Karazin Kharkiv National University

Department of Pediatrics

Differential diagnostics of cardial rhythm

and conduction disorders in children.
Emergency management of paroxysmal
rhythm disturbance and Morgagni-Adams-
Stokes syndrome.

Associate professor Tetiana Holovko

 Arrhythmias (сardiac dysrhythmias) or
abnormal heart rhythms
• There are many different kinds of abnormal heart rhythms that
may occur in children and adults. If an abnormal rhythm
occurs, it's important to find out what kind it is. Treatment
recommendations depend on its type. May be disorders of
frequency, regularity of rhythm, source of heart impulses
activations and it also means the dysfunction in connection or
sequence between atrial and ventricular activation.

• Arrhythmias in children are much less common than in adults.

Many pediatric dysrhythmias are normal variants that do not
require treatment or even further evaluation, but can be just as
life threatening.
 Etiological (trigger) factors of arrhythmias in
children

Cardial factors Extracardial factors

Congenital: 1. Infections
1. CHD (VSD, ASD, Ebstein 2. Psychogenic factors (stress)
anomaly, PDA, tetralogy 3. Diseases and injuries of
Fallow) nervous system
2. Carditis 4. Electrolyte disturbances
3. Cardiomyopathy (↓↑K+, ↓↑Ca2+, ↓Mg2+)
4. Anomalies of heart 5. Intoxications (Uremia)
conduction system (AV-block, 6. Drugs (antiarrhythmic agents,
WPW syndrome) digoxin, sympathomimetic
5. Mitral valve prolapse agents)
 Etiological (trigger) factors of arrhythmias in children

Cardial factors Extracardial factors

Acquired: 7. Dyshormonal conditions
1. Rheumatic fever (Thyrotoxicosis,
2. Nonrheumatic carditis pheochromocytoma)
3. Systemic diseases of connective 8. Traumas of chest and

tissue (SLE, systemic sclerosis, abdomen

dermatomyositis) 9. Alimentary conditions
Other: (obesity).
1. Idiopathic cardiomyopathy
2. Aneurysm
3. Heart tumors
 Working classification of cardiac rhythm
disorders in children
(Belocon N.A., Kuberger M.B., 1987):

I. Disorders of impulse
A. Nomotopic rhythm forming disorders:
1) Sinus arrhythmia;
2) Sinus bradycardia;
3) Sinus tachycardia;
4) Wandering pacemaker.
B. Heterotopic (ectopic) disorders of rhythm:
1. Premature beats or extra beats (extrasystoles):
a) Premature Atrial Contraction;
b) Premature Ventricular contractions.
2. Paroxysmal tachycardia:
а) supraventricular (atrial, atrioventricular);
б) ventricular.
3. Nonparoxysmal tachycardia:
а) atrial with atrioventricular block and without it;
б) from atrioventricular connection;
в) ventricular.
4. Atrial flutter and fibrillation.
5.Ventricular flutterer and fibrillation.
II. Disorders of conduction.
1. Sinoauriculal block.
2. Intra-atrial block.
3. Atrioventricular block(I,II,III degree).
4. Intraventricular block:
а) onesided, bothsided;
б) incomplete, complete;
в) permanent, transitory, intermittent.

III. Combined arrhythmia.

1. Sick sinus syndrome;
2. Atrioventricular dissociation;
3. Premature of ventricle excitement syndrome;
4. Long QT syndrome.
 Clinical picture (common sings):

• Feeling of palpitation (sinus tachycardia under

physical load or emotion);
• Feeling of intermission in heart work (extrasystole);
• Combination of high heart rate with sudden
beginning and end of palpitation paroxysms
(paroxysmal tachycardia);
• Syncope (Morgagni-Adams-Stokes attack);
• BP decreases.
Diagnostic techniques of cardiac rhythm disorders.
• Basic method for diagnosing of arrhythmias till now
is superficial ECG in 12 traditional leads;
• 24hr monitoring of ECG (Holter monitoring);
• Transesophageal ECG;
• Intraesophageal electrostimulation of heart.
 Normal Sinus Rhythm

Regularity: Regular
Rate: 60 - 100 beats per minute
P wave: Normal and upright; one P wave in front of every
QRS complex
PR: 0.12–0.20 seconds and constant
QRS: Less than 0.12 seconds
 Normal values of heart rate in children

Age Heart rate.

newborn 140 (<110 - >170)
10-30 days of life 140 (<110 - >170)
1-12 month 132 (<102 - >162)
1-2 years 124 (<94 - >154)
2-4 years 115 (<90 - >140)
4-6 years 106 (<86 - >126)
6-8 years 98 (<78 - >118)
8-10 years 88 (<60 - >108)
10 and older 80 (<60 - >100)
< - bradycardia, > - tachycardia.
 Percentile distribution of heart rate in age group from 0 to 18 years old according
to ECGscreening datas (M. Shcolnikova, D. Yegorova, 2012)
age percentile
2 5 50 95 98
0-7 days 100 110 139 170 180
1-2 weeks 115 121 153 186 200
3-5 months 106 114 136 171 183
6-12 months 99 105 127 170 185
1-2 years 80 92 123 188 195
2-3 years 74 88 112 150 171
3-4 years 76 83 101 128 149
4-5 years 76 80 98 118 125
5-6 years 71 74 92 115 127
6-7 years 70 71 92 115 120
7-8 years 65 70 88 114 125
8-9 years 62 66 87 114 125
9-10 years 60 65 83 109 115
10-11 years 60 53 81 108 117
11-12 years 57 60 81 111 120
12-13 years 53 60 81 106 115
13-14 years 56 61 80 107 111
14-15 years 56 61 80 109 115
15-16 years 54 60 78 103 110
16-18 years 49 56 72 107 114
Paediatric arrhythmias account for approximately 55.1 per
100,000 patients evaluated in paediatric emergency departments.
Sinus tachycardia is by far the most commonly reported
arrhythmia,
supraventricular tachycardia (SVT) which represents about 13%,
bradycardia accounting for about 6% of all cases.
Reentral tachycardia is common in infants and children with
congenital heart disease (CHD).
Some arrhythmias in the early post operative period like
premature atrial contraction’s (PAC’s) and premature ventricular
beats (bigeminy) are usually transient and well tolerated.
Others like junctional ectopic tachycardia (JET) and atrial flutter
may cause significant hemodynamic instability and compromise
or even sudden cardiac death.
Tachycardia it is condition when the heart rate is higher than the
age normal rate by 10-60 %. It can be divided on:
- mild (1 degree) - HR elevated on 10 %;
- moderate (2 degree) - HR elevated on 20 – 40 %;
- severe (3 degree) - HR elevated on 40 – 60 %
Also it is classified on:
- sinus, supraventricular and ventricular;
- paroxysmal and non-paroxysmal;
- reciprocal and automatic (ectopic);
- congenital and acquired;
- primary (idiopathic) and secondary, observed in pathology of the
cardiovascular or other system.
Narrow complex tachycardia (Supraventricular Tachycardias)
refers to any arrhythmia that originates above or at the bundle of
His. These are by far the most common tachycardias seen in
children, with an estimated incidence of 0.1–0.4%.
Three mechanisms of tachycardia have been described. In the
most common, re-entry tachycardia, the circuit can originate
within the atrium producing atrial flutter or fibrillation, at the
level of the AV node (AV node re-entry tachycardia), or via an
accessory pathway (AV re-entry tachycardia (AVRT)).

A second mechanism is automatic tachycardia, which results

from an enhanced automatic focus. This can be within the
sinus node as in sinus tachycardia or in foci elsewhere in the
atria.

