Related Experiment Video

Updated: Jul 16, 2026

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix
10:21

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

9.9K

Experimental Models of Hypertrophic Cardiomyopathy: A Systematic Review

Floor W van den Dolder1, Rafeeh Dinani1, Vincent A J Warnaar1

  • 1Department of Physiology, Amsterdam University Medical Center (UMC), Location VUmc, Amsterdam, the Netherlands; Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, the Netherlands.

JACC. Basic to Translational Science
|April 30, 2025
PubMed

Insights

Researchers reviewed hypertrophic cardiomyopathy (HCM) models, finding animal models and human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) show limited disease markers. Better reporting of study details is needed for optimal model selection.

Area of Science:

  • Cardiovascular Research
  • Genetics
  • Regenerative Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
  • Developing accurate models is crucial for understanding HCM pathogenesis and testing therapies.
  • Existing models vary in their ability to recapitulate key HCM hallmarks.

Purpose of the Study:

  • To systematically review experimental models of gene variant-induced hypertrophic cardiomyopathy.
  • To identify which major hallmarks of HCM are present in different model systems.
  • To guide researchers in selecting appropriate models for their specific research questions.

Main Methods:

  • Systematic literature review of 603 papers investigating HCM models.
  • Categorization of models into animal models and human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs).
  • Assessment of hallmark disease markers present in each model.

Main Results:

  • A shift towards using hiPSC-CMs is observed since 2010.
  • Only 36 mouse models exhibited 4 out of 6 HCM disease markers.
  • Only 17 hiPSC-CM models displayed 3 out of 4 HCM cell characteristics.
  • Incomplete reporting of sample size, sex, age, and disease characteristics was noted.

Conclusions:

  • Current experimental models, including animal and hiPSC-CMs, incompletely recapitulate hypertrophic cardiomyopathy.
  • There is a need for more comprehensive reporting of experimental details to improve model selection and reproducibility.
  • Further development of in vitro and in vivo models is required to better represent HCM complexity.

Keywords:
animal modelshuman iPSC-derived cardiomyocyteshypertrophic cardiomyopathystem cellssystematic review

More Related Videos

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

5.6K
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

2.9K

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 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...
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...
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...