Abstract
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.
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References
Burch M., et al., (1992). Myocardial disarray in Noonan syndrome. Br Heart J 68(6):586–8
Choudhury L., et al., (2002). Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 40(12):2156–64
Wilson J.M., et al., (2002). Magnetic resonance imaging of myocardial fibrosis in hypertrophic cardiomyopathy. Tex Heart Inst J 29(3):176–80
4. Moon J.C., et al., (2004). The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol 43(12):2260–4
Moon J.C., et al., (2003). Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 41(9):1561–7
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Hudsmith, L.E., Petersen, S.E., Francis, J.M. et al. Hypertrophic cardiomyopathy in Noonan Syndrome closely mimics familial hypertrophic cardiomyopathy due to sarcomeric mutations. Int J Cardiovasc Imaging 22, 493–495 (2006). https://doi.org/10.1007/s10554-005-9034-y
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DOI: https://doi.org/10.1007/s10554-005-9034-y