It can be distinguished from SVT by the presence of a normal

sinus P wave preceding every QRS complex. Also SVT can be
with abnormal Pwave. Sinus tachycardia rates can exceed 140
bpm in children and 180 bpm in infants but are usually less
than 200 bpm.
 Paroxysmal supraventricular tachycardia
Paroxysmal supraventricular tachycardia (paroxysmal SVT) is
an episodic condition with an abrupt onset and termination, and
may last anywhere from a few minutes to several hours, which
is why it is called paroxysmal.
Tachycardia refers to any heart rhythm where the heart beats
faster than normal for age. Supraventricular tachycardia ranges
in heart rate from 160 to 300 beats per minute.
Paroxysmal supraventricular tachycardia (PSVT) is the most
common symptomatic arrhythmia in young patients. It affects
children of all ages.
Supraventricular means “above the ventricles,” thus describing
any tachycardia that originates and/or involves structures above
the ventricles of the heart.
 Etiology of SVT

• SVT and paroxysmal SVT are triggered by a reentry

mechanism. This may be induced by premature atrial or
ventricular ectopic beats. Other triggers include
hyperthyroidism and stimulants, including caffeine, drugs, and
alcohol.
• Paroxysmal SVT is observed not only in healthy individuals; it
is also common in patients with mitral valve prolapse,
rheumatic heart disease, pericarditis, pneumonia, chronic lung
disease, and current alcohol intoxication. Digoxin toxicity also
may be associated with paroxysmal SVT.
 Classification of tachycardias

Tachycardias

Narrow Wide
complex complex
QRS QRS

AV reentry
(AVRT), Atrial Ventricular
AV Node re- tachycardia tachycardia
entry (AVNRT)
 SVT

SVT, involving atrial tissue SVT, involving sinoatrial

tissue

Sinus nodal
SVT, involving AV node
re-entrant
Atrial tachycardia tachycardia

AV re-entrant
tachycardia Inappropriate sinus
Atrial flutter tachycardia

Multifocal atrial AV nodal re-

tachycardia entrant tachycardia Sinus tachycardia

Permanent junctional
Atrial fibrillation reciprocating
tachyarrhythmia
 SV tachyarrhythmias involving atrial tissue:
• Multifocal atrial tachycardia is a tachyarrhythmia that arises
within the atrial tissue; it is composed of 3 or more P-wave
morphologies and heart rates. This arrhythmia is fairly uncommon;
it is typically observed in elderly patients with pulmonary disease.
The heart rate is greater than 100 bpm, and electrocardiographic
findings typically include an irregular rhythm, which may be
misinterpreted as atrial fibrillation;

Multifocal atrial tachycardia. Note the different P-wave morphologies and irregularly irregular ventricular response.
• Atrial tachycardia is a supraventricular tachycardia (SVT) that
does not require the atrioventricular (AV) junction, accessory
pathways, or ventricular tissue for its initiation and maintenance. It
is an arrhythmia originating in the atrial myocardium. It occurs in
persons with normal hearts and in those with structurally abnormal
hearts, including individuals with congenital heart disease
(particularly after surgery for repair or correction of congenital or
valvular heart disease). Enhanced automaticity, triggered activity, or
reentry may result in this rare tachycardia. The heart rate is regular
and is usually 120-250 bpm. The P-wave morphology is different
from the sinus P waves
and is dependent on
the site of origin of the
tachycardia.
This 12-lead electrocardiogram demonstrates an atrial tachycardia at a rate of approximately 150 beats per minute. Note
that the negative P waves in leads III and aVF (upright arrows) are different from the sinus beats (downward arrows).

Atrial tachycardia. The patient's heart rate is 151 bpm. P waves are upright in lead V1.
• Atrial flutter is a tachyarrhythmia
arising above the AV node with an
atrial rate of 250-350 bpm. The
mechanism behind atrial flutter is
generally reentrant in nature.
Typically, counterclockwise atrial
flutter is due to a macroreentrant
The patient's heart rate is approximately 135 bpm with 2:1
right atrial circuit; conduction. Note the sawtooth pattern formed by the flutter
waves.

• Atrial fibrillation is an extremely

common arrhythmia arising from
chaotic atrial depolarization. The
atrial rate is usually 300-600 bpm,
while the ventricular rate may be
170 bpm or more.
Electrocardiographic findings
characteristically include an
irregular rhythm with fibrillatory The patient's ventricular rate varies from 130-168 bpm. The
rhythm is irregularly irregular. P waves are not discernible.
atrial activity.
SV tachyarrhythmias involving
sinoatrial tissue:
• Sinus tachycardia is the most
common regular SVT. It has an
accelerated sinus rate that is a
physiologic response to a stressor.
It is characterized by a heart rate
Sinus tachycardia. Note that the QRS complexes are narrow
faster than 100 beats per minute and regular. The patient's heart rate is approximately 135
bpm. P waves are normal in morphology.
(bpm) and generally involves a
regular rhythm with p waves before
all QRS complexes. Underlying
physiologic stresses such as
hypoxia, hypovolemia, fever,
anxiety, pain, hyperthyroidism, and
exercise usually induce sinus
tachycardia;
• Inappropriate sinus tachycardia (IST) - healthy adults may have an
elevated resting heart rate and an exaggerated heart rate response to even
minimal exercise. This tachyarrhythmia is observed most commonly in
young women without structural heart disease;

• Sinus nodal reentrant tachycardia (SNRT) is due to a reentry circuit,

either in or near the sinus node. Therefore, it has an abrupt onset and
offset. The heart rate is usually 100-150 bpm, and electrocardiographic
tracings usually demonstrate a normal sinus P wave morphology;
Sinus nodal reentrant tachycardia (SNRT)
SVTs involving the atrioventricular node:
• AV nodal reentrant tachycardia (AVNRT)
it is one of the common causes of
paroxysmal SVT. AVNRT is diagnosed in
50-60% of patients who present with
regular narrow QRS tachyarrhythmia and is
often in people older than 20 years. The
The patient's heart rate is approximately 146 bpm
heart rate is 120-250 bpm and is typically with a normal axis. Note the pseudo S waves in leads
II, III, and aVF. Also note the pseudo R' waves in V1
quite regular. AVNRT may occur in healthy, and aVR. These deflections represent retrograde atrial
activation.
young individuals, and it occurs most
commonly in women. Most patients do not
have structural heart disease. However,
occasionally these individuals may have an
underlying heart condition such as
rheumatic heart disease, pericarditis,
myocardial infarction, mitral valve
prolapse, or preexcitation syndrome; Same patient as in the previous image. The patient is
in sinus rhythm following atrioventricular nodal
reentrant tachycardia.
• AV reentrant tachycardia (AVRT) is
another common form of paroxysmal
SVT. The incidence rate of AVRT in the
general population is 0.1-0.3%. AVRT
is more common in males than in
females (male-to-female ratio of 2:1),
and patients with AVRT commonly
present at a younger age. Commonly
associated with Wolff- Parkinson-
White. Also it is associated with the
Ebstein anomaly, although most
patients with AVRT do not have
evidence of structural heart disease.
AVRT results from the presence of 2 or
more conducting pathways;
specifically, the AV node and 1 or more
bypass tracts. In AVRT, 1 or more
accessory pathways connect the atria
and the ventricles. The accessory
pathways may conduct impulses in an Orthodromic atrioventricular reentrant tachycardia. This
anterograde manner, a retrograde patient has Wolff-Parkinson-White syndrome.
manner, or both.
Permanent junctional reciprocating tachyarrhythmia (PJRT) is a rare
form of supraventricular tachycardia. These are reentrant circuits in which
one limb includes the AV node. It is a subtype of AVRT. The heart rates in
PJRT can range from 200 to 300 in infancy and later on decreases from 250
in early childhood to 120 in adults. It is one of the causes of refractory SVT
in infants and children.
1.RP interval is longer than PR interval (because of the location and decremental
conduction properties of the AP)
2.1:1 AV ratio (no dissociation)
3.Inverted P wave are often visible in the inferior leads, II, III, and AVF
4.Does not require a critically timed extrasystole for initiation
5.Not associated with frequent premature atrial contractions
6.PR interval is never prolonged
7.The rates are typically slightly slower than typical SVT (can be confused with
sinus tachycardia)
8.Response to vagal maneuvers with gradual slowing of the tachycardia because
of prolongation of both RP and PR intervals and eventual termination but with
recurrence shortly afterwards
9.The AV conduction is usually sensitive to adenosine with tachycardia
terminating with AV or VA block but again recurring shortly afterwards
10.QRS is normal and narrow during sinus rhythm and in tachycardia.
PJRT can be difficult to treat and refractory to medical management. The
combination therapy with flecainide and amiodarone has been shown to be
slightly more successful in controlling tachycardia and reversing cardiomyopathy.
Ablation is generally indicated when there is difficulty in medical management
and some feel that it needs to be attempted early in PJRT after appropriate
electrophysiologic mapping.
Wolff-Parkinson-White Syndrome (WPW): Baseline resting ECG is
characterized by a short PR interval, wide QRS and delta wave which is a
manifestation of the accessory on sinus rhythm. WPW is marked by the delta
wave on the resting ECG. Atrial flutter or atrial fibrillation in the presence of
this type of accessory connection can result in VF.
 Ventricular tachycardia (VT) or ventricular fibrillation (VF) refers to any rhythm faster
than 100 (or 120) beats/min, with 3 or more irregular beats in a row, arising distal to the
bundle of His. The rhythm may arise from working ventricular myocardium, the distal
conduction system, or both. Wide QRS complex morphology and a different QRS
morphology than the usual QRS waveform characterize VT. Morphology may be
monomorphic (uniform), polymorphic (multiform). Often associated with structural heart
disease, particularly late (years) after repair. Other common clinical situations in which one
might see VT include dilated and
hypertrophic cardiomyopathy,
metabolic alterations including severe
hypoxia, acidosis, hyper/hypokalemia,
and drug toxicity such as cocaine,
digoxin, and tri-cyclic antidepressants.
Other conditions include myocarditis
and long Q-T syndrome.

