Changes in cardiac sympathetic nerve innervation and activity in pathophysiologic transition from typical to

Hidenobu Terai1, Masami Shimizu, Hidekazu Ino

  • 1Molecular Genetics of Cardiovascular Disorders, Division of Cardiovascular Medicine, Kanazawa University, Kanazawa, Japan. yonken1@med.kanazawa-u.ac.jp

Insights

Cardiac sympathetic nerve activity in hypertrophic cardiomyopathy (HCM) worsens with disease progression. Iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy effectively evaluates these pathophysiologic changes in HCM patients.

Area of Science:

  • Cardiology
  • Nuclear Medicine
  • Biomedical Imaging

Background:

  • Left ventricular (LV) systolic function in hypertrophic cardiomyopathy (HCM) is typically normal but can decline late in the disease.
  • Cardiac sympathetic nerve activity abnormalities in HCM are known but their progression is unclear.
  • Iodine-123 metaiodobenzylguanidine (123I-MIBG) myocardial scintigraphy assesses cardiac sympathetic nerve function.

Purpose of the Study:

  • To evaluate the relationship between 123I-MIBG scintigraphy findings and pathophysiologic changes in HCM.
  • To understand the progression of cardiac sympathetic nerve activity throughout the clinical course of HCM.

Main Methods:

  • 46 HCM patients and 18 controls underwent 123I-MIBG scintigraphy.
  • Patients were grouped by LV systolic function: normal (A), dysfunction (B), and dysfunction with dilatation (C).
  • Planar and SPECT imaging assessed early uptake, washout rate, and regional myocardial activity.

Main Results:

  • Group C (dysfunction with dilatation) showed significantly lower early 123I-MIBG uptake compared to controls.
  • Washout rates progressively increased from group A to C, indicating worsening sympathetic dysfunction.
  • Reduced regional uptake was most pronounced in group C, particularly in the interventricular septum.

Conclusions:

  • Cardiac sympathetic nerve abnormalities in HCM advance with LV systolic dysfunction and dilatation.
  • 123I-MIBG myocardial scintigraphy is a valuable tool for assessing pathophysiologic changes in HCM.
  • The findings support using 123I-MIBG to monitor disease progression in HCM.
Abstract

Related Concept Videos

Heart Failure II: Pathophysiology01:29

Heart Failure II: Pathophysiology

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...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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...
Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

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...
Sympathetic Activation01:16

Sympathetic Activation

The sympathetic division can influence tissues and organs by releasing norepinephrine at peripheral synapses and distributing epinephrine and norepinephrine through the bloodstream. In times of crisis or stress, sympathetic activation occurs, which is regulated by sympathetic centers in the hypothalamus. As a result, sympathetic activation prepares the body for physical exertion, rapid ATP production, and heightened alertness, allowing individuals to respond effectively to challenging or...
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System01:26

Heart Failure Drugs: Inhibitors of Renin-Angiotensin System

The activation of the sympathetic nervous system and the renin-angiotensin-aldosterone system (RAAS) contributes to cardiac remodeling, and inhibiting the RAAS is a pharmacological target in heart failure management. As a result, neurohumoral modulation is a crucial treatment principle for managing heart failure. This approach involves using medications like ACE inhibitors (ACEIs), angiotensin receptor blockers (ARBs), β-blockers, mineralocorticoid receptor antagonists (MRAs), and neutral...
Regulation of Heart Rates01:31

Regulation of Heart Rates

The regulation of heart rate is a complex process controlled by the autonomic nervous system (ANS), hormonal influences, and intrinsic cardiac mechanisms. The ANS has two main components: the sympathetic nervous system (SNS) and the parasympathetic nervous system (PNS).
The SNS increases heart rate through the release of norepinephrine and epinephrine, which act on beta-1 adrenergic receptors in the heart. This action increases the rate of depolarization in the sinoatrial (SA) node, the heart's...