < 0.001), ascending aorta 18.43 +/- 5.90 mm/m2 vs 17.02 +/- 4.79 (p < 0.001), aortic arch 16.12 +/- 4.73 mm/m2 vs 14.20 +/- 2.68 (p < 0.001). Pulmonary valve prolapse was seen in 10/22 (45.5%) vs 7/44 (15.9%), p < or = 0.03. Interatrial septal aneurysm was found in 3/22 (13.6%) vs 20/44 (45.5%), p < or = 0.03. There was a significative diference in the presence of atrial septal aneurysm between the adult group and control group (p < 0.001).

The incidence of cardiovascular abnormalities in our series is similar to that in the literature with the exception of the very high incidence of pulmonary valve prolapse vs control groups, then it suggests that the clinical manifestations of MFS are strikingly severe in the Mexican population. Also a high incidence of interatrial septal aneurysm (34.9%) in comparison to control groups (18.2%) was found.