ECG shows rapid monomorphic ventricular tachycardia (VT), 280 beats/min, associated with hemodynamic
collapse.
• Junctional ectopic tachycardia (JET) it is type of arrhythmia when the ectopic
focus initiates at or near the AV node. JET is usually caused by surgery around
the AV node and rates often range between 160 beats/min to as high as 280
beats/min. Characteristics include inverted P- waves in lead II and an R-P interval
which is short or absent. Primarily seen post re- warming from cardiopulmonary
bypass and within 3 days of the surgery.
• Nonparoxysmal junctional tachycardia (NPJT) are rare; they
presumably arise because of increased automaticity, triggered activity, or
both. They are usually observed following valvular surgery, after
myocardial infarction, during active rheumatic carditis, or with digoxin
toxicity. These tachycardias are also observed in children following
congenital heart surgery. Electrocardiographic findings include a regular
narrow QRS complex, although P waves may not be visible.
Automatic ectopic tachycardia (AET) local enhanced automatic focus of
certain cardiac myocytes in the atria or AV node. When this occurs at an
ectopic site within the atria, it is called atrial ectopic tachycardia. AET
occurs as a result of irritation of tissues during cardiac surgery, with
placement of intracardiac lines, application of sutures, or cutting tissue. Any
reason for dilated atria, cardiomyopathy or disease AV valves, ventricular
dysfunction can result in this rhythm disorder. It is due to enhanced
automacity of single or multiple foci outside the sinus node and is often
refractory to medical therapy and cardioversion. Rates are usually above
170-180 beats/min and beyond 200 beats/min. A block at the AV node can
cause AV dissociation, further contributing to hemodynamic instability in
addition to the rapid atrial rate. The rhythm may be variable, and may be
interspersed with periods of sinus rhythm. The rate can ramp up or slow
down over minutes.
 Morbidity and mortality
• Patients with symptomatic WPW syndrome have a small risk of
sudden death. Paroxysmal SVT may start suddenly and last
anywhere from seconds to days. Patients may or may not be
symptomatic, depending on their hemodynamic reserve, heart rate,
the duration of the paroxysmal SVT, and coexisting diseases.
• Paroxysmal SVT can result in heart failure, pulmonary edema,
myocardial ischemia, and/or myocardial infarction secondary to an
increased heart rate in patients with poor left ventricular function.
• Patients with WPW syndrome may be at risk for cardiac arrest if
they develop atrial fibrillation or atrial flutter in the presence of a
rapidly conducting accessory pathway.
• In the absence of manifest preexcitation (ie, WPW syndrome), the
risk of sudden death with paroxysmal SVT is extremely small.
The following symptoms are typical (but not
always present) with a rapid pulse of 150-300
beats per minute:

• Pounding chest (palpitations);

• Shortness of breath;
• Chest pain / heavy chest;
• Rapid breathing;
• Dizziness;
• Loss of consciousness (in serious cases).
 Ectopic atrial tachycardia
Ectopic atrial tachycardia is uncommon, accounting for about 10% of SVT
in children.
Ectopic atrial tachycardia is the most common cause of tachycardia-
induced cardiomyopathy due to its persistent and chronic nature. The
precise etiology is unknown
Trigger factors:
§ viral illness,
§ atrial tumors,
§ genetic predisposition.
 Clinical features

•Is predominantly observed in infants and children with structurally

normal hearts.
•Patients present with symptoms of chest pain, palpitations, pre-syncopal
or syncopal events. Older children may present with exercise intolerance
or be asymptomatic.
•Infants may present with feeding difficulties, diaphoresis with feeds or
in respiratory distress secondary to chronic tachycardia.
 ECG-sings:
Abnormal P wave morphology and axis;
Atrial rate variable, ranging from 120 to 300 bpm;
May exhibit a “warm up” period at initiation with progressive P-P
interval shortening and a “cooling down” period prior to termination and
>1:1 AV conduction can be seen in patients at rest or while asleep
without termination of the tachycardia.
 Further work-up
• serum electrolytes, complete blood count, toxicology screen. If
cardiac function is poor, additional laboratory evaluation should
include viral panel, blood culture and cardiac enzymes to help
differentiate infection versus tachycardia-induced cardiomyopathy.

• Cardiology consultation and echocardiographic evaluation is

recommended.

• Obtain 24-hour cardiac monitor to determine duration of tachycardia

and ventricular rates.
 Acute management
• Responds poorly to adenosine and DC cardioversion.
• First line treatment for symptomatic patients includes IV amiodarone.
Load with 5mg/kg bolus over 20–60 minutes.
• Can be followed by amiodarone maintenance drip at 10–15mg/kg/day.
• Minimally symptomatic patients do not require acute treatment.

Further treatment:

Children younger than 1 year and symptomatic patients should be admitted

for management of arrhythmia and congestive heart failure.
In the setting of normal cardiac function, beta-blockers can be useful
by slowing AV node conduction, lowering the ventricular rate and improving
symptoms.
More aggressive treatment consists of flecainide, amiodarone or sotalol
which have moderate success rates.
Patients who fail medical therapy can undergo catheter ablation.
 Prognosis
Spontaneous resolution may be observed in 30–50% of affected
children.

Children younger than 3 years have higher incidence of AET resolution

with treatment versus older children who are likely to require ablation
because of persistent tachycardia despite optimal medical management.
 Presentation of PSVT in infants
• A newborn may present with history of fetal tachycardia or with
signs of left ventricular dysfunction from tachycardia in fetal life.
Hydrops fetalis represents severe heart failure from persistent
rapid tachycardia.
• Neonates with no history of fetal tachycardia may present with
incessant and difficult to treat pathological tachycardia after birth.
PSVT may occur in the first days of life or somewhat later in the
first year of life.
• Most infants with PSVT have structurally normal hearts. In 15%
of infants, paroxysmal tachycardia is associated with heart disease,
drug administration or a febrile illness.
• Incessant tachycardia at a rate of more than 200/min leads to
progressive left ventricular dysfunction. Persistent very rapid
tachycardia at a rate of 250/min or more may produce life
threatening heart failure within few days.
 Symptoms of PSVT in infants

• Irritability, fussiness or lethargy.

