This section is for pediatric patients and families living with heart rhythm disorders and heart rhythm disorders related to congenital heart disease (CHD).
This content is developed in partnership with the Pediatric & Congenital Electrophysiology Society (PACES).
To find a pediatric and congenital heart rhythm provider, PACES offers additional resources.
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Assess Your AFib Risk
Atrial fibrillation (AFib) is the most common abnormal heart rhythm. In a normal heart, the four chambers of the heart beat in a steady, rhythmic pattern. With AFib, the atria (upper chambers of the heart) fibrillate (quiver or twitch quickly) and create an irregular rhythm.
A Child's Normal Conduction System
The sinus node (sinoatrial node or SA node) is the heart's own pacemaker. The electrical impulse is normally initiated here. The impulse travels across the atria (top 2 collecting chambers) stimulating contraction or "squeeze" and will eventually push blood into the ventricles (the bottom 2 pumping chambers).
A Child's Normal Heart
The heart is the hardest working muscle in the human body. Located almost in the center of the chest, the heart of a child is about the size of his/her fist. A child's heart works just as hard as an adult's heart. In fact, a baby's heart may beat up to 190 times a minute, while an adult's heart usually beats between 60 and 100 times a minute.
Arrhythmias & Congenital Heart Disease in Children
There are many different causes that can create rhythm disturbances in patients with congenital heart disease. The type of structural anatomy that you were born with, surgical procedures that have been performed on your heart, long term consequences of chamber enlargement and scar formation can alter the anatomy of the electrical system of the heart, resulting in rhythm disturbances. Rhythms can often be too fast or too slow, producing a wide range of symptoms that patients may report.
Arrhythmogenic Cardiomyopathy (AVC) in Children
Arrhythmogenic ventricular cardiomyopathy (AVC) is a rare condition that affects the heart muscle and can be associated with ventricular arrhythmias. Other names that are used interchangeably are arrhythmogenic right ventricular cardiomyopathy (ARVC), or arrhythmogenic right ventricular dysplasia (ARVD).
Atrial Fibrillation (AFib) in Children
Atrial fibrillation (AFib) is disorganized, rapid electrical discharges in the atria (top chambers), creating an irregular heartbeat in the atria that often does not coordinate with the heartbeat in the bottom chambers (ventricles). This rhythm can occur in children with normal heart structure and those with congenital heart disease. This rhythm can occur in children with normal heart structure and those with congenital heart disease.
Atrial Tachycardia in Children
Atrial tachycardia is a health condition (arrhythmia) where the atria of the heart as an electrical problem that causes the atria to beat at a rapid heart rate. Atrial tachycardia can include multiple types of fast heart rhythm from the upper chambers of the heart (atria).
Atrioventricular Reentrant Tachycardia (AVRT) in Children
AV reentrant tachycardia (AVRT) is a type of heart rhythm disorder that begins in the upper chambers of the heart (atria) and travels through an extra abnormal pathway, called an accessory pathway or bypass tract. An accessory pathway is an extra band of tissue/fibers that connect the atria to the ventricles.
AV-Nodal Reentrant Tachycardia (AVNRT) in Children
AV-nodal reentrant tachycardia (AVNRT) is a type of heart rhythm disorder that begins in the upper chambers of the heart (atria) and travels through an abnormal electrical circuit within the AV node.
Bradyarrhythmias in Children
Bradyarrhythmias are slower than normal heart rhythms (normal conduction system) and occur when the electrical impulse does not originate in the sinoatrial (SA) node, or when the electrical impulse travels too slowly from the SA node to the ventricles.
Brugada Syndrome (BrS) in Children
Brugada syndrome (BrS) is a channelopathy that can also cause dangerous ventricular arrhythmias in the heart. Patients with BrS can have an abnormal pattern on their ECG.
Cardiac (Heart) Ablation for Children
A cardiac (heart) ablation is a procedure to treat heart arrhythmias. An ablation is done by placing long, soft, flexible tubes called catheters into the big blood vessels in the body. These catheters are used for diagnosis of the arrhythmia and ablation where they burn or freeze an area in the heart to eliminate the source of the arrhythmia. Ablation can cure arrhythmias that occur in the upper chambers (atria) or lower chambers (ventricles) of the heart.
Cardiac MRI for Children
Cardiac MRI uses a powerful magnetic field, radio waves and a computer to produce the detailed structures of the heart. This is useful tool to evaluate structures that may be difficult to visualize by echocardiography.
Cardioversion in Children
Cardioversion is a medical procedure to treat heart arrhythmias and restore a normal heart rhythm.
