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Published on: June 14, 2016
Microvascular Dysfunction in Hypertrophic Cardiomyopathy
Francesco Pelliccia1, Franco Cecchi2, Iacopo Olivotto3
1Department of Cardiovascular Sciences, Sapienza University, 00166 Rome, Italy.
Insights
Myocardial ischemia, driven by coronary microvascular dysfunction (CMD), is key in hypertrophic cardiomyopathy (HCM). Early non-invasive assessment of CMD is crucial for managing heart failure and sudden cardiac death risk in HCM patients.
Area of Science:
- Cardiology
- Cardiovascular Pathophysiology
Background:
- Myocardial ischemia is a significant feature of hypertrophic cardiomyopathy (HCM), influencing heart failure (HF) and sudden cardiac death (SCD).
- Coronary microvascular dysfunction (CMD), without epicardial coronary artery disease, is the primary cause of ischemia in HCM.
- Assessing microcirculation non-invasively is challenging but vital for understanding HCM pathophysiology.
Purpose of the Study:
- To highlight the importance of assessing myocardial ischemia and CMD in hypertrophic cardiomyopathy.
- To emphasize the role of non-invasive multimodality imaging in evaluating microvascular dysfunction.
- To underscore the necessity of early CMD diagnosis for risk stratification and patient management in HCM.
Main Methods:
- Utilizing a multimodality approach for detailed assessment of microvascular dysfunction and ischemia.
- Employing non-invasive diagnostic tools including transthoracic Doppler echocardiography, positron emission tomography, and cardiac magnetic resonance.
- Integrating diagnostic work-up for myocardial ischemia into the routine management of HCM patients.
Main Results:
- Non-invasive assessment of CMD using advanced imaging techniques is feasible and recommended for all HCM patients.
- Myocardial ischemia contributes to cardiac fibrosis, left ventricular remodeling, heart failure, and arrhythmias in HCM.
- Comprehensive evaluation of ischemia aids in the risk stratification for sudden cardiac death in HCM.
Conclusions:
- Non-invasive assessment of coronary microvascular dysfunction should be a standard part of HCM evaluation.
- Early diagnosis and management of myocardial ischemia are critical for improving outcomes in HCM patients.
- Addressing CMD is a key challenge for improving the prognosis of hypertrophic cardiomyopathy.
Abstract:
Myocardial ischemia is an established pathophysiological feature of hypertrophic cardiomyopathy (HCM) that impacts various clinical features, including heart failure (HF) and sudden cardiac death (SCD). The major determinant of myocardial ischemia in HCM is coronary microvascular dysfunction (CMD) in the absence of epicardial coronary artery abnormalities. Despite the impossibility to directly visualize microcirculation in vivo, a multimodality approach can allow a detailed assessment of microvascular dysfunction and ischemia. Accordingly, the non-invasive assessment of CMD using transthoracic Doppler echocardiography, positron emission tomography, and cardiac magnetic resonance should now be considered mandatory in any HCM patient. Noteworthy, a complete diagnostic work-up for myocardial ischemia plays a major role in the approach of the patients with HCM and their risk stratification. Chronic and recurrent episodes of ischemia can contribute to fibrosis, culminating in LV remodeling and HF. Ischemia can potentially constitute an arrhythmic substrate and might prove to have an added value in risk stratification for SCD. Accordingly, strategies for the early diagnosis of CMD should now be considered an important challenge for the scientific community.
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