Case-based review of hypertrophic cardiomyopathy (HCM) differential diagnosis by cardiac MR (CMR) with late gadolinium enhancement (LGE)

Congress:

ECR 2020

Poster Number:

C-10058

Type:

Educational Exhibit

Keywords:

Performed at one institution, Observational, Retrospective, Hyperplasia / Hypertrophy, Haemodynamics / Flow dynamics, Congenital, Imaging sequences, Contrast agent-intravenous, Comparative studies, Ultrasound, MR-Diffusion/Perfusion, MR, Contrast agents, Cardiovascular system, Cardiac

Authors:

E. A. Mershina, O. Larina, D. Filatova, R. Myasnikov, M. Kharlap, V. Sinitsyn, S. Moiseev; MOSCOW/RU

DOI:

10.26044/ecr2020/C-10058

DOI-Link:

https://dx.doi.org/10.26044/ecr2020/C-10058

Learning objectives

To demonstrate the possibilities of CMR to clarify the etiology of left ventricle hypertrophy (LVH) (Fig.1). To illustrate the different types of LGE in various forms of HCM and other diseases, accompanied by LVH.

Background

LVH is an increase in myocardial mass of either genetic or secondary origin. Adaptive hypertrophy is usually expressed in changes of heart geometry, myocardial mass and function. It is characterized by an increase in cardiomyocytes volume and concomitant changes in the extracellular matrix. Unlike physiological hypertrophy the pathological tissue properties change: there is an increase in myocardial stiffness, which leads to diastolic dysfunction and an increased risk of heart failure and sudden cardiac death (SCD). Etiology of LVH include primary (HCM, storage diseases) and secondary...

Findings and procedure details

CMR with LGE was performed with 1.5 T systems. Different causes of LVH are illustrated with the representative cases from our database: sarcomeric HCM (n=65), amyloidosis (n=5), Fabry disease (n=15), hypertensive heart disease (n=10), Dannon disease (n=2). 1.HCM is a primary genetically heterogeneous non-inflammatory myocardial lesion, which is manifested by LVH (more than 15 mm) in the absence of objective reasons for this [4]. Cardiomyocyte apoptosis, microvascular ischemia and fibroblast proliferation leading to myocardial fibrosis may be a substrate for the development of diastolic disfunction...

Conclusion

CMR is currently considered as a first-line imaging modality for detecting and assessing of LVH. LGE is recognized as the method of choice for intramyocardial fibrosis evaluation. Different patterns of LGE in patients with LVH could be used as biomarkers for differential diagnosis of underlying diseases.Comparison of CMR data with clinical manifestations of the disease should contribute to selection of the optimal treatment strategy and may improve the patient’s prognosis.

Personal information and conflict of interest

D. Filatova; Moscow/RU - Author at Lomonosov Moscow State University E. Mershina; Moscow/RU - Author at Lomonosov Moscow State University V. E. Sinitsyn; Moscow/RU - Author at Lomonosov Moscow State University O. Larina; Moscow/RU - Author at Lomonosov Moscow State University R. Myasnikov; Moscow/RU - Author at National Medical Research Center for Preventive Medicine M. Kharlap; Moscow/RU - Author at National Medical Research Center for Preventive Medicine S. Moiseev; Moscow/RU - Author at Lomonosov Moscow State University

References

1. Karvonen M, et al. Magnetocardiographic indices of left ventricular hypertrophy. J Hypertens. 2002;20(11):2285-92. 2. Heckbert SR, et al. Traditional cardiovascular risk factors in relation to left ventricular mass, volume, and systolic function by cardiac magnetic resonance imaging: the Multiethnic Study of Atherosclerosis. J Am Coll Cardiol. 2006;22:2285-92. 3. Bluemke DA, et al. The relationship of left ventricular mass and geometry to incident cardiovascular events: the MESA (Multi-Ethnic Study of Atherosclerosis) study. J Am Coll Cardiol. 2008;52(25):2148-55. 4. Maron BJ. Hypertrophic cardiomyopathy: a systematic review....