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A 27 year old female with Noonan syndrome and hypertrophic cardiomyopathy underwent cardiovascular magnetic resonance imaging. These images showed asymmetrical septal hypertrophy with maximal left ventricular end-diastolic wall thickness of 25 mm. Following administration of gadolinium, areas of hyperenhancement were seen in the anterior, anteroseptal and lateral walls. This is the first report of focal gadolinium hyperenhancement in hypertrophic cardiomyopathy due to Noonan syndrome and suggests that myocardial fibrosis can be imaged by MR hyperenhancement as seen previously in sarcomeric hypertrophic cardiomyopathy.

Original publication

DOI

10.1007/s10554-005-9034-y

Type

Journal article

Journal

Int J Cardiovasc Imaging

Publication Date

06/2006

Volume

22

Pages

493 - 495

Keywords

Adult, Cardiomyopathy, Hypertrophic, Cardiomyopathy, Hypertrophic, Familial, Contrast Media, Female, Gadolinium DTPA, Humans, Magnetic Resonance Imaging, Mutation, Noonan Syndrome, Sarcomeres