• Poor feeding,
• Tachypnea,
• Diaphoresis,
• Poor color;

It is symptoms of tachycardia masquerade those of many other

common illnesses in infancy. It is important to advise parents to
check infant’s heart rate when the child is quiet or asleep. Parents
may verify heart rate at the time of feeding by placing hand on
the child’s chest or with stethoscope.
• In infants atrio-ventricular reentry tachycardia (AVRT)
resulting from the presence of an accessory atrio-
ventricular (A-V) bypass tract accounts for
approximately 80% of all cases of PSVT.
• Permanent junctional reciprocating tachycardia (PJRT)
and automatic atria tachycardia are much less common.
• Atrial flutter may occur in newborns and usually
resolves within few days or with administration of
digoxin.
• When tachycardia is recognized, it is important to
obtain an echocardiogram in order to rule out structural
heart disease and to assess ventricular function
 Presentation of PSVT in children and adolescents
• palpitations,
• chest pain,
• shortness of breath,
• dizziness,
• syncope or near syncope,
• pallor,
• and diaphoresis.
Patients may describe that the heart is fluttering in the throat.
In a healthy child, supraventricular tachycardia usually presents with
palpitations but rarely, it may present as syncope or near syncope. This may
occur in patients with WPW who develop atrial fibrillation and rapid
conduction down the accessory pathway to the ventricles. Syncope is unusual
and may indicate life threatening arrhythmia which necessitates rapid
diagnosis and treatment. The onset of SVT can also cause a decrease in
cardiac output with resultant hypotension, decreased cerebral perfusion
pressure, and syncope.
 Diagnosis

Making an diagnosis can sometimes be difficult, especially when

there is no dcocumented record of the tachycardia.
The diagnosis of PSVT is based on thorough clinical history and
electrocardiograms (ECG’s) or rhythm strips recorded at the time of
tachycardia and during sinus rhythm. It is very important to
document prolonged attacks of arrhythmia so that their physician can
make a proper diagnosis. An ECG has to be repeated after
tachycardia resolves. Usually, most supraventricular tachycardias
have a narrow QRS complex on ECG. Rarely, supraventricular
tachycardia with aberrant conduction can produce a wide-complex
tachycardia that can mimic ventricular tachycardia, such as
antidromic AVRT.
An exercise stress test may induce tachycardia and allow for its
documentation.
Ambulatory Holter monitoring may be successful in recording
paroxysmal tachycardia in patients with frequent recurrences of
PSVT.
Holter is not likely to document PSVT in patients with very
infrequent bouts of tachycardia although it may show intermittent
ventricular preexcitation pointing to WPW syndrome.
It is important to determine whether a wide-complex tachycardia
is an SVT or a ventricular tachycardia, since they are treated
differently. Ventricular tachycardia has to be treated
appropriately, since it can quickly degenerate to ventricular
fibrillation and death.
 Acute treatment
Regardless of the type of PSVT there are certain general principles of
therapy which apply to all patients. Physicians should teach their
patients maneuvers which increase parasympathetic (vagal) tone,
slow down conduction through the A-V node and break the reentry
circuit responsible for PSVT in most cases.
An ice-bag can be used at any age with good success. It is important
to place an ice-bag around the nose and mouth in order to make it
effective. Immersion of the face in ice-cold water may also terminate
PSVT.
In the Valsalva maneuver patients are asked to take a deep breath and
bear down. Valsalva maneuver may be combined with other measures
such as an ice-bag or pressure on the abdomen.
Carotid sinus massage, carried out by firmly pressing the bulb at
the top of one of the carotid arteries in the neck, is effective but is
often not recommended due to risks of stroke in those with
plaque in the carotid arteries.
Applying pressure on the eyes with closed eyelids must never be
used because of a significant risk of injury to the eyes and retinal
detachment.
Acute treatment in the emergency room depends on patient’s symptoms.
Children with unstable vital signs or signs of heart failure require electric
cardioversion. Stable vital signs and normal blood pressure allow for less
aggressive measures such as those described above.
Intravenous administration of adenosine as a rapid bolus is safe in children
of all ages. Adenosine, an ultrashort acting AV nodal blocking agent, is
indicated if vagal maneuvers are not effective. Adenosine is quickly cleared
from circulation by cellular uptake, mostly by endothelial cells and
erythrocytes. The half life in the blood stream is less than 10 seconds. The
standard dose ranges from 50 to 200 micrograms per kilogram of body
weight.
The most commonly observed side effects are transient chest
discomfort, dyspnea, facial flushing, sinus pauses and A-V block.
Significant bradycardia may occur in patients with sinus node
dysfunction. Adenosine may produce bronchoconstriction in patients
with asthma.
If adenosine does not work, atrioventricular (AV) nodal blocking
agents like calcium channel blockers or beta-blockers should be
used.
Calcium channel blockers should not be administered to children
under 2 years of age because of a risk of cardiovascular collapse.
Patients with symptomatic Wolff-Parkinson-White (WPW)
syndrome should not be treated with calcium channel blockers or
digoxin unless the pathway is known to be of low risk (long
anterograde refractory period). This is because of the potential for
rapid ventricular rates should atrial fibrillation or atrial flutter
occur, which can result in cardiac arrest.
Patients with preexcited atrial fibrillation should not be treated
with intravenous AV nodal blocking agents, such as adenosine,
beta-blockers, calcium channel blockers, and digoxin. If the
patient is hemodynamically stable, intravenous procainamide
should be administered. If the patient is unstable, direct current
cardioversion should be performed.
 Chronic management
Chronic management of PSVT should be individualized. In general, the
decision to treat a child with PSVT depends on the child’s age, symptoms
produced by paroxysmal tachycardia and the effect of PSVT on the quality of
life.
Prophylactic treatment with antiarrhythmic medications is prescribed for
infants younger than one year of age because of nonspecific nature of
symptoms and the risk of life-threatening heart failure from unrelenting rapid
tachycardia. Beta blockers are prescribed most frequently. Digoxin may be
used in infants without ventricular preexcitation.
At one year of age, prophylactic treatment could be withheld in order to see
if tachycardia recurs. More than one half of toddlers with history of PSVT
after birth will be free from tachycardia at least until school age when
children are able to recognize symptoms of arrhythmia. Prophylactic
treatment with medications may be extended into preschool years if
tachycardia recurs or parents are unable to recognize recurrent arrhythmia.
In school-aged children, the decision to treat PSVT usually depends
on its effect on the quality of life. Infrequent PSVT accompanied by
mild symptoms does not warrant any treatment especially if a child is
able to interrupt tachycardia with appropriate maneuvers.
Consumption of caffeine (coffee, tea, soft drinks containing caffeine)
may increase the likelihood of recurrent tachycardia. Patients report
more problems with PSVT if they take betaadrenergic medications
(salbutamol) or certain decongestants (pseudoephedrine,
antihistamines).
It is crucial to treat all patients with WPW syndrome who present
with syncope or who participate in competitive sports. This
recommendation also applies to isolated ventricular preexcitation
with no history of paroxysmal tachycardia. It is important to
remember that prophylactic treatment with medications does not cure
the child from tachycardia but merely suppresses arrhythmia.
 Electrical cardioversion

If the patient is unstable or other treatments have not been

effective, cardioversion may be used, and is almost always
effective.

