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Left ventricular function in patients with hypertrophic cardiomyopathy and its relation to myocardial fibrosis and
Dimitrios Maragiannis1, Paulino A Alvarez2, Mohamad G Ghosn2
1Cardiovascular Imaging Section, 401 General Army Hospital of Athens, Athens, Greece.
Insights
In hypertrophic cardiomyopathy (HCM), increased myocardial extracellular volume (ECV) and native T1 relaxation time are linked to diastolic dysfunction. These changes correlate with reduced exercise capacity in HCM patients.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Biomedical Engineering
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart disease characterized by abnormal thickening of the heart muscle.
- Left ventricular (LV) diastolic dysfunction and reduced exercise tolerance are common clinical manifestations in HCM.
- Myocardial extracellular volume (ECV) quantification using cardiac magnetic resonance (CMR) and native T1 relaxation times offer insights into myocardial tissue characteristics.
Purpose of the Study:
- To investigate the relationship between myocardial ECV, LV diastolic function, and exercise tolerance in patients with HCM.
- To assess differences in native T1 relaxation time and ECV between HCM patients and healthy controls.
Main Methods:
- Forty-five HCM patients with preserved ejection fraction underwent CMR and transthoracic echocardiography.
- CMR was utilized to measure LV volumes, mass, ejection fraction, LA volumes, scar burden, native T1, post-contrast T1, and ECV.
- Echocardiography assessed outflow tract gradients, mitral inflow, annular velocities, circumferential strain, and diastolic strain rates. Exercise tolerance was evaluated by exercise duration and peak oxygen consumption.
Main Results:
- HCM patients exhibited significantly increased native T1 relaxation time and ECV compared to controls.
- Both native T1 relaxation time and ECV were significantly associated with LV diastolic dysfunction, circumferential strain, diastolic strain rate, and peak oxygen consumption.
- Higher ECV and native T1 values correlated with worse diastolic function and reduced exercise tolerance.
Conclusions:
- Increased myocardial ECV and native T1 relaxation time are prevalent in HCM patients with preserved ejection fraction.
- These altered myocardial tissue properties are directly related to impaired LV diastolic function.
- The structural changes contribute to diminished exercise capacity in individuals with HCM.
Abstract:
We sought to determine the relation between myocardial extracellular volume (ECV), left ventricular (LV) diastolic function, and exercise tolerance in patients with hypertrophic cardiomyopathy (HCM). Forty five HCM patients with an ejection fraction >50% and no previous septal reduction therapy underwent imaging by CMR and transthoracic echocardiography. CMR was used to quantify LV volumes, mass, EF, LA volumes, scar burden, pre and post contrast T1 relaxation times and ECV. Echocardiography was used to measure outflow tract gradients, mitral inflow and annular velocities, circumferential strain, systolic, early and late diastolic strain rates. Exercise duration and peak oxygen consumption were noted. HCM patients had increased native T1 relaxation time and ECV vs. controls [ECV controls: 24.7 (23.2-26.4) vs. HCM: 26.8 (24.6-31.3)%, P = 0.014]. Both parameters were significantly associated with LV diastolic dysfunction, circumferential strain, diastolic strain rate and peak oxygen consumption (r = -0.73, P < 0.001). Compared to controls, HCM patients have significantly longer native T1 relaxation time and higher ECV. These structural changes lead to worse LV global and segmental diastolic function and in turn reduced exercise tolerance.
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