Validating an Idiopathic Dilated Cardiomyopathy Diagnosis Using Cardiovascular Magnetic Resonance: The Dilated Cardiomyopathy Precision Medicine Study.
Validating an Idiopathic Dilated Cardiomyopathy Diagnosis Using Cardiovascular Magnetic Resonance: The Dilated Cardiomyopathy Precision Medicine Study.
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DOI：
10.1161/circheartfailure.121.008877
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发表时间：
2022-05
影响因子：
9.7
通讯作者：
Hershberger, Ray E.
中科院分区：
文献类型：
--
作者：
Haas, Garrie J.;Zareba, Karolina M.;Ni, Hanyu;Bello-Pardo, Erika;Huggins, Gordon S.;Hershberger, Ray E.
关键词：
冠状动脉造影是鉴别扩张型心肌病（DCM）病因的基础，包括特发性或缺血性心肌病。心血管磁共振（CMR）的晚期钆增强（LGE）已成为识别心肌瘢痕和确定病因的方法。DCM精准医学研究纳入了经专家临床审查排除缺血性或其他心肌病后，因特发性DCM导致左心室扩张和功能障碍的患者。缺血性心肌病被定义为CAD在≥1条心外膜冠状动脉的血管造影中狭窄>50%。研究入选不需要CMR，但在对可用CMR报告的事后分析中，LGE模式被分类为1）无LGE，2）缺血模式LGE：内膜下/透壁，3）非缺血LGE：中层心肌/心外膜。在评价的1，204例特发性DCM患者中，396例（32.9%）既往接受过CMR研究;其中327例（396例中的82.6%）接受过LGE成像（平均年龄46岁; 53.2%为男性; 55.4%为白色）; 327例患者中的178例（54.4%）显示LGE，178例患者中的156例LGE与特发性DCM一致。其余22例为透壁或内膜下LGE。在这22例患者中，冠状动脉造影正常（13例），显示管腔不规则（3例），远端血栓（1例），CAD伴冠状动脉狭窄<50%（1例），或不可用（4例）。在DCM精准医学研究队列中招募的327名具有LGE-CMR数据的先证者中，22名（6.7%）确定了LGE的缺血模式，所有这些患者均患有专家临床审查裁定的特发性DCM。clinicaltrials.gov，NCT 03037632
Coronary angiography to identify coronary artery disease (CAD) has been foundational to distinguish the etiology of dilated cardiomyopathy (DCM), including the assignment of idiopathic or ischemic cardiomyopathy. Late gadolinium enhancement (LGE) with cardiovascular magnetic resonance (CMR) has emerged as an approach to identify myocardial scar and identify etiology. The DCM Precision Medicine Study included patients with left ventricular dilation and dysfunction attributed to idiopathic DCM, after expert clinical review excluded ischemic or other cardiomyopathies. Ischemic cardiomyopathy was defined as CAD with >50% narrowing at angiography of ≥1 epicardial coronary artery. CMR was not required for study inclusion, but in a post hoc analysis of available CMR reports, patterns of LGE were classified as 1) no LGE, 2) ischemic-pattern LGE: subendocardial/transmural, 3) non-ischemic LGE: midmyocardial/epicardial. Of 1,204 idiopathic DCM patients evaluated, 396 (32.9%) had a prior CMR study; of these, 327 (82.6% of 396) had LGE imaging (mean age 46 years; 53.2% male; 55.4% white); 178 of the 327 (54.4%) exhibited LGE, and 156 of the 178 had LGE consistent with idiopathic DCM. The remaining 22 had transmural or subendocardial LGE. Of these 22, coronary angiography was normal (13), showed luminal irregularities (3), a distant thrombus (1), CAD with <50% coronary artery narrowing (1), or was not available (4). Of 327 probands enrolled in the DCM Precision Medicine study cohort who had LGE-CMR data available, an ischemic-pattern of LGE was identified in 22 (6.7%), all of whom had idiopathic DCM as adjudicated by expert clinical review. clinicaltrials.gov, NCT03037632