Catheter Ablation Procedures

Elimination of the arrhythmic substrate and permanent cure from

almost all forms of PSVT can be achieved through percutaneous
catheter ablation or by means of surgery. Catheter ablation
procedures are performed in an electrophysiology laboratory and
are similar to cardiac catheterization with the use of fluoroscopy.
Radiofrequency current (RF) ablation is employed most frequently.
 Prevention and cure
Once the acute episode has been terminated, ongoing treatment may be
indicated to prevent a recurrence of the arrhythmia. Patients who have a
single isolated episode, or infrequent and minimally symptomatic episodes
usually do not warrant any aggressive or invasive treatment except
observation.
Patients who have more frequent or disabling symptoms from their episodes
generally warrant some form of preventative therapy. A variety of drugs
including simple AV nodal blocking agents like beta-blockers and verapamil,
as well as anti-arrhythmics may be used, usually with good effect, although
the risks of these therapies need to be weighed against the potential benefits
and side effects.
For most tachycardia caused by a re-entrant pathway, radiofrequency ablation
is probably the best option. This is a low risk procedure that uses a catheter
inside the heart to deliver radio frequency energy to locate and destroy the
abnormal electrical pathways. Ablation has been shown to be highly effective
- up to 98% effective.
 Atrial flutter
In children, atrial flutter usually occurs in fetal life or shortly following
birth. It accounts for about 30% of fetal tachycardia, 18% of tachycardias in
newborn and only 8% in older children.
Atrial flutter is a reentrant arrhythmia circuit confined to the atrial
chambers. As a rule, atrial flutter originates in the right atrium. Atrial flutter
involves a single reentry circuit within the atrial muscle, most commonly
around the borders of the tricuspid valve. Hemodynamic compromise is
determined by the duration of flutter and the degree of AV block, with 1:1
AV conduction resulting in the most significant instability.
 Etiology
Most fetuses and neonates with atrial flutter have structurally normal hearts.

Conditions, which are associated with atrial flutter:

• Congenital heart disease (such as Ebstein anomaly of the tricuspid valve,

AV septal defects);
• post-operative patients (Fontan procedures, atrial septal closure and
tetralogy of Fallot (TOF) repair);
• Some newborns and young children have associated conditions or
anomalies (atrial septal aneurysms, restrictive cardiomyopathies);
• Surgery-induced inflammation of the pericardium, scarring, and volume
overload;
• Atrial flutter have been related to obesity, alcohol consumption, and
hyperthyroidism.
 Clinical Presentation
In fetuses is diagnosed in-
utero during fetal ultrasound
evaluation;
• Fetal hydrops common if
prolonged;
In newborn and infants:
• Most newborns are asymptomatic
unless tachycardic>48hours;
• Infants with prolonged
tachycardia may present with
history of poor feeding, irritability,
lethargy, diaphoresis and pallor.
In older children:
• Regular or irregular palpitation;
• Syncope, severe presyncope;
• Weakness or fatigue;
• Low energy levels;
• Pounding, pain or pressure in the
chest
• Shortness of breath;
• Fainting or dizziness.
 Physical Examination
• Physical examination in patients with atrial flutter should assess
the likely conduction ratio and rate of flutter and assess for signs
of associated ventricular dysfunction or heart failure. Depending
on the ventricular rate and the individual's tolerance to that rate,
symptoms may range from palpitations, dyspnea, presyncope, or
syncope to sudden death.
• If the ventricular response is rapid, atrial flutter may cause
significant morbidity secondary to hemodynamic deterioration due
to low cardiac output.
• If the ventricular response is slow enough to permit a sustained
arrhythmia, atrial thrombosis with consequent thromboembolism
may result. In patients who have undergone surgery for congenital
heart disease, new onset of atrial arrhythmias such as atrial flutter
may indicate elevated right atrial pressure and, thus, the need for
surgery.
 Complications

• Low cardiac output;

• Brain and other end-organ injury;
• Sudden or subacute death;
• Heart failure;
• Thrombosis and thromboembolism.
 Workup
ECG-sings:
• Regular atrial rates of 240–360 bpm;
• AV ratio >1:1, variable but generally regular, ventricular rate 120–240;
• Typical sawtooth appearance may be seen in lead II, III and aVF and
• Normal appearing QRS complex;
• Irregularly irregular R-R intervals

Rhythm strip depicting lead II of a patient with atrial flutter

with an atrial rate of 300 beats per minute (bpm).
Atrioventricular conduction rate is variable at 2:1 and 3:1.
Therefore, the ventricular rate ranges from 100-150 bpm.
• Electrophysiology (EP) study — a small, thin catheter
(or wire) is inserted through a vein into the heart. This
will allow doctors to locate the sites that are causing
the arrhythmias.
• Stress test — a test that shows how the heart is
functioning during exercise.
• Heart monitors — these are small monitors that are
worn by the child from anywhere from 24 hours to
one month. They are used to detect any abnormal
heart rhythms.
 Acute management

•Vagal maneuvers or adenosine do not convert this rhythm but may

increase the degree of AV block, unmasking underlying flutter waves.

•If patient has rapid ventricular response with reduced cardiac output, or
for elective cardioversion, use DC cardioversion starting at 0.5J/kg,
increasing to 1–2J/kg if needed.

•If stable, pharmacotherapy can be aimed at rhythm control with IV

amiodarone or ibutilide, or at rate control with beta-blockers or calcium-
channel blockers.

•In patients with pacemaker, pace termination can be attempted by the

cardiologist.
• Fetal atrial flutter is the second most common intrauterine
tachyarrhythmia. Treatment is aimed at controlling ventricular rate
and, thus, avoiding hydrops fetalis. First-line treatment is digoxin
administered to the mother, which provides a conversion rate to
sinus rhythm of 45-52%. In addition, its positive inotropic effect
may be beneficial.

• Sotalol has also been used in numerous cases with success. Maternal
drug levels were not reliable predictors of successful therapy.
Flecainide alone or in combination with digoxin is used as second-
line treatment. Fetal atrial flutter in a structurally normal heart
seldom recurs after conversion before or after birth, and postnatal
suppressive antiarrhythmic therapy may not be necessary.

• Diltiazem can provide rapid, consistent, and safe temporary

ventricular rate control in children.
 Treatment
Medical care should be broadly directed at the following:
• Ensuring hemodynamic stability before, during, and after
conversion to sinus rhythm;
• Minimizing influences favoring initiation or maintenance of atrial
arrhythmias (e.g., electrolyte disturbances, pericardial effusion,
indwelling atrial lines or catheters);
• Excluding or managing complications (e.g., ventricular
dysfunction, thromboembolic phenomena);
• Restoring.
Drug therapy of atrial flutter in children can be classified under
the 3 broad headings of ventricular rate control, acute conversion,
and chronic suppression.
• Digoxin is relatively safe for preventing rapid conduction of atrial flutter
via the atrioventricular (AV) node to the ventricles, and some evidence
indicates that this reduces symptomatology during flutter.
• Nevertheless, digoxin is unlikely to be particularly effective in the acute
conversion or prevention of atrial flutter recurrence. It is devoid of
negative inotropic effects (as is amiodarone) and is useful to control
ventricular rate when using propafenone, flecainide, or procainamide.

Intravenous procainamide has been used with variable success to effect

acute conversion of atrial flutter to sinus rhythm. Procainamide infusion
should be preceded by digitalization to prevent procainamide-induced
acceleration of AV node conduction to the ventricles.

