[Serial clinical and echocardiographic evaluation in children with Marfan syndrome].
OBJECTIVE
To describe the clinical cardiac manifestations and temporal evolution of Marfan syndrome in children; to estimate the incidence of annuloaortic ectasia and mitral valve prolapse; and to evaluate tolerability and efficacy of beta-blockers in these patients.


METHODS
During one year, 21 children with Marfan syndrome underwent serial clinical and echocardiographic examinations. Echocardiograms assessed: the presence of mitral valve prolapse, aortic root diameter, mitral and aortic valves regurgitation, and aortic enlargement during beta-blocker therapy. Eleven patients had two measurements of the aortic root taken one year apart.


RESULTS
The children were asymptomatic throughout the study. Mitral prolapse was found in 11 (52%) children. Annuloaortic ectasia occurred in 16 (76%) patients and found to be mild in 42.8%, moderate in 9.5%, and severe in 23.8%. One of these patients underwent aortic valve replacement and repair of the ascending aorta by the Bentall-De Bono technique, with good results. Heart rate decreased by 13.6% (from 85 to 73 bpm; p < 0.009) with the use of beta-blockers; however, aortic root diameter increased by 1.4 mm/year (p < 0.02). One child could not be given beta-blockers due to bronchial asthma, and no significant side effects were observed in the remaining children, including one who also had bronchial asthma.


CONCLUSIONS
The children remained asymptomatic throughout the study, the use of beta-blockers led to a significant decrease in heart rate, and no significant adverse effects were observed. Contrary to the literature, incidence of annuloaortic ectasia was high among the study population, greater than that of mitral valve prolapse, even during beta-blocker therapy.
Lopez
Victor Manuel Oporto
VM
Universidade Federal de São Paulo, São Paulo, SP. victoroporto666@hotmail.com
Perez
Ana Beatriz Alvarez
AB
Moisés
Valdir Ambrósio
VA
Gomes
Lourdes
L
Pedreira
Patricia da Silveira
Pda S
Silva
Célia C
CC
Campos Filho
Orlando
O
Carvalho
Antônio Carlos C
AC
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D017677:Age Distribution; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate; D006801:Humans; D007223:Infant; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000188:drug therapy; D008945:Mitral Valve Prolapse / Q000000981:diagnostic imaging* / Q000188:drug therapy; D011446:Prospective Studies; D017678:Sex Distribution
D000319:Adrenergic beta-Antagonists