Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease - Evidence for a disease specific abnormality of the myocardial interstitium
Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease - Evidence for a disease specific abnormality of the myocardial interstitium
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DOI：
10.1016/j.ehj.2003.09.017
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发表时间：
2003-12-01
影响因子：
39.3
通讯作者：
Elliott, PM
中科院分区：
文献类型：
--
作者：
Moon, JCC;Sachdev, B;Elliott, PM
安德森-法布里病 (AFD) 是一种 X 连锁鞘脂代谢紊乱，是特发性左心室肥大的一个原因，但肥厚的机制尚不清楚。钆增强心血管磁共振可以检测局灶性心肌纤维化。我们假设 AFD 中存在过度增强。 方法和结果 18 名患有 AFD 的男性（平均 43±14 岁）和 8 名女性杂合子（平均 48±12 岁）接受了电影和晚期钆心血管磁共振检查。九名伴侣（50%）的心肌过度增强范围为总心肌的 3.4% 至 20.6%（平均 7.7+/-5.7%）；在伴侣中，过度增强百分比与左心室质量指数相关（r=0.78，P=0.0002），但与射血分数或左心室容积无关。在四名（50%）杂合女性（平均 4.6%）中也发现了较小的过度增强。在 12 名 (92%) 钆摄取异常的患者中，过度增强发生在基底下外侧瓦特，与心肌梗死不同，它不是心内膜下的。在两名患有严重 LVH（左心室肥厚）和收缩功能障碍的患者中，其他心肌节段也存在额外的过度增强。 结论 这些观察结果表明，AFD 中发生心肌纤维化，并可能导致肥厚和该疾病的自然史。 (C) 2003 年欧洲心脏病学会。由爱思唯尔有限公司出版。保留所有权利。
Aims Anderson-Fabry Disease (AFD), an X-linked disorder of sphingolipid metabolism, is a cause of idiopathic left ventricular hypertrophy but the mechanism of hypertrophy is poorly understood. Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD.Methods and results Eighteen mates (mean 43 14 years) and eight female hetero-zygotes (mean 48 12 years) with AFD underwent cine and late gadolinium cardiovascular magnetic resonance. Nine mate (50%) had myocardial hyperenhancement ranging from 3.4% to 20.6% (mean 7.7+/-5.7%) of total myocardium; in mates, percentage hyperenhancement related to LV mass index (r=0.78, P=0.0002) but not to ejection fraction or left ventricular volumes. Lesser hyperenhancement was also found in four (50%) heterozygous females (mean 4.6%). In 12 (92%) patients with abnormal gadolinium uptake, hyperenhancement occurred in the basal infero-lateral watt where, unlike myocardial infarction, it was not sub-endocardial. In two mate patients with severe LVH (left ventricular hypertrophy) and systolic impairment there was additional hyperenhancement in other myocardial segments.Conclusion These observations suggests that myocardial fibrosis occurs in AFD and may contribute to the hypertrophy and the natural history of the disease. (C) 2003 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.