The Vaughan Williams class III agents may be used for acute
conversion of atrial flutter and fibrillation. This drugs more effective
than other medications in converting atrial flutter.
 Doses of digoxin in children
Use doses at the lower end of the spectrm when treating heart failure
Reduce dose by 20-25% when changing from oral formulation or IM to IV therapy
• Premature neonate: PO: 1st loading dose, 10-15 mcg/kg; 2nd and 3rd loading doses, 5-7.5 mcg/kg q6-8hr for 2
doses; maintenance: 5-7.5 mcg/kg/day divided q12hr; IV/IM: 1st loading dose, 7.5-12.5 mcg/kg; 2nd and 3rd
loading doses, 3.75-6.25 mcg/kg q6-8hr for 2 doses; maintenance: 4-6 mcg/kg/day divided q12hr
• Full-term neonate: PO: 1st loading dose, 12.5-17.5 mcg/kg; 2nd and 3rd loading doses, 6.25-8.75 mcg/kg q6-8hr for
2 doses; maintenance: 6-10 mcg/kg/day divided q12hr; IV/IM: 1st loading dose, 10-15 mcg/kg; 2nd and 3rd loading
doses, 5-7.5 mcg/kg q6-8hr for 2 doses; maintenance: 5-8 mcg/kg/day divided q12hr
• Infants & children 1-24 months: PO: 1st loading dose, 17.5-30 mcg/kg; 2nd and 3rd loading doses, 8.75-15 mcg/kg
q6-8hr for 2 doses; maintenance: 10-15 mcg/kg/day divided q12hr; IV/IM: 1st loading dose, 15-25 mcg/kg; 2nd and
3rd loading doses, 7.5-12.5 mcg/kg q6-8hr for 2 doses; maintenance: 7.5-12 mcg/kg/day divided q12hr
• 2-5 years: PO: 1st loading dose, 15-20 mcg/kg; 2nd and 3rd loading doses, 8.75-10 mcg/kg q6-8hr for 2 doses;
maintenance: 7.5-10 mcg/kg/day divided q12hr; IV/IM: 1st loading dose, 12.5-17.5 mcg/kg; 2nd and 3rd loading
doses, 6.25-8.75 mcg/kg q6-8hr for 2 doses; maintenance: 6-9 mcg/kg/day divided q12hr
• 5-10 years: PO: 1st loading dose, 10-17.5 mcg/kg; 2nd and 3rd loading doses, 5-8.75 mcg/kg q6-8hr for 2 doses;
maintenance: 5-10 mcg/kg/day divided q12hr; IV/IM: 1st loading dose, 7.5-15 mcg/kg; 2nd and 3rd loading doses,
3.75-7.5 mcg/kg q6-8hr for 2 doses; maintenance: 4-8 mcg/kg/day divided q12hr
• >10 years & <100 kg: PO: 1st loading dose, 5-7.5 mcg/kg; 2nd and 3rd loading doses, 2.5-3.75 mcg/kg q6-8hr for 2
doses; maintenance: 2.5-5 mcg/kg/day; IV/IM: 1st loading dose, 4-6 mcg/kg; 2nd and 3rd loading doses, 2-3
mcg/kg q6-8hr for 2 doses; maintenance: 2-3 mcg/kg/day
 Doses of amiodarone in children

Drug Resistant Refractory Cardiac Arrhythmias (Off-label)

PO: Age <1 year: 600-800 mg/1.73 m² q24hr or divided q12hr; continue therapy for 4-14 days and/or
until adequate control achieved; if initial treatment effective, decrease dosage to 200-400 mg/1.73 m²
q24hr or divided q12hr
Age >1 year: Until adequate control, 10-15 mg/kg/day PO qDay or divided q12hr; if effective, reduce to
5 mg/kg/day PO qDay or divided q12hr: IV Loading dose (limited data): 5 mg/kg IV over 30-60 min:
Maintenance dose: 0.005 mg/kg/min IV infusion; may increase to 20 mcg/kg/min per 24 hr; consider
converting to oral therapy within 24-48 hr
Pulseless Ventricular Tachycardia or Ventricular Fibrillation (PALS dosing) (Off-label)
5 mg/kg IV/IO rapid bolus; not to exceed 300 mg/dose; may repeat twice to maximum 15 mg/kg during
acute treatment
Supraventricular Tachycardia (Off-label)
Infants/children/adolescents: 5 mg/kg IV over 1 hr initially; follow with 5 mg/kg/day for 47
hr; Maintenance: 10-20 mg/kg/day for 7-10 days; follow with 3-20 mg/kg/day
 Doses of procainamide in children
• IM Administration: 20-30 mg/kg/day IM divided q6hr; not to
exceed 4 g/day;
• IV Administration: Loading dose: 3-6 mg/kg IV over 5
minutes, not to exceed 100 mg/dose;
may repeat q5-10min PRN not to exceed 15 mg/kg/dose
Maintenance: 0.02-0.08 mg/kg/min IV infusion; not to exceed 2 g/24 hours
• To convert from IV to PO: Total mg/24 hr IV dose divided into 4 daily SR doses, round
to dosage form
Adjust dose to patient's response

Doses of diltiazem in children

1.5-2 mg/kg/day PO divided q8hr; not to exceed 6 mg/kg/day, up to 360 mg/day.

Doses of sotalol in children

Supraventricular Tachycardia
<2 years old: Reduce dose; see manufacturer's package insert for details
≥2 years old: 30 mg/m² PO q8hr initially; may be titrated up to 180 mg/m²/day PO
Ventricular Arrhythmias (Orphan)
Orphan designation for treatment of life-threatening ventricular arrhythmias in pediatric
patients
• Cardioversion is used if medication is not effective. In this
procedure, an electric shock is delivered to the heart in an
attempt to convert the heartbeat to a normal rhythm. This is
performed under mild sedation.
• Catheter ablation is rarely used in children. A small, flexible
catheter (or tube) is inserted into a vein or artery and is gently
guided into the heart. The source of the arrhythmia is located
and the tissue that is sending the multiple signals is then
destroyed. Patients are usually able to leave the hospital within
24 hours. Some studies show possible links to heredity, while
others attribute childhood AF to congenital heart abnormalities
or post-surgical complications.
 Prognosis
Morbidity and mortality in patients with atrial flutter largely depend on the
following factors:
1) Age at presentation: The fetus with atrial flutter may
have significant morbidity and be at risk for mortality. Mortality in
newborns with atrial flutter is uncommon. Most patients remain in sinus
rhythm following their initial conversion, and the need for antiarrhythmic
prophylaxis in these patients during infancy is discussed. In patients with
postoperative atrial flutter that develops late following repair of congenital
heart disease, the severity of presentation depends on the atrial flutter rate,
conduction ratio, and presence of ventricular dysfunction.
2) Cardiac anatomy (normal anatomy vs congenital heart disease);
3) Integrity and anatomy of the myocardial conduction system (normal
sinus node vs sinus node dysfunction; AV block vs normal AV node, with or
without accessory pathways);
4) Ventricular function;
5) Prompt recognition of the arrhythmia and initiation of adequate therapy.
 Further work-up and disposition

•Laboratory work-up: serum electrolytes and thyroid function tests;

•Cardiology consultation and echocardiogram to rule underlying
structural heart disease, tachycardia-mediated cardiomyopathy and
thrombus;
•Admit for observation;
•Anticoagulation therapy for episodes >48 hours and in all Fontan
patients.
Atrial fibrillation (also called AFib or AF) is mostly seen in
children with underlying structural heart disease and in those who have
undergone cardiac surgery. This arrhythmia is very rare in healthy
children.
Atrial fibrillation demonstrates a rapid atrial rate (300-500 beats per
minute) with a very chaotic pattern, and an irregularly irregular
ventricular rhythm. It is associated with next conditions:
cardiomyopathies,
myocarditis,
pericarditis,
hyperthyroidism,
WPW-syndrome,
genetic predisposition.
 Clinical features

• Children and adolescents present with complaints of palpitations.

Weakness and signs of congestive heart failure may be seen.
• In patients presenting with syncope, WPW should be highly
suspected.

Electrocardiogram findings
• Chaotic/irregular atrial waves best seen in lead V1;
•Atrial rates of 350–600 bpm;
• No discrete, uniform P waves;
•AV ratio >1:1;
•Variable, changing ventricular response rate (irregularly irregular),
ranging from 110 to 200 bpm and
• Normal appearing QRS complex, except in WPW.
 Acute management

• If the patient has rapid ventricular response with reduced cardiac

output, DC cardioversion starting at 2J/kg is used.
• For stable patients, pharmacotherapy aimed at ventricular rhythm or
rate control can be used. IV rhythm control agents include
amiodarone and ibutilide. Less effective are sotalol, digoxin and
procainimide.
• Cardioversion in stable patients with unknown or prolonged (>48
hours) duration of tachycardia should be delayed until assessment
for atrial thrombus is made with echocardiography.
 Further work-up and disposition
• Once in normal sinus rhythm, repeat EKG.
• Laboratory work-up: serum electrolytes, thyroid function, complete
blood count, toxicology screen. If cardiomyopathy is suspected,
additional laboratory evaluation should include viral panel, blood
culture and cardiac enzymes.
• Echocardiography is indicated in all cases.
• Admit for observation and treatment.
• Initiate anticoagulation therapy in most cases.

