title: [Marfan syndrome in childhood: cardiovascular manifestations. Echocardiographic changes].
abstract: We report the cases of eight children with Marfan syndrome. Seven (87.5%) were diagnosed with cardiopathy, everyone with auscultatory findings. Electrocardiographic patterns were nonspecific. Enlargement of the aortic root was present in two of the cases as seen by thorax x-rays. Echocardiography detected the presence of mitral valve prolapse in 87.5% of the patients (7 cases) and aortic enlargement in 75% of the patients (6 cases). Echocardiography also detected the presence of aortic dysplasia, tricuspid valve prolapse and right and left ventricular hypertrophy. During the follow-up period, no case had cardiac failure. There was no mortality. The aortic enlargement was progressive and was not modified by propranolol treatment. Surgical treatment was not needed. Family history related to this condition was present in a very small percentage (37.5%). We comment on one infantile form of Marfan syndrome with its own phenotype different from that of classical Marfan syndrome.
authors.affiliation: Sección de Cardiología Infantil, Hospital Doce de Octubre, Madrid.
authors.forename: A J
authors.initials: AJ
authors.lastname: Carbajo Ferreira
authors.forename: A
authors.initials: A
authors.lastname: Pérez Martínez
authors.forename: F
authors.initials: F
authors.lastname: Gutiérrez Larraya
authors.forename: J L
authors.initials: JL
authors.lastname: Barrionuevo Porras
authors.forename: A
authors.initials: A
authors.lastname: Jabón Serradilla
authors.forename: G
authors.initials: G
authors.lastname: Merino Batres
mesh_terms: D002318:Cardiovascular Diseases / Q000175:diagnosis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging*
vernacular_title: Síndrome de Marfan en la infancia: clínica cardiovascular. Alteraciones ecocardiográficas.