综合心脏磁共振 T1、T2 和细胞外容积成像用于定义杜氏肌营养不良症的心肌病变。
Comprehensive cardiac magnetic resonance T1, T2, and extracellular volume mapping to define Duchenne cardiomyopathy.
机构信息
Thomas P. Graham Jr Division of Pediatric Cardiology, Department of Pediatrics, Vanderbilt University Medical Center, Monroe Carell Jr Children's Hospital at Vanderbilt, 2220 Children's Way, Suite 5230, TN, 37232, Nashville, USA.
Department of Biostatistics, Vanderbilt University Medical Center, Nashville, TN, USA.
出版信息
J Cardiovasc Magn Reson. 2023 Jul 31;25(1):44. doi: 10.1186/s12968-023-00951-y.
BACKGROUND
Cardiomyopathy is the leading cause of death in Duchenne muscular dystrophy (DMD). Cardiac magnetic resonance (CMR) parametric mapping sequences offer insights into disease pathophysiology. We propose a novel approach by leveraging T2 mapping in conjunction with T1 and extracellular volume (ECV) mapping to perform a virtual myocardial biopsy. While previous work has attempted to describe myocardial changes in DMD, our inclusion of T2 mapping enables comprehensive categorization of myocardial tissue characteristics of fibrosis, edema, and fat to better understand the pathological composition of the myocardium with disease progression.
METHODS
DMD patients (n = 49; median: 12 years-old) underwent CMR, including T1, T2, and ECV. Categories were defined as normal, isolated high T1 (normal ECV, high T1, normal T2), fibrosis (high ECV, normal or high T1, normal T2), edema (normal or high ECV, normal or high T1, high T2), fat (normal ECV, low T1, high T2) or fibrofatty (high ECV, low T1, high T2).
RESULTS
Median left ventricular ejection fraction (LVEF) was 59% with 27% having LVEF < 55%. Those with normal LVEF and no late gadolinium enhancement (37%) were younger in age (10.5 ± 2.6 vs. 15.0 ± 4.3 years-old, p < 0.001). Native T1 was elevated in at least one slice in 82% of patients. Those with high T2 at any slice (27%) were older (p = 0.005) and had lower LVEF (p = 0.005) compared with subjects with normal T2 (73%). The most common myocardial characterization was fibrosis (43%) followed by isolated high T1 (24%). Of the 13 with high T2, ten were categorized as edema, two as fibrofatty, and one as fat.
CONCLUSION
CMR parametric mapping sequences offer insights into Duchenne cardiomyopathy pathophysiology, which should drive development of therapeutic interventions aimed at these targets. Myocardial fibrosis is common in DMD. Patients with elevated T2 were older and had lower LVEF. Though fat infiltration was present, the majority of subjects with elevated T2 met criteria for myocardial edema.
背景
扩张型心肌病是杜氏肌营养不良症（DMD）患者的主要死亡原因。心脏磁共振（CMR）参数映射序列可深入了解疾病的病理生理学。我们提出了一种新方法，利用 T2 映射结合 T1 和细胞外容积（ECV）映射来进行虚拟心肌活检。虽然之前的研究已经尝试描述 DMD 中的心肌变化，但我们纳入 T2 映射可以更全面地对纤维化、水肿和脂肪等心肌组织特征进行分类，从而更好地了解随着疾病进展心肌的病理组成。
方法
49 名 DMD 患者（中位年龄：12 岁）接受 CMR 检查，包括 T1、T2 和 ECV。分类标准为正常、孤立性 T1 升高（正常 ECV、T1 升高、T2 正常）、纤维化（高 ECV、T1 正常或升高、T2 正常）、水肿（正常或高 ECV、T1 正常或升高、T2 升高）、脂肪（正常 ECV、T1 降低、T2 升高）或纤维脂肪（高 ECV、T1 降低、T2 升高）。
结果
左心室射血分数（LVEF）中位数为 59%，其中 27%的患者 LVEF＜55%。那些 LVEF 正常且无晚期钆增强（37%）的患者年龄较小（10.5±2.6 岁与 15.0±4.3 岁，p＜0.001）。82%的患者至少有一个层面的 T1 升高。任何层面 T2 升高的患者（27%）年龄较大（p=0.005），LVEF 较低（p=0.005），与 T2 正常（73%）的患者相比。最常见的心肌特征是纤维化（43%），其次是孤立性 T1 升高（24%）。在 13 名 T2 升高的患者中，10 名归类为水肿，2 名归类为纤维脂肪，1 名归类为脂肪。
结论
CMR 参数映射序列可深入了解杜氏肌营养不良症心肌病的病理生理学，这应推动针对这些靶点的治疗干预措施的发展。心肌纤维化在 DMD 中很常见。T2 升高的患者年龄较大，LVEF 较低。尽管存在脂肪浸润，但大多数 T2 升高的患者符合心肌水肿的标准。
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