Related Experiment Video

Updated: Jul 5, 2025

Implantation of Electroencephalogram and Electrocardiogram Telemetry Devices in Neonatal Rabbit Kits
06:46

Implantation of Electroencephalogram and Electrocardiogram Telemetry Devices in Neonatal Rabbit Kits

Published on: February 28, 2025

263

Inherited Arrhythmias in the Pediatric Population: An Updated Overview

Marco Valerio Mariani1, Nicola Pierucci1, Francesca Fanisio2

  • 1Department of Cardiovascular, Respiratory, Nephrological, Aenesthesiological and Geriatric Sciences, "Sapienza" University of Rome, 00161 Rome, Italy.

Medicina (Kaunas, Lithuania)
|January 23, 2024
PubMed

Insights

Pediatric cardiomyopathies and electrical heart diseases, often genetic, cause significant morbidity in children. This review updates knowledge on their genetics, characteristics, and outcomes, highlighting differences from adult conditions.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Pediatric cardiomyopathies (CMs) and electrical diseases are genetic disorders causing heart muscle abnormalities.
  • These conditions are major causes of illness and death in children, with dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) being most common.
  • Research and treatment guidelines for pediatric cases often rely on adult data, despite differing outcomes.

Purpose of the Study:

  • To review current evidence on the genetics, clinical features, management, and outcomes of primary pediatric CMs and electrical diseases.
  • To provide an updated overview of conditions including DCM, HCM, arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), long QT syndrome (LQTS), and short QT syndrome (SQTS).

Main Methods:

  • Literature review of recent studies on pediatric cardiomyopathies and electrical diseases.
  • Synthesis of evidence regarding genetic underpinnings, clinical presentations, and therapeutic approaches.
  • Analysis of outcomes data specific to the pediatric population.

Main Results:

  • Genetic variants are key to the etiology of these pediatric heart conditions.
  • Significant differences exist between pediatric and adult cardiac disease manifestations and outcomes.
  • Current management often extrapolates from adult protocols due to limited pediatric-specific research.

Conclusions:

  • Updated understanding of pediatric cardiomyopathies and electrical diseases is crucial for improved patient care.
  • Further research focusing on pediatric-specific data is needed to refine management strategies.
  • Recognizing the unique aspects of these diseases in children is essential for optimizing treatment and outcomes.

Keywords:
inherited arrhythmiaspediatric cardiomyopathiessudden cardiac death

More Related Videos

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

1.2K
Optimization of Transesophageal Atrial Pacing to Assess Atrial Fibrillation Susceptibility in Mice08:05

Optimization of Transesophageal Atrial Pacing to Assess Atrial Fibrillation Susceptibility in Mice

Published on: June 29, 2022

2.8K

Related Concept Videos

Mechanism of Cardiac Arrhythmias01:28

Mechanism of Cardiac Arrhythmias

Arrhythmias are irregular heart rhythms occurring when the heart's electrical impulses become abnormal. These disturbances can lead to various symptoms, depending on their severity and the underlying cause. Some common factors contributing to arrhythmias include hypoxia, ischemia, electrolyte imbalances, excessive catecholamine exposure, drug toxicity, and muscle overstretching. Arrhythmias can be classified into two main types based on the rate and site of origin of abnormal heart rhythms.
922
Disturbances in Heart Rhythm01:28

Disturbances in Heart Rhythm

Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow...
961
ECG Interpretation of Arrhythmias I: Sinus Arrhythmias01:16

ECG Interpretation of Arrhythmias I: Sinus Arrhythmias

Arrhythmias are disturbances in the heart's rhythm that lead to abnormal heartbeats. These irregularities can originate from different parts of the heart and are classified based on their origin and nature.
Types of Arrhythmias
Sinus Node Arrhythmias
Sinus Bradycardia: Originating from the sinoatrial (SA) node, sinus bradycardia involves slower impulses, resulting in a heart rate of less than 60 beats per minute (bpm). Causes include sleep, vagal stimulation, beta-blockers, hypothyroidism,...
216
Antiarrhythmic Drugs: Class I Agents as Sodium Channel Blockers01:22

Antiarrhythmic Drugs: Class I Agents as Sodium Channel Blockers

Class I antiarrhythmic drugs are used to treat various types of arrhythmias or irregular heart rhythms. These drugs block the sodium (Na+) channels in the cardiac cells, thereby affecting the movement of electrical impulses across the heart. Class I antiarrhythmic drugs are divided into three subgroups: Class IA, Class IB, and Class IC, each with distinct mechanisms of action and effects on the heart.
Class 1A Antiarrhythmic Drugs: These drugs work by moderately blocking sodium channels,...
1.4K
Antiarrhythmic Drugs: Class II Agents as β-Adrenergic Blockers01:24

Antiarrhythmic Drugs: Class II Agents as β-Adrenergic Blockers

Adrenergic stimulation generally impacts cardiac rate and rhythm. Specifically, stimulation of the β-adrenoceptors triggers an increase in intracellular calcium ion influx and pacemaker currents, which may cause arrhythmias. Catecholamines like adrenaline also demonstrate β2-adrenoceptor-mediated hypokalemia, impacting cardiac action potential and disrupting the normal cardiac rhythm. Class II antiarrhythmic drugs are β-adrenoceptor antagonists or β-blockers, which...
746
Electrophysiology of Normal Cardiac Rhythm01:19

Electrophysiology of Normal Cardiac Rhythm

The normal cardiac rhythm is a synchronized electrical activity that facilitates the regular and coordinated contraction of the heart muscle. This process is essential for efficient blood circulation throughout the body. The fundamental elements involved in establishing and maintaining this rhythm include the unique electrical properties of cardiac muscle cells, the sinoatrial (SA) node's pacemaker function, the specialized conducting system, and the ionic mechanisms underlying each phase...
4.4K