The clinical course and echocardiographic features of Marfan's syndrome in childhood
T. Geva, J. Hegesh and M. Frand
Department of Pediatrics C, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
The clinical and echocardiographic manifestations in 25 patients with
Marfan's syndrome diagnosed during infancy and childhood (mean [+/- SD]
age, 8.1 +/- 4.8 years; range 0 to 16 years) were evaluated. Twenty-one
patients (84%) had a midsystolic click, 11 patients (44%) had mitral
regurgitation (MR), and five patients (20%) had combined MR and aortic
regurgitation (AR). Echocardiography demonstrated mitral valve prolapse in
all 25 patients, aortic root dilatation in 20 patients (80%), AR in seven
patients (28%), and aortic aneurysm in five patients (20%). During the
follow-up period (mean, 5 +/- 4.5 years), progressive AR and aortic
aneurysm were documented in four patients, progressive MR in three
patients, and progressive aortic root dilatation in two patients. Five
patients (22%) died during the follow-up period. Among patients with a
positive family history of Marfan's syndrome, MR was less frequent as
compared with sporadic cases (29.4% vs 75%, respectively). Progressive
cardiovascular involvement was more frequent among patients diagnosed
before 10 years of age compared with those diagnosed later (60% vs 12.5%,
respectively). Cardiovascular involvement was a common feature of childhood
Marfan's syndrome, causing significant morbidity and mortality. Sporadic
cases and children diagnosed before 10 years of age represented a
particularly high-risk group.
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