JACC:扩张型心肌病患者基因环境相互作用的预后意义

2015-09-15 崔倩 译 MedSci原创

多因素发病导致扩张性心肌病(DCM),使得分层困难。最近MOGE(S)(形态功能,器官受累,遗传性或家族性,病因,分类阶段)解决了这个问题。本研究的目的是调查DCM患者MOGE(S)分类的适用性和预后相关性。本研究的参与者来自于荷兰的马斯特里赫特心肌病登记患者,并排除了缺血性,心脏瓣膜,高血压,和先天性心脏疾病的患者。所有其他患者进行了全面诊断工作,包括遗传评估和心内膜心肌活检。本研究共包括213

多因素发病导致扩张性心肌病(DCM),这使得DCM分层困难。最近MOGE(S)(形态功能、器官受累、遗传性或家族性、病因、分类阶段)解决了这个问题。本研究的目的是调查DCM患者MOGE(S)分类的适用性和预后相关性。

本研究的参与者来自于荷兰的马斯特里赫特心肌病登记患者,这些患者排除了缺血性、心脏瓣膜、高血压、和先天性心脏疾病。所有患者进行了全面诊断工作,包括遗传评估和心内膜心肌活检。

本研究共包括213例连续性的DCM患者:35(16%)例患者表现出器官受累,70(33%)例患者具有遗传性或家族性DCM,其中16(8%)例患者有致病突变。155(73%)例至少找到1个因素的患者中,48(23%)例有多于1个的可能因素。左心室逆转重塑在非遗传或非家族性DCM的患者中比遗传或家族性DCM患者中常见(40% vs 25%,P=0.04)。在平均随访47个月后,器官受累和更高的纽约心脏病协会功能分级以及不良结果相关(分别为P<0.001和p=0.02)。遗传性或家族性DCM本身没有预后意义,但当它伴随着额外的致病环境因素如重大病毒载量、免疫介导的因子、节律紊乱、或有毒的触发因素,就会导致一个更坏的结果(p=0.03)。较高的MOGE(S)存在属性(≥2 vs ≤1属性)表现出不良预后(P=0.007)。

该MOGE(S)分类在DCM中是适用的,并且每个属性或基因与环境的相互作用均与结果有关。重要的是,多个属性的存在是不利的结果的预测因子。最后,研究建议对涉及多个可能的病因的MOGE(S)进行调整。

原始出处:

Mark R. Hazebroek, Suzanne Moors, Robert Dennert, et al.Prognostic Relevance of Gene-Environment Interactions in Patients With Dilated Cardiomyopathy,JACC,2015.9.15

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    2015-09-24 lsj637
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    2016-03-03 hbwxf
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    2015-09-17 ylz8405
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