Hypertrophic Cardiomyopathy 2020
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HEART FAILURE (HJ EISEN, SECTION EDITOR)
Hypertrophic Cardiomyopathy 2020 James Kogut 1 & Eric D. Popjes 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review To briefly review the pathophysiology and natural history of hypertrophic cardiomyopathy (HCM) and to describe the diagnosis, assessment, and contemporary management strategies. Recent Findings HCM-related mortality remains low; however, symptoms due in large part to LVOT obstruction remain a clinical dilemma. Several medical therapies have been shown to reduce symptoms and improve functional capacity, including several recent phase 2 clinical trials involving the novel myosin modulator mavacamten. In patients with refractory symptoms, septal reduction therapy or advanced therapies remain viable options in many cases. Summary HCM is a complex and heterogeneous disease with diverse presentations and variable anatomy and clinical outcomes. The majority of patients will remain asymptomatic or with minimal symptoms and long-term survival remains high. For symptomatic patients, a variety of medical therapies, along with septal reduction therapies, have been shown to reduce symptoms and improve functional capacity. Keywords Clinical heterogeneity . Left ventricular hypertrophy . Left ventricular outflow obstruction . Sudden cardiac death . Septal reduction therapy . Inherited cardiomyopathy
Introduction Hypertrophic cardiomyopathy (HCM) is a heterogeneous and complex disease. It affects people of all ages and ethnic groups and does so in different ways and to various degrees of severity. Its range of phenotypic expression is wide and its natural history can be tragic in some and benign in many others. While some may suffer sudden cardiac death (SCD) from ventricular arrhythmias, others may live a normal life with few symptoms or complications [1•]. It has been recognized as the most common cause of SCD in young and athletic people, but with increased knowledge about and recognition of HCM, it is clear that the risk of SCD is not as high as previously thought. HCM also appears to be far more common than previously thought, affecting approximately 1 in This article is part of the Topical Collection on Heart Failure * Eric D. Popjes [email protected] James Kogut [email protected] 1
PennState Health Milton S. Hershey Medical Center, 500 University Drive, Hershey, PA 17033, USA
500 individuals or even more by some estimates, making it the most common inheritable cardiac genetic disease [2]. HCM is defined as the presence of a hypertrophied, nondilated left ventricle (LV) in the absence of a cardiac or systemic condition that is known to cause the degree of hypertrophy seen. The hypertrophy is usually asymmetric and most commonly and prominently involves the intra-ventricular septum. Histologically, there is myocyte hypertrophy and disarray, interstitial fibrosis, and microvascular pathology and dysfunction. It is a genetically determined disease that involves genetic variations in sarcomeric p
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