Evidence for Marfan cardiomyopathy
Evidence for Marfan cardiomyopathy
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DOI：
10.1093/eurjhf/hfq127
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发表时间：
2010-10-01
影响因子：
18.2
通讯作者：
Mohiaddin, Raad H.
中科院分区：
文献类型：
--
作者：
Alpendurada, Francisco;Wong, Joyce;Mohiaddin, Raad H.
马凡综合征（MFS）是一种遗传性结缔组织疾病，经常累及心血管系统。由于瓣膜反流是一种常见的并发症，因此心脏可能会受到影响。然而，是否存在原发性心肌病仍存在争议。我们的目的是使用心血管磁共振评估马凡相关心肌病的存在。我们回顾性评估了 68 名连续的未进行心血管手术或显着瓣膜反流的成年患者。估计左心室和右心室体积、射血分数和质量，并与健康对照人群的已发表数据进行比较。还评估了患者的心力衰竭、主动脉尺寸和瓣膜疾病。四分之一 (25.0%) 的马凡患者左心室射血分数 (LVEF) 降低，其中 25.0% 的左心室舒张末期增加，30.8% 的左心室收缩末期容量增加。右心室射血分数降低 10.3%，右心室舒张末期容积增加 11.8%，收缩末期容积增加 13.2%。在单变量分析中，未发现 LVEF 降低与年龄、性别、主动脉指数、二尖瓣脱垂或瓣膜反流之间存在关联。这项研究支持马凡患者亚组中存在原发性心肌病。双心室扩大和功能障碍通常是轻微的、无症状的，且独立于其他心血管表现。需要进一步的研究来评估这种情况的根本原因和自然史。 MFS 的常规监测和治疗可能需要进行调整，不仅要防止主动脉根部扩张，还要支持心肌功能。
Marfan syndrome (MFS) is an inherited connective tissue disease which frequently involves the cardiovascular system. The heart can be affected since valvular regurgitation is a common complication. However, there is still debate whether a primary cardiomyopathy exists. Our aim was to evaluate the existence of a Marfan-related cardiomyopathy using cardiovascular magnetic resonance.We retrospectively evaluated 68 consecutive adult patients with no cardiovascular surgery or significant valvular regurgitation. Left ventricular and right ventricular volumes, ejection fraction, and mass were estimated and compared with published data on a healthy control population. Patients were also assessed for heart failure, aortic dimensions, and valve disease.One quarter (25.0%) of Marfan patients had reduced left ventricular ejection fraction (LVEF), with 25.0% having increased left ventricular end-diastolic and 30.8% having increased end-systolic volumes. The right ventricular ejection fraction was reduced in 10.3%, with increased right ventricular end-diastolic volumes in 11.8% and increased end-systolic volumes in 13.2%. On univariate analysis, no association was found between reduced LVEF and age, gender, indexed aortic dimensions, presence of mitral valve prolapse, or valve regurgitation.This study supports the existence of a primary cardiomyopathy in a subgroup of Marfan patients. The biventricular enlargement and dysfunction is usually mild, asymptomatic, and independent from other cardiovascular manifestations. Further studies are needed to assess underlying causes and natural history of this condition. Routine monitoring and treatment in MFS may need to be tailored not only to prevent aortic root expansion but also to support myocardial function.