Related Experiment Video

Updated: Aug 6, 2025

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

3.3K

Sudden cardiac death in childhood hypertrophic cardiomyopathy

Keval Thakkar1, Adnan Rafiq Karajgi2, Anusha Manoj Kallamvalappil3

  • 1G.M.E.R.S. Medical College and General Hospital, Gandhinagar, India.

Disease-A-Month : DM
|March 17, 2023
PubMed

Insights

Sudden cardiac death (SCD) is a major risk for children with hypertrophic cardiomyopathy (HCM). Current risk assessment tools, derived from adult data, are inadequate, necessitating pediatric-specific strategies for SCD prevention in childhood HCM.

Area of Science:

  • Cardiology
  • Pediatrics
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) poses a significant mortality risk in children, primarily due to sudden cardiac death (SCD).
  • Existing risk stratification methods for SCD in HCM are largely based on adult data, with limited applicability to pediatric populations.
  • Childhood-onset HCM presents unique challenges, including higher SCD rates compared to adults, underscoring the need for specialized approaches.

Purpose of the Study:

  • To review the epidemiology, pathophysiology, genetic factors, and complications associated with SCD in pediatric HCM.
  • To evaluate the effectiveness of current risk stratification tools and preventive strategies for SCD in children with HCM.
  • To discuss the need for and potential development of pediatric-specific risk stratification methodologies for SCD in HCM.

Main Methods:

  • Comprehensive literature search of Medline, Cochrane, Embase, and Google Scholar up to October 2021.
  • Inclusion of studies using keywords and MeSH terms related to HCM, SCD, and childhood-onset HCM.
  • Systematic review and synthesis of data on etiology, risk factors, genetics, complications, and management of SCD in pediatric HCM.

Main Results:

  • Childhood-onset HCM is associated with substantial lifelong morbidity and mortality, with a higher incidence of SCD than in adults.
  • Current preventive strategies, including implantable cardioverter-defibrillator (ICD) placement, rely on risk factor thresholds with limited discriminatory power in children.
  • Significant disparities exist between pediatric and adult HCM cohorts, highlighting the inadequacy of adult-derived risk stratification.

Conclusions:

  • Pediatric-specific risk stratification for SCD in HCM is critically needed due to differences from adult populations.
  • Future research should focus on developing and validating novel risk assessment tools tailored for children with HCM.
  • Advances in disease-modifying therapies offer potential to improve outcomes and alter disease expression in young individuals with HCM.

Keywords:
CardiologyChildhoodHypertrophic cardiomyopathyInternal medicineSudden cardiac death

More Related Videos

A Modified Sonographic Algorithm for Image Acquisition in Life-Threatening Emergencies in the Critically Ill Newborn11:27

A Modified Sonographic Algorithm for Image Acquisition in Life-Threatening Emergencies in the Critically Ill Newborn

Published on: April 7, 2023

6.3K
Implantation of Electroencephalogram and Electrocardiogram Telemetry Devices in Neonatal Rabbit Kits06:46

Implantation of Electroencephalogram and Electrocardiogram Telemetry Devices in Neonatal Rabbit Kits

Published on: February 28, 2025

360

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...
18
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...
26
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,...
14
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...
21
Coronary Artery Disease III: Clinical Manifestations01:30

Coronary Artery Disease III: Clinical Manifestations

Coronary Artery Disease (CAD) is a primary health risk worldwide, leading to significant morbidity and mortality. The condition arises from the buildup of atherosclerotic plaques within the coronary arteries, resulting in diminished blood supply to the heart muscle.The clinical manifestations of CAD vary widely, from asymptomatic stages to severe, life-threatening conditions. Understanding these manifestations is crucial for early diagnosis and effective management.Angina Pectoris: The Warning...
19
Introduction Cardiac Emergencies01:30

Introduction Cardiac Emergencies

Cardiac emergencies are critical situations involving the heart that require immediate medical intervention to prevent severe complications or death. These emergencies often arise from underlying heart conditions that impair the heart's ability to function correctly.Types of Cardiac EmergenciesThe most common types of cardiac emergencies include Acute Coronary Syndrome (ACS), myocardial infarction (MI), cardiac arrest, and heart failure.Acute Coronary Syndrome (ACS)Acute Coronary Syndrome (ACS)...
38