Prognosis
Despite medical therapy, atrial fibrillation has a high recurrence rate
and often requires catheter or surgical intervention. Chronic
anticoagulation therapy is indicated in patients with persistent or
recurrent atrial fibrillation. Ablation of the AV node with implantation
of a pacemaker may be necessary in some refractory cases.
 Ventricular tachycardia
is a potentially life-threatening arrhythmia recognized as a cause of
sudden death in both adults and pediatrics. It is rare in children and
accounts for about 6% of patients followed for tachycardias. Defined as
a tachycardia originating below the bundle of His, rates can range from
just over the sinus rate to well over 200 bpm. Episodes lasting less than
30 seconds are termed as non-sustained VT and those more than 30
seconds as sustained VT. VT can further be classified as monomorphic,
with a regular rate and a single QRS morphology, versus polymorphic,
with variability in rate and QRS morphology. The same basic
mechanisms of automacity, reentry and triggered tachycardia exist in
VT as for other arrhythmias.

Etiology: idiopathic, drug toxicity, cardiomyopathy, myocarditis,

cardiac tumors, metabolic abnormalities.
Clinical features Electrocardiogram findings
• Dizziness, • Hallmarks are prolonged QRS duration
• Palpitations, for age and VA dissociation with
• syncope, ventricular rates exceeding atrial rates
• cardiac arrest. (AV ratio < 1:1).
• Rates range from ~110 to >200 bpm.
• When VA conduction is 1:1, VT cannot
be excluded. Features consistent with the
diagnosis of VT rather than SVT with a
wide QRS include variation in RR
interval and presence of fusion
complexes.
• Complexes may appear uniform or vary
from beat to beat as in polymorphic VT.
Acute management Prognosis
• If unstable, synchronized In the setting of a structurally normal
cardioversionis started at heart and stable, well-tolerated
2J/kg and repeated, monomorphic VT, many minimally
increasing the dose if symptomatic patients can be followed
needed. closely without therapy. Such patients
• If stable, may attempt may benefit from B-blockers to reduce
amiodarone at 5mg/kg ectopy. Chronic treatment otherwise
IV over 30–60 minutes depends on rate, duration, symptoms,
or procainimide at type of VT and the presence of genetic
15mg/kg IV over 30–60 channelopathy such as long QT.
minutes. Aggressive antiarrhythmic therapy
usually in addition to implantable
cardioverterdefribrillator (ICD)
placement is required for life-
threatening VT.
 Further work-up
• History should focus on prior symptoms, symptoms suggestive of
myocarditis or long-standing cardiomyopathy, and the possibility of drug
toxicity, as well as a thorough family history for known arrhythmias or
history of sudden death.
• Once in normal sinus rhythm, repeat EKG to rule out underlying
abnormalities including long QT, Brugada, arrhythmogenic right ventricular
cardiomyopathy, structural heart disease, electrolyte abnormalities and
ischemia.
• Laboratory work-up should include toxicology screen, serum electrolytes,
complete blood count, viral panel, blood culture and cardiac enzymes.
• Cardiac consultation and echocardiographic evaluation are done to rule
underlying structural heart disease, cardiomyopathy, cardiac tumors.
• Admit for observation.
• After cardioversion, return to sinus rhythm may be transient and continual
infusion of amiodarone may be required.
 Long QT syndrome

The congenital long QT syndrome is a genetic disorder of prolonged cardiac

repolarization that may cause cardiac arrest and sudden death. Abnormalities
in cardiac ion channels predispose patients to a characteristic polymorphic
VT called “torsades de pointes”. Events are often precipitated by adrenergic
stimuli such as exercise, especially swimming, emotional stress, exposure to
loud noises or even sleep. Acquired long QT may also occur and can be
caused by drugs, underlying medical conditions or electrolyte imbalances.

Clinical features

• Patients may present with presyncope, syncope, seizures, or cardiac

arrest.
• Although rare, infants can present with poor feeding, or with episodes of
lethargy, cyanosis or poor perfusion.
 Electrocardiogram findings
• Sinus rhythm ECG, QTc of >460 in post-pubertal females and 450 in
others, best obtained from lead II (Bazett Formula QTc= QT Interval/√-
RR).
• Borderline QTc>440 ms in the setting of clinical symptoms and/or family
history should be investigated.
• Abnormal T wave morphology including notching and low amplitude.
• Torsade de pointes seen during events.

Acute management
• For torsades de pointes, perform emergent defibrillation followed by
administration of magnesium sulfate and possibly lidocaine.
• Correct underlying problem if acquired long QT.
• Intravenous beta-blockade may calm an adrenergic storm.
 Further work-up and management in
suspected congenital long QT syndrome
• Obtain thorough family history of rhythm abnormalities, sudden death,
deafness.
• Find out all medications.
• Review history of event that may have triggered arrhythmia.
• Obtain electrolytes and treat underlying abnormalities.
• If presented with symptoms or documented VT, admit for observation,
cardiology consultation and treatment.
• For patients presenting with non-cardiac issues and noted to have
abnormal QTc interval, out-patient cardiology follow-up may be arranged.
• Limit any physical activity until a follow-up cardiac examination.
• Provide list to the patients of medications known to prolong QT that
should be avoided.
• Immediate family members should also be screened with 12-lead EKGs.
 Prognosis

Prognosis is poor in untreated symptomatic patients, with an annual

mortality of 20%.
B-blockers are the mainstream therapy and reduce risk of sudden death
to about 6% annually but do not eliminate it completely.
High risk patients may benefit from ICD placement which has been
shown to reduce mortality risk.
Factors known to increase risk include:
- history of previous syncope,
- deafness,
- previous torsade,
- female gender and genotype.
Premature beats or extra beats most often cause irregular
heart rhythms.

The two most common types of ectopic heartbeats are:

Premature ventricular contractions (PVC) - start in the
ventricles;
Premature atrial contractions (PAC) - start in the upper
chambers (atria).

Causes:
Sometimes ectopic heartbeats are seen with:
Changes in the blood, such as a low potassium level (hypokalemia)
Decrease in blood supply to the heart
Heart muscle disease (cardiomyopathy)
Ectopic beats may be caused or made worse by smoking, alcohol
use, caffeine, stimulant medicines, and some street drugs.
Premature beats are very common in normal children
and teenagers - most people have them at some time.
Usually no cause can be found and no special treatment
is needed. The premature beats may disappear later
without any treatment.
Symptoms include:
Feeling your heart beat (palpitations);
Feeling like your heart stopped or skipped a beat;
Feeling of occasional, forceful beats.
Note: There may be no symptoms.
 Signs of Premature Atrial Contraction
(atrial extrasystole) on ECG
1) Premature appearance of P wave and QRS complex;
2) Negative P wave in standart leads;
3) P-R interval more frequenly is shortened but may be normal and
prolonged;
4) QRS complex is of normal form (or aberrant);
5) Compensatory pause incomplete (under usual QRS complex
configuration).
 Causes of PVCs
• Cardiac acute ischemia;
• Myocarditis;
• Cardiomyopathy dilated or hypertrophic;
• Myocardial contusion
• Mitral valve prolapse;
• Hypoxia and/or hypercapnia;
• Medications (e.g., digoxin, sympathomimetics, tricyclic
antidepressants, aminophylline, caffeine)
• Illicit substances (e.g., cocaine, amphetamines, alcohol,
tobacco)
• Hypomagnesemia;
• Hypokalemia;
• Hypercalcemia.
 Signs of ventricular extrasystole (premature
ventricular contractions (PVCs) on ESG

PVCs are characterized by premature and bizarrely shaped QRS

complexes usually wider than 120 msec on with the width of the
ECG. These complexes are not preceded by a P wave, and the T wave
is usually large, and its direction is opposite the major deflection of
the QRS. Widening and deformation of the QRS complex. Changing
of ST-T interval.
 Clinical Presentation of PVCs
The clinical significance of PVCs depends on their frequency,
complexity, and hemodynamic response.
PVCs in young, healthy patients without underlying structural heart
disease are usually not associated with any increased rate of mortality.

