Related Experiment Video

Updated: Aug 29, 2025

Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples
14:39

Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples

Published on: April 21, 2014

17.5K

Ventricular arrhythmia and sudden cardiac death in hypertrophic cardiomyopathy: From bench to bedside

Hua Shen1, Shi-Yong Dong2, Ming-Shi Ren1,3

  • 1Division of Adult Cardiac Surgery, Department of Cardiovascular Medicine, The Sixth Medical Center, Chinese PLA General Hospital, Beijing, China.

Frontiers in Cardiovascular Medicine
|September 5, 2022
PubMed

Insights

Identifying hypertrophic cardiomyopathy (HCM) patients at risk for sudden cardiac death (SCD) is crucial. Current risk scores are insufficient, necessitating novel molecular insights and personalized prevention strategies for ventricular arrhythmias.

Area of Science:

  • Cardiology
  • Molecular Biology
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) patients face risks of ventricular arrhythmias and sudden cardiac death (SCD).
  • Current risk stratification algorithms by ACC/AHA and ESC have limitations in identifying high-risk early-stage HCM patients.
  • SCD can occur unexpectedly, even in young, asymptomatic individuals, highlighting the need for new risk factors.

Purpose of the Study:

  • To review molecular mechanisms underlying ventricular arrhythmias and SCD in HCM.
  • To explore novel therapeutic targets and personalized prevention strategies for SCD in HCM.

Main Methods:

  • Review of studies utilizing surgical specimens, transgenic animal models, and patient-derived induced pluripotent stem cells (hiPSCs).
  • Analysis of molecular pathways including ion channel remodeling and calcium (Ca2+) homeostasis.
  • Evaluation of current and emerging therapeutic interventions.

Main Results:

  • Ion channel remodeling, Ca2+ dysregulation, and altered myofilament sensitivity contribute to arrhythmogenesis in HCM.
  • These molecular changes can lead to altered action potential duration (APD), reentry, and triggered activities (EADs, DADs).
  • Novel drugs targeting late sodium current and myosin show promise in preventing HCM-related arrhythmias.

Conclusions:

  • Existing SCD risk scores are inadequate for early-stage HCM.
  • Understanding molecular mechanisms is key to developing effective SCD prevention strategies.
  • Personalized prevention combining genetic screening, hiPSC-CM testing, machine learning, and advanced ECG is essential for managing SCD risk in HCM.

Keywords:
calcium homeostasisgene screeninghypertrophic cardiomyopathy (HCM)ion channelreentrysarcomeresudden cardiac death (SCD)

More Related Videos

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model03:45

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

3.4K
An Approach to Study Shape-Dependent Transcriptomics at a Single Cell Level06:02

An Approach to Study Shape-Dependent Transcriptomics at a Single Cell Level

Published on: November 2, 2020

5.8K

Related Concept Videos

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...
38
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
19
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
29
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
42
Disturbances in Heart Rhythm01:29

Disturbances in Heart Rhythm

Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
1.2K
Mechanism of Cardiac Arrhythmias01:28

Mechanism of Cardiac Arrhythmias

Arrhythmias are irregular heart rhythms occurring when the heart's electrical impulses become abnormal. These disturbances can lead to various symptoms, depending on their severity and the underlying cause. Some common factors contributing to arrhythmias include hypoxia, ischemia, electrolyte imbalances, excessive catecholamine exposure, drug toxicity, and muscle overstretching. Arrhythmias can be classified into two main types based on the rate and site of origin of abnormal heart rhythms.
1.0K