Hypertrophic cardiomyopathy in Noonan Syndrome closely mimics familial hypertrophic cardiomyopathy due to sarcomeric mutations
Hypertrophic cardiomyopathy in Noonan Syndrome closely mimics familial hypertrophic cardiomyopathy due to sarcomeric mutations
复制标题
DOI：
10.1007/s10554-005-9034-y
复制
发表时间：
2006-06-01
影响因子：
2.1
通讯作者：
Neubauer, S
中科院分区：
文献类型：
--
作者：
Hudsmith, LE;Petersen, SE;Neubauer, S
一位27岁女性，患有努南综合征和肥厚性心肌病，接受了心血管磁共振成像。这些图像显示不对称的室间隔肥厚，最大左室舒张末期壁厚25mm。注射钆后，在前壁、前隔膜和侧壁可见高强化区。这是Noonan综合征引起的肥厚性心肌病的第一个局灶性钆高增强的报告，表明心肌纤维化可以通过MR高增强成像，就像以前在肌瘤性肥厚性心肌病中看到的那样。
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.