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

Lieke Rozendaal | Zendy; Nico A. Blom | Zendy; Yvonne HilhorstHofstee | Zendy; Arend D.J. ten Harkel | Zendy

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

Author(s) -

Lieke Rozendaal,

Nico A. Blom,

Yvonne HilhorstHofstee,

Arend D.J. ten Harkel

Publication year - 2011

Publication title -

case reports in medicine

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.2

H-Index - 20

eISSN - 1687-9627

pISSN - 1687-9635

DOI - 10.1155/2011/172109

Subject(s) - medicine , arachnodactyly , cardiology , muscle contracture , pulmonary artery , ductus arteriosus , marfan syndrome , surgery

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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