Hyperpolarized-MRI in Hypertrophic Cardiomyopathy: A Narrative Review.

Abstract

Hypertrophic cardiomyopathy is a genetically inherited cardiac disorder that presents with diverse clinical phenotypes. It is associated with significant adverse outcomes, including arrhythmias and sudden cardiac death. Current gold-standard diagnostic methods include echocardiography and cardiac magnetic resonance imaging. These imaging modalities are the cornerstone in identifying structural abnormalities and aiding risk stratification. However, they fail to capture the preceding cellular and metabolic disturbances that underpin disease progression. Hyperpolarized magnetic resonance imaging (HP-MRI) is an emerging imaging technique that enables non-invasive and non-ionizing visualization of metabolic pathways. HP-MRI enhances the signal of metabolites like [1-¹³C]pyruvate, providing insights into metabolic pathways. Alterations in the metabolic pathways of cardiomyocytes are central to HCM pathophysiology. HP-MRI may be able to delineate the metabolic consequences of sarcomere mutations and distinguish HCM from phenocopies such as glycogen storage disorders or cardiac amyloidosis. More importantly, it has the potential to detect early metabolic shifts and thus play a role in early diagnosis, personalized risk stratification, and monitoring therapeutic response. Although still in experimental stages with technical challenges, HP-MRI has demonstrated considerable potential in preclinical and small-scale studies, exhibiting effectiveness in the diagnosis and monitoring of malignancies across a substantial number of investigations. Further research focusing on larger cohorts and integrating HP-MRI with traditional cardiovascular imaging may pave the way for its clinical use, as well as risk stratification, in HCM.