The Role Of Hypertrophic Cardiomyopathy The Disease - amazonia.fiocruz.br

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The guideline encourages shared decision-making between the clinician and patient as essential when determining treatment course and updates recommendations for sudden cardiac death SCD risk assessment and HCM center referrals based on the latest evidence. HCM is a disease that causes the heart muscle to become abnormally thick, which can make it harder for the heart to pump enough blood to the body. Many patients with HCM are asymptomatic, but those who do have symptoms suffer from fainting, chest pain, shortness of breath or heart palpitations. The guideline updates the previous version, which was issued in , to offer recommendations on the evaluation and management of patients with HCM and is geared to cardiovascular clinicians as well as non-cardiovascular clinicians. People with HCM may have additional medical conditions such as heart failure, atrial fibrillation and ventricular arrythmias, which means treatment decisions are not always clear cut. While the guideline writers recognize that patients with HCM can be evaluated and treated by a cardiovascular care team, recommendations are made for patients with severe HCM, or those facing complex decisions, to be referred to multidisciplinary HCM centers to receive optimal care. The Role Of Hypertrophic Cardiomyopathy The Disease.

The Role Of Hypertrophic Cardiomyopathy The Disease Video

Hypertrophic cardiomyopathy - signs and symptoms, causes, pathophysiology, treatment

The highly organized transverse T-tubule membrane system represents the ultrastructural substrate for excitation—contraction coupling in ventricular myocytes.

Introduction

While the architecture and function of T-tubules have been well described in animal models, there is limited morpho-functional data on T-tubules in human myocardium. Hypertrophic cardiomyopathy HCM is a primary disease of the heart muscle, characterized by different clinical presentations at the various stages of its progression.

Moreover, by comparing pathological changes observed in myectomy samples with those introduced by acute experimentally induced detubulation, we discuss the role of T-tubular disruption as a part of the complex excitation—contraction coupling remodeling process that occurs during disease progression. Lastly, we highlight how T-tubule morpho-functional changes may be related to patient genotype and we discuss the possibility of a primitive remodeling of the T-tubule system in rare HCM forms associated with genes coding for proteins implicated in T-tubule structural integrity, formation and maintenance.

T-tubules are transverse and deep invaginations of the surface sarcolemma running along the Z-line regions in mammalian ventricular myocytes. A spatial and geometrical rearrangement of the residual T-tubular system with a greater proportion of tubules running in the longitudinal and oblique directions, and an increase in the mean T-tubular diameter were also observed. Interestingly, in animal models of secondary hypertrophy as well as of physiological hypertrophy, hand in hand with increased cell dimensions, T-tubule proliferation and density increase have been described.

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While the architecture and function of T-tubules have been well described on animal model hearts, T-tubule morpho-functional data on human cardiac samples are scarce. In particular, some human data are available on Heart Failure HF and atrial fibrillation, but little is known on human primary and secondary left ventricular LV hypertrophy, including hypertrophic cardiomyopathy HCM during the non-failing stage of the disease.

HCM is the most prevalent primary disorder of the cardiac muscle, with a prevalence of 1 in worldwide Maron and Maron It is characterized by asymmetric LV hypertrophy, unexplained by increased loading conditions or other systemic diseases. Despite the huge heterogeneity of clinical manifestations, most patients maintained a compensated hypertrophic disease stage Olivotto et al.

Rarely the disease evolves into HF, i.

The pathophysiology of HCM relies on the close interplay between the primary effects of the gene mutation, directly causing a dysfunction of the myofilaments, and the secondary maladaptive E—C coupling changes.]