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Related Concept Videos

Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

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Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
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Heart Failure II: Pathophysiology01:29

Heart Failure II: Pathophysiology

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Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
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Heart Failure I: Introduction01:27

Heart Failure I: Introduction

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Heart failure refers to a clinical syndrome caused by structural or functional cardiac disorders that prevent the heart from pumping an adequate amount of blood to meet the body's metabolic needs. This condition often arises from myocardial infarction or ischemia, leading to decreased cardiac output, reduced tissue perfusion, impaired gas exchange, fluid volume imbalance, and decreased functional ability.Heart failure can result from disruptions in the mechanisms that regulate cardiac output...
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Disorders of the Autonomic Nervous System01:18

Disorders of the Autonomic Nervous System

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The autonomic nervous system (ANS) is an intricate network of nerves that controls functions such as the regulation of heart rate, digestion, and blood pressure regulation. When this system malfunctions, it can lead to various disorders that affect multiple bodily functions. One common feature of many autonomic disorders is the involvement of smooth blood vessels, which play a crucial role in regulating blood flow throughout the body.
Raynaud's disease, also known as Raynaud's...
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Heart Failure IV: Classification and Diagnostic Evaluation01:30

Heart Failure IV: Classification and Diagnostic Evaluation

19
Heart failure can be classified in various ways, with the most common classifications based on physical activity limitations, disease progression, severity, and treatment strategies.The Functional Classification of Heart Failure divides patients into four categories based on physical activity limitation due to symptom burden.Class I: Patients in this class have cardiac disease but no physical activity limitations. Ordinary activities like walking, climbing stairs, or routine tasks do not cause...
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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Quantitative Autonomic Testing
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Autonomic failure: Clinicopathologic, physiologic, and genetic aspects.

David S Younger1

  • 1Department of Clinical Medicine and Neuroscience, CUNY School of Medicine, New York, NY, United States; Department of Medicine, Section of Internal Medicine and Neurology, White Plains Hospital, White Plains, NY, United States.

Handbook of Clinical Neurology
|August 10, 2023
PubMed
Summary

Autonomic failure arises from neurodegenerative, inherited, and autoimmune disorders. Understanding their distinct causes, pathology, and clinical features is crucial for diagnosis and treatment.

Keywords:
Autoimmune autonomic ganglionopathyFamilial dysautonomiaMultiple system atrophyParkinson's diseasePure autonomic failure

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Area of Science:

  • Neuroscience
  • Pathology
  • Clinical Medicine

Background:

  • Autonomic failure is a complex condition with diverse etiologies, including neurodegenerative diseases, inherited disorders, and autoimmune conditions.
  • Understanding the clinicopathologic features of these disorders is essential for accurate diagnosis and effective management.
  • This chapter provides a comprehensive overview of major autonomic disorders.

Approach:

  • Reviewing historical background and etiopathogenesis.
  • Detailing neuropathological features and diagnostic criteria.
  • Discussing current treatment strategies.

Key Points:

  • Multiple System Atrophy (MSA) involves central autonomic failure with glial cytoplasmic inclusions (GCIs).
  • Parkinson's disease and pure autonomic failure exhibit Lewy pathology with distinct autonomic deficits.
  • Hereditary sensory and autonomic neuropathy type III (familial dysautonomia) is a congenital disorder affecting baroreceptor function.
  • Autoimmune autonomic ganglionopathy targets ganglionic nicotinic acetylcholine receptors, causing subacute autonomic failure.

Conclusions:

  • Autonomic disorders present with varied clinical and pathological manifestations.
  • Accurate diagnosis relies on integrating clinical presentation, neuropathology, and genetic or immunological findings.
  • Effective management requires a multidisciplinary approach tailored to the specific underlying cause.