Abstract
ObjectivesTo quantify resting myocardial blood flow (MBF) in the left ventricular (LV) wall of HCM patients and to determine the relationship to important parameters of disease: LV wall thickness, late gadolinium enhancement (LGE), T2-signal abnormalities (dark and bright signal), LV outflow tract obstruction and age.Materials and MethodsSeventy patients with proven HCM underwent cardiac MRI. Absolute and relative resting MBF were calculated from cardiac perfusion MRI by using the Fermi function model. The relationship between relative MBF and LV wall thickness, T2-signal abnormalities (T2 dark and T2 bright signal), LGE, age and LV outflow gradient as determined by echocardiography was determined using simple and multiple linear regression analysis. Categories of reduced and elevated perfusion in relation to non- or mildly affected reference segments were defined, and T2-signal characteristics and extent as well as pattern of LGE were examined. Statistical testing included linear and logistic regression analysis, unpaired t-test, odds ratios, and Fisher’s exact test.Results804 segments in 70 patients were included in the analysis. In a simple linear regression model LV wall thickness (p<0.001), extent of LGE (p<0.001), presence of edema, defined as focal T2 bright signal (p<0.001), T2 dark signal (p<0.001) and age (p = 0.032) correlated inversely with relative resting MBF. The LV outflow gradient did not show any effect on resting perfusion (p = 0.901). Multiple linear regression analysis revealed that LGE (p<0.001), edema (p = 0.026) and T2 dark signal (p = 0.019) were independent predictors of relative resting MBF. Segments with reduced resting perfusion demonstrated different LGE patterns compared to segments with elevated resting perfusion.ConclusionIn HCM resting MBF is significantly reduced depending on LV wall thickness, extent of LGE, focal T2 signal abnormalities and age. Furthermore, different patterns of perfusion in HCM patients have been defined, which may represent different stages of disease.
Highlights
Hypertrophic cardiomyopathy (HCM) is a complex and relatively common genetic disorder with a prevalence of 0.2%
Multiple linear regression analysis revealed that late gadolinium enhancement (LGE) (p,0.001), edema (p = 0.026) and T2 dark signal (p = 0.019) were independent predictors of relative resting myocardial blood flow (MBF)
In HCM resting MBF is significantly reduced depending on left ventricular (LV) wall thickness, extent of LGE, focal T2 signal abnormalities and age
Summary
Hypertrophic cardiomyopathy (HCM) is a complex and relatively common genetic disorder with a prevalence of 0.2%. It is characterized by left ventricular (LV) hypertrophy in the absence of any other cardiac or systemic disease. Myocardial perfusion abnormalities are common in HCM patients and seem important for pathophysiology and prognosis [10]. They may be related to abnormal intramyocardial coronary arteries [11], inadequate capillary density in relation to increased myocardial mass or impairment of LV relaxation and may lead to myocardial ischemia and scarring. A higher degree of microvascular dysfunction has been shown to be an independent predictor for worse prognosis and death in HCM patients [16]
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