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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| Format: | Article |
| Language: | English |
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Wiley
2011-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2011/172109 |
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| author | L. Rozendaal N. A. Blom Y. Hilhorst-Hofstee A. D. J. Ten Harkel |
| author_facet | L. Rozendaal N. A. Blom Y. Hilhorst-Hofstee A. D. J. Ten Harkel |
| author_sort | L. Rozendaal |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-38f300a6ad4a4db69159ca14b555f906 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2011-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-38f300a6ad4a4db69159ca14b555f9062025-02-03T01:32:08ZengWileyCase Reports in Medicine1687-96271687-96352011-01-01201110.1155/2011/172109172109Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal DefectL. Rozendaal0N. A. Blom1Y. Hilhorst-Hofstee2A. D. J. Ten Harkel3Department of Pediatric Cardiology, Willem Alexander Children and Youth Center, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The NetherlandsDepartment of Pediatric Cardiology, Willem Alexander Children and Youth Center, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The NetherlandsDepartment of Clinical Genetics, Leiden University Medical Center, 2300 RC Leiden, The NetherlandsDepartment of Pediatric Cardiology, Willem Alexander Children and Youth Center, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The NetherlandsWe 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.http://dx.doi.org/10.1155/2011/172109 |
| spellingShingle | L. Rozendaal N. A. Blom Y. Hilhorst-Hofstee A. D. J. Ten Harkel Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect Case Reports in Medicine |
| 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 |
| url | http://dx.doi.org/10.1155/2011/172109 |
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