Related Experiment Video

Updated: Jun 8, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
03:45

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

Published on: August 8, 2022

Restrictive cardiomyopathy in childhood

Susan W Denfield1, Steven A Webber

  • 1Lillie Frank Abercrombie Division of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin MC-19345C, Houston, TX 77030, USA. swdenfie@texaschildrenshospital.org

Heart Failure Clinics
|September 28, 2010
PubMed

Insights

Pediatric restrictive cardiomyopathy is a rare heart condition with poor outcomes. This review covers its causes, diagnosis, and management in children.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research

Background:

  • Restrictive cardiomyopathy (RCM) in children is rare globally.
  • It can be idiopathic or secondary to endomyocardial fibrosis, particularly in endemic regions.
  • RCM in pediatric patients carries a poor prognosis once symptoms manifest.

Purpose of the Study:

  • To provide a comprehensive review of pediatric restrictive cardiomyopathy.
  • To cover definitions, epidemiology, etiologies, genetics, phenotypes, clinical presentation, diagnosis, outcomes, and management.
  • To consolidate current knowledge on this rare pediatric condition.

Main Methods:

  • Literature review of pediatric restrictive cardiomyopathy.
  • Synthesis of data on definitions, epidemiology, and etiologies.
  • Compilation of information on genetics, clinical presentation, and diagnostic approaches.

Main Results:

  • RCM in childhood is either rare and idiopathic or linked to endomyocardial fibrosis.
  • The condition has a poor prognosis after symptom onset, irrespective of the cause.
  • Genetic factors and "overlap" phenotypes contribute to the complexity of RCM.

Conclusions:

  • Early diagnosis and appropriate management are crucial for improving outcomes in pediatric RCM.
  • Further research into the poorly understood etiologies, particularly endomyocardial fibrosis, is warranted.
  • Understanding the genetic basis and diverse phenotypes is key to effective RCM care in children.

More Related Videos

A Doxorubicin-induced Cardiomyopathy Model in Adult Zebrafish08:09

A Doxorubicin-induced Cardiomyopathy Model in Adult Zebrafish

Published on: June 7, 2018

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia09:36

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia

Published on: December 22, 2023

Related Concept Videos

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 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 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 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...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...