Related Experiment Video

Updated: Jun 19, 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

Diastolic dysfunction and its histopathological correlation in obstructive hypertrophic cardiomyopathy in children

Shaji C Menon1, Benjamin W Eidem, Joseph A Dearani

  • 1Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA. shaji.menon@utah.edu

Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography
|October 10, 2009
PubMed

Insights

Myocyte disarray is a key factor in diastolic dysfunction for pediatric obstructive hypertrophic cardiomyopathy (HCM). This finding in young HCM patients highlights a critical mechanism for diastolic dysfunction.

Area of Science:

  • Cardiology
  • Pathology
  • Pediatric Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) histopathology shows myocyte hypertrophy, disarray, and fibrosis.
  • Limited data exists correlating echocardiographic findings with histopathology in HCM.

Purpose of the Study:

  • To investigate the relationship between echocardiographic parameters and histopathologic findings in pediatric obstructive HCM.
  • To elucidate the mechanism of diastolic dysfunction in young HCM patients.

Main Methods:

  • Retrospective review of echocardiographic data from 45 pediatric obstructive HCM patients undergoing septal myectomy.
  • Comparison of echocardiographic data with histologic findings from myectomy specimens.

Main Results:

  • Histopathology revealed significant myocyte hypertrophy (100%), disarray (98%), interstitial fibrosis (95%), and subendocardial fibrosis (97%).
  • Multivariate analysis showed a significant link between myocyte disarray and echocardiographic markers of left ventricular diastolic dysfunction.

Conclusions:

  • Myocyte disarray is a primary contributor to diastolic dysfunction in pediatric obstructive HCM.
  • These findings offer new insights into HCM pathophysiology, particularly diastolic dysfunction in young patients.
Abstract

More Related Videos

Quantification of Global Diastolic Function by Kinematic Modeling-based Analysis of Transmitral Flow via the Parametrized Diastolic Filling Formalism11:04

Quantification of Global Diastolic Function by Kinematic Modeling-based Analysis of Transmitral Flow via the Parametrized Diastolic Filling Formalism

Published on: September 1, 2014

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...
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 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,...
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 IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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...