Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect

We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After sur...

Descripción completa

Detalles Bibliográficos
Autores principales: Rozendaal, L., Blom, N. A., Hilhorst-Hofstee, Y., Ten Harkel, A. D. J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2011
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3137971/
https://www.ncbi.nlm.nih.gov/pubmed/21776272
http://dx.doi.org/10.1155/2011/172109
Descripción
Sumario:We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

Ejemplares similares