Giant cell aortitis and Noonan syndrome.
An 18-year-old girl with Noonan syndrome was diagnosed with progressive aneurysmal dilatation of the ascending aorta. Histopathological examination revealed giant cell aortitis. Connective tissue abnormalities leading to aortic root dilatation and the sinuses of Valsalva aneurysm have been reported in Noonan syndrome. This report is the first description of giant cell aortitis in Noonan syndrome and may provide a link between aortic aneurysm, and giant cell granuloma of bone in Noonan syndrome.
Menon
Shaji
S
Mayo Congenital Heart Center, Mayo Clinic, Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Rochester, MN 55905, USA. menon.shaji@mayo.edu
Pierpont
Mary E
ME
Driscoll
David
D
D000293:Adolescent; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D015897:Comorbidity; D005260:Female; D013700:Giant Cell Arteritis / Q000453:epidemiology*; D006801:Humans; D018810:Magnetic Resonance Angiography; D009634:Noonan Syndrome / Q000453:epidemiology* / Q000473:pathology