Liu Zhan-Qiu, Maforo Nyasha G, Magrath Patrick, Prosper Ashley, Renella Pierangelo, Halnon Nancy, Wu Holden H, Ennis Daniel B
Department of Radiology, Stanford University, Palo Alto, CA 94305, USA.
Cardiovascular Institute, Stanford University, Palo Alto, CA 94305, USA.
Diagnostics (Basel). 2024 Nov 27;14(23):2673. doi: 10.3390/diagnostics14232673.
In boys with Duchenne muscular dystrophy (DMD), cardiomyopathy has become the primary cause of death. Although both positive late gadolinium enhancement (LGE) and reduced left ventricular ejection fraction (LVEF) are late findings in a DMD cohort, LV end-systolic circumferential strain at middle wall (E) serves as a biomarker for detecting early impairment in cardiac function associated with DMD. However, E derived from cine Displacement Encoding with Stimulated Echoes (DENSE) has not been quantified in boys with DMD. We aim to: (1) use cine DENSE to quantify regional E in LGE negative (-) boys with DMD and healthy controls; and (2) compare E with LVEF in terms of differentiating DMD boys with LGE (-) from healthy boys. 10 LGE (-) boys with DMD and 12 healthy boys were enrolled prospectively in an IRB-approved study for CMR at 3T. Navigator-gated cine DENSE was used to obtain short-axis mid-ventricular data and estimate global and regional E. Group-wise differences were tested via a Wilcoxon rank-sum test. Within-group differences were tested via a Skillings-Mack test followed by pairwise Wilcoxon signed-rank tests. A binomial logistic regression model was adopted to differentiate between DMD boys with LGE (-) and healthy boys. When compared to healthy boys, LGE (-) boys with DMD demonstrated significantly impaired septal E [-0.13 (0.01) vs. -0.16 (0.03), = 0.019]. In comparison to the E in other segments, both groups of boys exhibited significantly reduced septal E and significantly elevated lateral E. Septal E outperformed LVEF in distinguishing DMD boys with LGE (-) from healthy boys. Reduced septal E may serve as an early indicator of cardiac involvement in LGE (-) DMD boys prior to reduced LVEF and a positive LGE finding.
在患有杜氏肌营养不良症（DMD）的男孩中，心肌病已成为主要死因。尽管钆延迟增强（LGE）阳性和左心室射血分数（LVEF）降低在DMD队列中都是晚期表现，但室壁中部的左心室收缩末期圆周应变（E）可作为检测与DMD相关的心脏功能早期损害的生物标志物。然而，在患有DMD的男孩中，尚未对基于电影激发回波位移编码（DENSE）的E进行量化。我们的目的是：（1）使用电影DENSE对LGE阴性（-）的DMD男孩和健康对照者的局部E进行量化；（2）在区分LGE（-）的DMD男孩和健康男孩方面，将E与LVEF进行比较。10名LGE（-）的DMD男孩和12名健康男孩前瞻性地纳入了一项经机构审查委员会（IRB）批准的3T心脏磁共振成像（CMR）研究。使用导航门控电影DENSE获取短轴心室中部数据，并估计整体和局部E。通过Wilcoxon秩和检验进行组间差异检验。组内差异通过Skillings-Mack检验，随后进行成对Wilcoxon符号秩检验。采用二项式逻辑回归模型区分LGE（-）的DMD男孩和健康男孩。与健康男孩相比，LGE（-）的DMD男孩表现出明显受损的间隔E [-0.13（0.01）对-0.16（0.03），P = 0.019]。与其他节段的E相比，两组男孩的间隔E均显著降低，侧壁E显著升高。在区分LGE（-）的DMD男孩和健康男孩方面，间隔E优于LVEF。间隔E降低可能是LGE（-）的DMD男孩在LVEF降低和LGE阳性发现之前心脏受累的早期指标。
