Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy

Ingegerd Ostman-Smith1, Göran Wettrell, Barry Keeton

  • 1Division of Paediatrics, Department of Clinical Sciences, Sahlgren Academy, Queen Silvia Childreńs Hospital SE-416 85, Gothenburg, Sweden. ingegerd.ostman-smith@pediat.gu.se

Insights

Hypertrophic cardiomyopathy (HCM) poses the highest risk for sudden cardiac death in children aged 9-13.9 years, necessitating earlier screening than current guidelines recommend.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a leading inherited cause of sudden cardiac death in children.
  • Current screening guidelines recommend HCM assessment starting at 12-15 years of age.

Purpose of the Study:

  • To determine the specific age range at highest risk for sudden cardiac death in pediatric patients with HCM.
  • To evaluate age- and gender-specific mortality rates in children with HCM.

Main Methods:

  • A cohort study involving 150 children from six pediatric cardiology centers, including those who experienced sudden death.
  • Analysis of age- and gender-specific mortality, compared with national death registry data.
  • Calculation of sudden death mortality rates within specific age brackets.

Main Results:

  • Sudden arrhythmia deaths occurred in 31 children under 19 years old.
  • The annual sudden death mortality rate averaged 7.2% between 9-13.9 years, significantly higher than the 1.7% observed after 16 years (P=0.025).
  • Sudden death risk peaked earlier in girls (10-11 years) and showed a male predominance after age 15. National data confirmed higher HCM mortality in 8-16 year olds compared to 17-30 year olds.

Conclusions:

  • The findings indicate that children with a family history of HCM are at the highest risk for sudden cardiac death at a younger age than currently screened.
  • Early screening for hypertrophic cardiomyopathy in children is crucial, particularly within the 9-13.9 age group.
Abstract

Related Concept Videos

Pathophysiology of Heart Failure01:17

Pathophysiology of Heart Failure

Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
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 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 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...
Cellular Adaptation II: Hypertrophy01:26

Cellular Adaptation II: Hypertrophy

Hypertrophy is the increase in the size of individual cells, resulting in the enlargement of a tissue or organ. Unlike hyperplasia, which involves an increase in cell number, hypertrophy is characterized by an increase in cell volume. This process often occurs in response to higher functional demand or hormonal stimulation, leading to the production of more structural proteins and organelles, thereby enhancing the cells' work capacity.There are two primary types of hypertrophy: physiological...