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...

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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
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author Rozendaal, L.
Blom, N. A.
Hilhorst-Hofstee, Y.
Ten Harkel, A. D. J.
author_facet Rozendaal, L.
Blom, N. A.
Hilhorst-Hofstee, Y.
Ten Harkel, A. D. J.
author_sort Rozendaal, L.
collection PubMed
description 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.
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spelling pubmed-31379712011-07-20 Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect Rozendaal, L. Blom, N. A. Hilhorst-Hofstee, Y. Ten Harkel, A. D. J. Case Rep Med Case Report 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. Hindawi Publishing Corporation 2011 2011-07-12 /pmc/articles/PMC3137971/ /pubmed/21776272 http://dx.doi.org/10.1155/2011/172109 Text en Copyright © 2011 L. Rozendaal et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Rozendaal, L.
Blom, N. A.
Hilhorst-Hofstee, Y.
Ten Harkel, A. D. J.
Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title_full Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title_fullStr Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title_full_unstemmed Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title_short Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect
title_sort dilatation of the great arteries in an infant with marfan syndrome and ventricular septal defect
topic Case Report
url 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
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AT tenharkeladj dilatationofthegreatarteriesinaninfantwithmarfansyndromeandventricularseptaldefect

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