Constancy of Spatial Variation in Diffuse Myocardial Disease
Implications for Diagnosing Disease
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See Article by Nordin et al
Variability is the law of life, and as no two faces are the same, so no two bodies are alike, and no two individuals react alike and behave alike under the abnormal conditions which we know as disease.1
—Sir William Osler
Spatially Heterogeneous Myocardial Involvement in Diffuse Diseases
Many ostensibly diffuse disease processes of the heart affect the myocardium variably, where involvement and severity vary by left ventricular location. Such diffuse disease processes that exhibit spatially heterogeneous involvement may include genetic disease (eg, hypertrophic cardiomyopathy), infiltrative disease (eg, amyloidosis), combined infiltrative and genetic disease (Fabry disease [FD]), valvular heart disease (aortic stenosis), and any of the disease processes that promote myocardial fibrosis and remodeling (eg, hypertension). Surprisingly, detailed phenotyping reveals that the severity of myocardial involvement often varies spatially in these diseases that affect the myocardium globally.
Historically, left ventricular hypertrophy manifested by increased myocardial thickness provides a simple, readily detectable parameter to demonstrate variable myocardial involvement. Hypertrophic cardiomyopathy with asymmetrical hypertrophy provides perhaps the best-known exemplar. In this scenario, despite a genetic disease affecting the genotype of each myocardial cell, …