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) in Children
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a heart rhythm problem (arrhythmia). If you have it, your heartbeat is faster and irregular at times. When this happens, it’s harder for the heart to pump enough.
Dilated Cardiomyopathy (DCM) in Children
Dilated cardiomyopathy (DCM) is a disease of the heart muscle. In DCM, the bottom chamber (left ventricle) of the heart becomes enlarged (dilated), thinning the ventricle walls and reducing the heart's ability to squeeze effectively and pump blood efficiently.
Drug Infusion Studies in Children
A drug (medication) infusion study is used to try and help an electrophysiologist determine if a patient has certain kinds of cardiac (heart) genetic syndromes or channelopathies (genetic syndromes). It can help diagnose patients who have been thoroughly evaluated but still don’t have a confirmed diagnosis that explains their symptoms.
Echocardiogram (Echo) for Children
Echocardiogram (or echo) uses sound waves for show images of your heart. An echo can show how well the heart is functioning (squeezing) and will identify if there are any structural heart abnormalities.
Ectopic Atrial Tachycardia (EAT) in Children
Ectopic atrial tachycardia (EAT), sometimes called atrial tachycardia (AT), is an abnormally fast heart rate originating from the top of the heart.
Electrocardiogram (ECG/EKG) for Children
An electrocardiogram (ECG), sometimes called an EKG, is a noninvasive test that records the electrical signals of your heart and can help diagnose many common heart problems.
Electrophysiology (EP) Studies in Children
An electrophysiology study involves studying the heart’s electrical activity to look for irregularities that cause arrhythmias. Electrophysiology (EP) studies are typically performed in patients with known abnormally fast heart rates, called tachyarrhythmias.
Event Monitors for Children
Event monitors are a special type of long term rhythm monitor. Some are programmed to automatically record arrhythmias, or has to be activated with symptoms by the patient. Some are worn continuously and some are only used with symptoms.
Exercise Stress Tests for Children
Exercise ECGs, also called Exercise Stress Tests require electrodes to be placed on the skin just like resting ECGs.
Genetic Syndromes in Children: Channelopathies & Cardiomyopathies
Heart muscle cells contract because of movement of certain molecules (called "ions") across the walls (called "membranes") of the cells. Channelopathies increase a child's risk for life-threatening heart rhythms and for sudden cardiac death (SCD). It is important to remember that while channelopathies are serious medical conditions, they are frequently treatable when they are detected and properly diagnosed.
Genetic Testing & Counseling for Children
Genetic testing and genetic counseling can be valuable tools when evaluating patients, and their family members, for channelopathies. The genetic changes that cause some channelopathies may even guide your cardiologist or electrophysiologist (cardiologist who specializes in heart rhythm conditions) to choose one specific medication. If a known disease-causing genetic mutation (change in the gene’s sequence or pattern) is found in a patient, it can help direct future testing in other family members to determine their risk of developing the disease.
Heart Block in Children
Heart block, also called atrioventricular block or AV block, is a term for slowing, or “blocking” of the electrical impulses in the heart. Heart block does not refer to a physical block of the blood supply or blood flow through the heart. It occurs when the electrical signals originating from the sinus node are either delayed or completely prevented from traveling to the ventricles. This can be caused by congenital defects in the heart’s conduction system – which can occur in hearts with or without structural congenital heart disease – or can result from other circumstances, such as infection, surgery, or treatment with certain medications.
Holter Monitors for Children
Holter monitors are noninvasive rhythm monitors that record every heart beat over a certain period; usually 24-48 hours.
Hypertrophic Cardiomyopathy (HCM) in Children
Hypertrophic cardiomyopathy (HCM) is one of the most common heritable cardiac condition and is the most common cause of sudden cardiac arrest in the young, affecting about 1 in 500 people.
Implantable Cardioverter Defibrillators (ICDs) in Children
An implantable cardioverter defibrillator (ICD) is a special type of cardiac device that can recognize abnormal heart rhythms in the bottom part of the heart and deliver therapy if needed to restore the heart to a normal rhythm.
Implantable Loop Recorders (ILRs) in Children
Implantable loop recorders (ILR) are small event monitors implanted under the skin that are battery-powered and can stay in place for years. ILRs allow for long-term monitoring of your heart rhythm. ILRs can automatically detect arrhythmias based and have a patient-activated recorder for symptomatic episodes.
Junctional Bradycardia in Children
Junctional rhythm occurs when the electrical impulse in the heart starts in the atrio-ventricular node (AV node) instead of the sinoatrial (SA) node. Junctional rhythms can often be normal, but sometimes they are too fast or too slow. Junctional bradycardia is a type of junctional rhythm where there heart rate is slower than the normal heart rate for the patient’s age.
