The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance

Journal of Cardiovascular Magnetic Resonance

Review

Carolyn Ho¹  Marc K Halushka²  Theodore P Abraham³  Daniel P Judge³  Radwa A Noureldin⁴  Marcelo S Nacif⁴  Songtao Liu⁵  David A Bluemke⁵ 

[1] Cardiovascular Division, Brigham and Women's Hospital, Boston, MA, USA;Department of Pathology, Johns Hopkins University, Baltimore, MD, USA;Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA;Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, MD, USA;Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, MD, USA;Molecular Biomedical Imaging Laboratory, National Institute of Biomedical Imaging and Bioengineering, Bethesda, MD, USA;

关键词: Right Ventricular;    Cardiac Magnetic Resonance;    Left Ventricular Hypertrophy;    Late Gadolinium Enhancement;    Myocardial Fibrosis;   

DOI  :  10.1186/1532-429X-14-17

 received in 2011-06-14, accepted in 2012-02-20,  发布年份 2012

来源: Springer

【 摘 要 】

Hypertrophic cardiomyopathy (HCM) is the most common genetic disease of the heart. HCM is characterized by a wide range of clinical expression, ranging from asymptomatic mutation carriers to sudden cardiac death as the first manifestation of the disease. Over 1000 mutations have been identified, classically in genes encoding sarcomeric proteins. Noninvasive imaging is central to the diagnosis of HCM and cardiovascular magnetic resonance (CMR) is increasingly used to characterize morphologic, functional and tissue abnormalities associated with HCM. The purpose of this review is to provide an overview of the clinical, pathological and imaging features relevant to understanding the diagnosis of HCM. The early and overt phenotypic expression of disease that may be identified by CMR is reviewed. Diastolic dysfunction may be an early marker of the disease, present in mutation carriers prior to the development of left ventricular hypertrophy (LVH). Late gadolinium enhancement by CMR is present in approximately 60% of HCM patients with LVH and may provide novel information regarding risk stratification in HCM. It is likely that integrating genetic advances with enhanced phenotypic characterization of HCM with novel CMR techniques will importantly improve our understanding of this complex disease.

【 授权许可】

Unknown   
© Noureldin et al; licensee BioMed Central Ltd. 2012. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.