Related Experiment Video

Updated: Jun 2, 2026

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
09:36

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

Published on: December 22, 2023

Role of TRPM4 ion channel in pediatric arrhythmic syndromes

Olesya V Melnik1,2, Olga E Kulichik3, Anastasiya K Zaytseva4

  • 1Institute of Molecular Biology and Genetics, Almazov National Medical Research Centre, Saint Petersburg 197341, Sankt-Peterburg, Russia.

World Journal of Clinical Pediatrics
|June 1, 2026
PubMed

Insights

Transient Receptor Potential Melastatin 4 (TRPM4) gene variants are linked to heart rhythm disorders in children. Understanding TRPM4 dysfunction is key to diagnosing and treating pediatric arrhythmias.

Area of Science:

  • Cardiology
  • Genetics
  • Ion Channel Physiology

Background:

  • TRPM4 ion channel is crucial for cardiomyocyte function, calcium regulation, and cardiac conduction.
  • Pathogenic TRPM4 variants are frequently associated with conduction disorders and arrhythmias in pediatric patients.
  • TRPM4 dysfunction is implicated in Brugada syndrome and long QT syndrome.

Purpose of the Study:

  • To review the role of TRPM4 variants in pediatric arrhythmic syndromes.
  • To discuss the molecular mechanisms underlying TRPM4 dysfunction.
  • To present clinical cases highlighting TRPM4's role in pediatric arrhythmia pathogenesis.

Main Methods:

  • Literature review of TRPM4 gene variants and their association with cardiac conditions.
  • Analysis of molecular mechanisms of TRPM4 ion channel dysfunction.
  • Compilation of clinical illustrations and case presentations.

Main Results:

  • TRPM4 variants significantly contribute to the development of various pediatric arrhythmic syndromes.
  • Both gain-of-function and loss-of-function TRPM4 variants can lead to cardiac dysfunction.
  • Clinical manifestations range from conduction abnormalities to complex arrhythmias.

Conclusions:

  • TRPM4 plays a critical role in the molecular pathogenesis of arrhythmias in young individuals.
  • Genetic testing for TRPM4 variants is important for diagnosing and managing pediatric cardiac rhythm disorders.
  • Further research into TRPM4 dysfunction can inform novel therapeutic strategies.

Keywords:
ArrhythmiaCardiac conductionChildrenPathophysiologyTransient receptor potential channels

More Related Videos

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV412:09

Yeast Luminometric and Xenopus Oocyte Electrophysiological Examinations of the Molecular Mechanosensitivity of TRPV4

Published on: December 31, 2013

Related Concept Videos

Antiarrhythmic Drugs: Class IV Agents as Calcium Channel Blockers01:20

Antiarrhythmic Drugs: Class IV Agents as Calcium Channel Blockers

Class IV antiarrhythmic drugs, such as verapamil and diltiazem, block calcium channels. They primarily affect the heart, slowing the conduction in calcium-dependent tissues like the SA and AV nodes. These drugs manage reentrant supraventricular tachycardia (SVT) and reduce ventricular rate in atrial flutter/fibrillation.
Verapamil, a calcium channel blocker, inhibits calcium movement across myocardial cell membranes and vascular smooth muscle. This results in the dilation of coronary and...
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.
Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
Dysrhythmias VI: Management of Dysrhythmias01:25

Dysrhythmias VI: Management of Dysrhythmias

Dysrhythmia management involves a multifaceted approach, incorporating pharmacological treatments, medical procedures, surgical interventions, lifestyle modifications, and patient education.Pharmacological ManagementAntiarrhythmic Drugs:Class I (Sodium Channel Blockers): This class includes quinidine and procainamide, which reduce the speed of impulse conduction in the heart, stabilize the cardiac membrane, and control arrhythmias. Quinidine and procainamide are Class IA agents that prolong the...
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 of...
Disturbances in Heart Rhythm01:29

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 heart...