Sharka Ilir, Panichella Giorgia, Grigoratos Chrysanthos, Muca Matilda, De Gori Carmelo, Keilberg Petra, Novani Giovanni, Barra Valerio, Hlavata Hana, Bianchi Matteo, Zai Denisa Simona, Frijia Francesca, Clemente Alberto, Todiere Giancarlo, Barison Andrea
Department of Cardiology, University Hospital "Mother Teresa", 1005 Tirana, Albania.
Department of Experimental and Clinical Medicine, University of Florence, 50121 Florence, Italy.
Medicina (Kaunas). 2025 May 10;61(5):875. doi: 10.3390/medicina61050875.
: Nonischemic cardiomyopathies comprise a wide spectrum of heart muscle disorders characterized by different morphological, functional, and tissue abnormalities. Cardiovascular magnetic resonance (CMR) represents the gold standard imaging modality for assessing cardiac morphology, systolic function, and tissue characterization, thereby aiding in early diagnosis, precise phenotyping, and tailored treatment. The aim of this review is to provide an up-to-date overview of CMR techniques for studying myocardial perfusion and their applications to nonischemic cardiomyopathy, not only to rule out an underlying ischemic aetiology but also to investigate the pathophysiological characteristics of microcirculatory dysfunction in these patients. : We performed a structured review of the literature focusing on first-pass gadolinium perfusion sequences, stress protocols, and emerging pixel-wise perfusion mapping approaches. Studies were selected to illustrate the methods for image acquisition, post-processing, and quantification of myocardial blood flow (MBF) and myocardial perfusion reserve (MPR), as well as to highlight associations with clinical endpoints. : First-pass CMR perfusion imaging reliably detects diffuse and regional microvascular dysfunction across cardiomyopathies. Semi-quantitative parameters (e.g., upslope, MPRI) and quantitative MBF mapping (mL/g/min) have demonstrated that impaired perfusion correlates with disease severity, extent of fibrosis, and adverse outcomes, including heart failure hospitalization, arrhythmias, and mortality. Novel automated pixel-wise mapping enhances reproducibility and diagnostic accuracy, distinguishing coronary microvascular dysfunction from balanced three-vessel disease. Microvascular dysfunction-present in approximately 50-60% of dilated cardiomyopathy (DCM), 40-80% of hypertrophic cardiomyopathy (HCM), and >95% of cardiac amyloidosis (CA) patients-has emerged as a key driver of adverse outcomes. Perfusion defects appear early, often preceding overt hypertrophy or fibrosis, and provide incremental prognostic value beyond conventional CMR metrics. : CMR represents a powerful tool for detecting myocardial perfusion abnormalities in nonischemic cardiomyopathies, improving phenotyping, risk stratification, and personalized management. Further standardization of quantitative perfusion techniques will facilitate broader clinical adoption.
非缺血性心肌病包括一系列广泛的心肌疾病,其特征为不同的形态、功能和组织异常。心血管磁共振成像(CMR)是评估心脏形态、收缩功能和组织特征的金标准成像方式,有助于早期诊断、精确分型和个性化治疗。本综述的目的是提供CMR技术用于研究心肌灌注及其在非缺血性心肌病中的应用的最新概述,不仅用于排除潜在的缺血病因,还用于研究这些患者微循环功能障碍的病理生理特征。
我们对文献进行了结构化综述,重点关注首过钆灌注序列、负荷方案和新兴的逐像素灌注映射方法。选择这些研究以说明心肌血流(MBF)和心肌灌注储备(MPR)的图像采集、后处理和定量方法,以及突出与临床终点的关联。
首过CMR灌注成像能够可靠地检测各种心肌病中的弥漫性和区域性微血管功能障碍。半定量参数(如上升斜率、MPRI)和定量MBF映射(mL/g/min)已表明灌注受损与疾病严重程度、纤维化程度以及不良结局相关,包括心力衰竭住院、心律失常和死亡率。新型自动逐像素映射提高了可重复性和诊断准确性,可区分冠状动脉微血管功能障碍与三支血管均衡病变。微血管功能障碍在大约50%-60%的扩张型心肌病(DCM)、40%-80%的肥厚型心肌病(HCM)和>95%的心脏淀粉样变性(CA)患者中出现,已成为不良结局的关键驱动因素。灌注缺损出现较早,通常先于明显的肥厚或纤维化,并提供超越传统CMR指标的额外预后价值。
CMR是检测非缺血性心肌病中心肌灌注异常、改善分型、风险分层和个性化管理的有力工具。定量灌注技术的进一步标准化将促进其在临床上更广泛的应用。