Vascular abnormalities, including reduced myocardial perfusion reserve, are common in hypertrophic cardiomyopathy and correlate with structural remodeling and adverse outcomes across 130 studies.
Systematic Review (n=130)
What are the modalities for assessing vascular function in HCM and how do vascular changes relate to disease progression and clinical outcomes?
Vascular abnormalities, particularly reduced myocardial perfusion reserve, are common in hypertrophic cardiomyopathy and are closely linked to disease progression, structural remodeling, and adverse clinical outcomes.
AIMS: Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy along with (micro)vascular abnormalities. These include cardiac and peripheral vascular dysfunction, as well as structural vascular changes. This review summarizes the modalities for assessing (micro)vascular function and explores how vascular changes relate to disease progression, including structural remodeling, fibrosis, and clinical outcomes. METHODS AND RESULTS: A systematic search of multiple databases was conducted to identify studies investigating vascular changes in patients with HCM, varying in disease severity. Human studies were included without time restrictions and studies involving hypertrophy secondary to other conditions (e.g., hypertension, aortic stenosis) were excluded.A total of 130 studies were included. Vascular changes were assessed with Positron Emission Tomography (n = 24), Cardiac Magnetic Resonance Imaging (n = 21), angiography (n = 18), scintigraphy and Single Photon Emission Computed Tomography (n = 30), peripheral measures (n = 12), and histology (n = 29). Perfusion abnormalities were common in HCM. While resting myocardial blood flow appears to be similar between HCM patients and controls, myocardial perfusion reserve is consistently reduced, correlating with early disease features, structural remodeling, and adverse outcomes. Peripheral vascular dysfunction is also frequently observed, though findings vary and mechanisms remain unclear. CONCLUSION: Vascular abnormalities play a key role in the pathophysiology of HCM, and are closely linked to disease progression and prognosis. Data are limited regarding the role of vascular dysfunction in genotype-positive/phenotype-negative individuals and individuals with early or mild disease. Future research should focus on evaluating cardiac and peripheral vascular function across the disease spectrum to better understand underlying mechanisms and potential targets for intervention.
Visch et al. (Tue,) conducted a systematic review in Hypertrophic cardiomyopathy (n=130). Vascular abnormalities vs. Controls was evaluated on Vascular changes and their relation to disease progression, structural remodeling, fibrosis, and clinical outcomes. Vascular abnormalities, including reduced myocardial perfusion reserve, are common in hypertrophic cardiomyopathy and correlate with structural remodeling and adverse outcomes across 130 studies.