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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

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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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Disorders of the Autonomic Nervous System01:18

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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.
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Quantitative Autonomic Testing
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Multimodal Autonomic Biomarkers Predict Phenoconversion in Pure Autonomic Failure.

S Koay1,2,3, E Vichayanrat1, F Bremner2,4

  • 1Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, UK.

Annals of Clinical and Translational Neurology
|July 23, 2025
PubMed
Summary
This summary is machine-generated.

Pure autonomic failure (PAF) can progress to multiple system atrophy (MSA) or Lewy body diseases (LBD). Specific autonomic biomarkers like pupillary function and plasma noradrenaline can predict this progression in PAF patients.

Keywords:
Parkinson's diseaseautonomic failureautonomic testingmultiple system atrophyorthostatic hypotensionpure autonomic failure

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Area of Science:

  • Neurology
  • Autonomic Neuroscience
  • Synucleinopathies

Background:

  • Pure autonomic failure (PAF) is characterized by autonomic dysfunction without other neurological signs.
  • A significant proportion of PAF patients develop central neurological features, meeting criteria for multiple system atrophy (MSA) or Lewy body diseases (LBD).
  • Distinguishing between these conditions and predicting progression is crucial for patient management.

Purpose of the Study:

  • To investigate multimodal autonomic biomarkers for differentiating PAF, MSA, and LBD.
  • To identify predictors of phenoconversion from PAF to MSA or LBD.
  • To assess the utility of non-invasive autonomic assessments in predicting disease trajectory.

Main Methods:

  • An observational cohort study involving 391 patients with alpha-synucleinopathy.
  • Comprehensive autonomic assessments including cardiovascular testing, plasma noradrenaline, pupillometry, and questionnaires.
  • Logistic regression modeling to identify predictive autonomic biomarkers for phenoconversion in PAF patients.

Main Results:

  • PAF patients exhibited more severe orthostatic hypotension, lower supine plasma noradrenaline, and sympathetic pupillary deficits compared to MSA and LBD.
  • 26% of PAF patients phenoconverted to MSA or LBD over a median of 13 years.
  • Normal pupillary function, heart rate response ≥ 10 bpm, and supine noradrenaline ≥ 200 pg/mL predicted phenoconversion; younger age and higher noradrenaline predicted MSA conversion.

Conclusions:

  • Normal pupillary function, intact cardiovagal responses, and specific plasma noradrenaline levels are indicators of future phenoconversion in PAF.
  • Younger patients with higher supine plasma noradrenaline levels are more prone to developing MSA.
  • Multimodal autonomic assessment effectively differentiates alpha-synucleinopathies and predicts PAF phenoconversion.