Junctional Tachycardia (JET) in Children
Junctional tachycardia, sometimes called junctional ectopic tachycardia (JET), is an abnormally fast heart rate originating from the atrioventricular (AV) junction.
Junctional Tachycardia in Children
Junctional tachycardia, sometimes called junctional ectopic tachycardia (JET), is an abnormally fast heart rate originating from the atrioventricular (AV) junction. The AV junction is a group of cells that is part of the heart’s natural electrical system and is located just below the atrioventricular node. When the AV junction becomes irritated or excited, it can start sending out electrical signals faster than the sinus node, the heart’s natural pacemaker.
Left Ventricular Non-Compaction (LVNC) in Children
Left ventricular non-compaction (LVNC) is a disease where the left ventricular muscle on the echocardiogram appears soft and spongy instead of smooth and compact.
Long QT Syndrome (LQTS) in Children
Long QT syndrome (LQTS) is a condition that affects the flow of ions into and out of the cells in the heart. This abnormal flow of ions can prolong the patient’s QT interval, which is measured on an ECG. The prolonged QT interval affects how electrical impulses travel through the heart. Electrical activity may be transmitted abnormally through the lower heart chambers (ventricles) leading to potentially life-threatening ventricular arrhythmias. LQT syndrome is usually, but not always, inherited.
Pacemakers for Children
A pacemaker is a device that uses low energy electrical pulses to prompt the heart to beat whenever a pause in the rhythm is detected. They can help to coordinate electrical signals between the upper and lower chambers so they will beat in a coordinated fashion. Pacemakers can help speed up a slow heart rate.
Permanent Junctional Reciprocating Tachycardia (PJRT) in Children
Permanent junctional reciprocating tachycardia, more commonly referred to as PJRT, is a rare form of supraventricular tachycardia (SVT), or abnormal fast heartbeats, typically seen in infants and children.
Premature Atrial Complexes (PACs) in Children
Premature atrial complexes (PACs), sometimes called atrial premature complexes (APCs), are caused by activation of the electrical system in the top of the heart earlier than expected.
Premature Ventricular Contraction (PVC) in Children
Premature ventricular contractions, more commonly known as PVCs are extra heartbeats that arise from one of the two bottom chambers of the heart (left or right ventricle)
Remote Monitoring for Children
Patients with cardiac devices such as pacemaker, ICD or ILR have the option of remote monitoring from home. This provides information about the device function (including how the leads are functioning and battery life), abnormal heart rhythm and signs of heart failure to your cardiology team.
Restrictive Cardiomyopathy in Children
Restrictive cardiomyopathy (RCM) is a rare disease where the heart muscle becomes rigid or stiff. This makes it difficult for the ventricles to relax and properly fill with blood
Signal Averaged ECG
Signal averaged ECG is a noninvasive ECG that is processed to be able to identify low amplitude signals at the end of the ventricular complex. Findings of late potentials are representative of delayed ventricular activation.
Sinus Node Dysfunction
The sinus node (SA node) consists of a group of specialized cells within the right atrium and is the heart’s natural pacemaker. Normally, the heart’s electrical impulse starts in the SA node. This important structure allows the heart rate to increase or decrease in response to the body's activity level. Sinus node dysfunction (SND) can occur in patients with congenital heart disease (CHD), especially those who have had cardiac surgery which results in scarring within the right atrium.
Supraventricular Tachycardia (SVT) in Children
Supraventricular tachycardia, most commonly referred to as SVT, is the most common type of abnormal fast heart rhythms in children. SVT is a broad term and includes many different forms all with similar type of symptoms. The type of SVT is classified based on the path of electrical signal during the tachycardia. SVT begins in the upper chambers of the heart, (Atria), travels through an abnormal electrical circuit within the AV node (AV node reentrant tachycardia or AVNRT), or, through an extra abnormal pathway, called an accessory pathway or bypass tract (AV reentrant tachycardia or AVRT). An accessory pathway is an extra band of tissue/fibers that connect the atria to the ventricles.
Vagal Maneuvers in Children
Vagal maneuvers can stop certain types of SVT in children
Ventricular Tachycardia in Children
Ventricular tachycardia (VT) is an arrhythmia that causes the bottom chambers of the heart (ventricles) to beat faster and independent from the top chambers. VT is defined as 3 consecutive abnormal ventricular beats.
Wolff-Parkinson-White Syndrome (WPW)
Wolff-Parkinson-White (WPW) syndrome is an abnormality of the heart’s electrical conduction system which can be associated with supraventricular tachycardia (SVT).