Related Experiment Video

Updated: Sep 25, 2025

An Approach to Study Shape-Dependent Transcriptomics at a Single Cell Level
06:02

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

Published on: November 2, 2020

5.8K

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic

Gabrielle Norrish1,2, Tao Ding3, Ella Field1

  • 1Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom (G.N., E.F., E.C., J.P.K.).

Circulation. Arrhythmia and Electrophysiology
|May 2, 2022
PubMed

Insights

Maximal left ventricular wall thickness (MLVWT) in children with hypertrophic cardiomyopathy (HCM) shows an inverted U-shaped relationship with sudden cardiac death (SCD) risk. Additional risk factors amplify this risk, necessitating a comprehensive approach to SCD prevention.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Genetics

Background:

  • Maximal left ventricular wall thickness (MLVWT) is a known risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM).
  • The relationship between left ventricular hypertrophy severity and SCD risk in adults with HCM is nonlinear.
  • This complex relationship has not been well-defined in pediatric HCM populations.

Purpose of the Study:

  • To investigate the relationship between left ventricular hypertrophy and SCD risk in a large international cohort of pediatric HCM patients.
  • To determine if the nonlinear relationship observed in adults is also present in children with HCM.
  • To evaluate the impact of coexisting risk factors on SCD risk in pediatric HCM.

Main Methods:

  • A cohort of 1075 children (aged 1-16 years) with HCM was analyzed from the International Paediatric Hypertrophic Cardiomyopathy Consortium.
  • Noninvasive clinical data were collected at baseline and follow-up.
  • Five-year estimated SCD risk was calculated using the HCM Risk-Kids tool, with MLVWT Z-scores categorized.

Main Results:

  • MLVWT Z-scores were categorized as <10 (58.1%), 10 to <20 (31.1%), and ≥20 (13.3%).
  • Higher MLVWT Z-scores correlated with heart failure symptoms, syncope, left ventricular outflow tract obstruction, left atrial dilatation, and ventricular tachycardia.
  • A 5-year SCD event occurred in 10.7% of patients. The relationship between SCD risk and MLVWT Z-score was an inverted U-shape, peaking at Z-score +23. Patients with MLVWT Z-score ≥20 had coexisting risk factors in 71.3% of cases.
  • Five-year freedom from SCD was 95.6% (<10 Z-score), 87.4% (10 to <20 Z-score), and 86.0% (≥20 Z-score).

Conclusions:

  • An inverted U-shaped relationship exists between left ventricular hypertrophy and SCD risk in pediatric HCM.
  • The presence of additional risk factors has a cumulative effect on SCD risk.
  • MLVWT is valuable for risk stratification but should not solely guide decisions regarding implantable cardioverter-defibrillator implantation in children with HCM.
Abstract

Keywords:
adultchilddeath, suddenhumanshypertrophic cardiomyopathy

More Related Videos

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix10:21

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

10.2K
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

3.8K

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...
73
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,...
41
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...
78
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...
86
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...
48
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...
53