• Patients are usually asymptomatic.

• Cannon A waves or the increased force of contraction due to
postextrasystolic potentiation of contractility can cause palpitations
and neck and/or chest discomfort.
• The patient may report feeling that his or her heart "stops" after a
PVC.
• Patients with frequent PVCs or bigeminy may report syncope.
This symptom is due to either inadequate stroke volume or
decreased cardiac output caused by the condition effectively
halving the heart rate.
• Long runs of PVCs can result in hypotension.
• Exercise can increase or decrease the PVC rate.
• The ectopic beat may produce a diminished or absent pulse
depending on the force of the ventricular contraction.
• Neurologic findings: Agitation and findings of sympathetic
activation (e.g., dilated pupils, warm and dry skin, tremor,
tachycardia, hypertension) suggest that catecholamines may be
the cause of the ectopy.
 Laboratory Studies
• Obtain serum electrolyte levels, in particular potassium levels.
Consider checking the magnesium level, especially in patients with
low potassium levels.
• In selected patients, a drug screen may be helpful.
• For patients taking medication with known proarrhythmic effects
(e.g., digoxin, theophylline), drug levels may be useful.
ECG findings may include the following:
• Left ventricular hypertrophy;
• Active cardiac ischemia (ST-segment depression or elevation and
or T-wave inversion);
• In patients with previous MI - Q waves or loss of R waves, bundle
branch block;
• Electrolyte abnormalities (hyperacute T waves, QT prolongation);
• Drug effects (QRS widening, QT prolongation);
On ECG, PVCs may be premature in relation to the next expected beat of
the basic rhythm.
The pause after the premature beat is usually a fully compensatory pause.
The R-R interval surrounding the premature beat is equal to double the basic
R-R interval, showing that the ectopic beat did not reset the sinus node.
PVCs may appear in a pattern of bigeminy, trigeminy, or quadrigeminy,
which describe a pattern of PVCs occurring every other, every third, or
every fourth beat, respectively.
PVCs with identical morphologies on a tracing are called monomorphic or
unifocal. If the PVCs demonstrate 2 or more different morphologies, they
are referred to as multiform, pleomorphic, or polymorphic.

bigeminy

quadrigeminy
• PVCs usually are described in terms of the Lown
grading system for premature beats. The higher
the grade, the more serious the ectopy.
• Grade 0 = No premature beats;
• Grade 1 = Occasional (< 30/h);
• Grade 2 = Frequent (>30/h);
• Grade 3 = Multiform;
• Grade 4 = Repetitive (A = Couplets, B = Salvos
of = or > 3);
• Grade 5 = R-on-T pattern.
• Holter 24-hour monitors are useful in quantifying and
characterizing ventricular ectopy. Holters also have
been used to determine treatment efficacy in patients
with frequent or complex PVCs. More than 60% of
healthy, middle-aged men have ventricular ectopy on
Holter monitoring.
• Echocardiography is useful not only in evaluating the
ejection fraction, which is important in determining the
prognosis and also in identifying valvular disease or
ventricular hypertrophy.
 Treatment
The decision to treat premature ventricular contractions (PVCs) in the emergency or
outpatient settings depends on the clinical scenario. In the absence of cardiac disease,
isolated, asymptomatic ventricular ectopy, regardless of configuration or frequency,
requires no treatment.
Correct electrolyte imbalances, particularly those of magnesium, calcium, and
potassium.
Hypoxia: Treat the underlying cause; secure the ABCs and provide oxygen. Drug
toxicity: Specific therapy is indicated for certain toxic effects. First-line therapy for
ectopy without hemodynamic significance in patients post-MI is beta-blockade.
Only in the setting of symptomatic, complex ectopy is lidocaine likely to benefit.
Lidocaine is especially useful when symptomatic ectopy is associated with a
prolonged QT interval, as it does not lengthen the QT interval as other antiarrhythmic
agents do.
Amiodarone is also a useful agent to suppress ectopy/VT if hemodynamically
significant. Additional beneficial effects include coronary vasodilation and increased
cardiac output via a reduction in systemic vascular resistance.
 Treatment

The following may help reduce ectopic heartbeats for some

people:
- Limiting caffeine, alcohol and tobacco;
- Regular exercise for people who are inactive

Most ectopic heartbeats do not need to be treated. The condition is

only treated if your symptoms are severe or if the extra beats occur
very often. The cause of the heartbeats, if it can be found, may
also need to be treated.
 BRADYCARDIAS
Bradycardias are a group of disorders that include sinus node dysfunction
and abnormal conduction through the AV node. AV conduction
abnormalities include first-, second- or third-degree heart block.
Sinus bradycardia can result from multiple disease states, most of which are
not primary cardiac such as: increased intracranial pressure, electrolyte
abnormalities,
respiratory compromise,
Hypothyroidism,
certain medications.

The exception is sinus node dysfunction, an abnormality of impulse

generation and sinus node conduction usually caused in pediatrics by direct
injury or disruption of blood supply to the node from previous cardiac
surgery. Associated atrial reentry tachycardias are common and when they
occur, the term “brady-tachy syndrome” is applied.
The need for acute treatment for sinus bradycardia itself is rare. The
presence of sinus bradycardia in an otherwise healthy appearing child is
generally of no concern, although anorexia nervosa should be
considered. In the symptomatic patient with sinus bradycardia, urgent
determination of the underlying cause is very importance. Atropine and
epinephrine increases sinus rates in most patients. Symptomatic patients
with true sinus node dysfunction and/or those with coexistent
tachycardias are likely to require elective pacemaker implantation.
Atrioventricular Block occurs when atrial
depolarizations fail to reach the ventricles or when atrial
depolarization is conducted with a delay.
Three degrees of AV block are recognized.

First-degree AV block consists of

prolongation of the PR interval on
the electrocardiogram (ECG) (>
200 msec in adults and > 160 msec
in young children).
First-degree atrioventricular block. PR
interval is constant and is 280 msec.
Second-degree atrioventricular (AV) block occurs when
regular atrial systoles (i.e., non-premature atrial systoles)
intermittently fail to conduct to the ventricles.
Second-degree AV block may occur in many different patterns, and
the block may be physiologically located at any level between the
atria and ventricles. Second-degree AV block is not a disease in and
of itself; it is a pattern of cardiac rhythm that is most typically
recognized on electrocardiogram (ECG). On the basis of certain
ECG characteristics, second-degree AV block is divided into
Mobitz I AV block (or Wenckebach AV block) and Mobitz II AV
block (or non-Wenckebach AV block). These classifications have
clinical and prognostic implications.
In second-degree AV block, the QRS has a normal duration, unless
baseline bundle branch block is also present.
 Mobitz I (Wenckebach) AV block
The most common pattern of second-degree AV block consists of gradual
prolongation of the PR interval leading up to a nonconducted P wave.
Mobitz I (Wenckebach) AV block may occur normally in the presence of
increased vagal tone, such as in athletes or during sleep. However, a small
number of patients may have AV node disease or a progressive conduction
system disorder. Mobitz I AV block is also pathologic when it occurs during
exercise. An episode of Mobitz I AV block usually consists of 3-5 beats,
with a ratio of nonconducted to conducted beats of 4:3, 3:2, and so forth (P-
wave:QRS complexes).

P-wave

This rhythm strip is an example of classic Mobitz I, or Wenckebach, AV block, in which the PR interval
prolongs by sequentially smaller increments, with consequent shortening of the RR intervals until the
blocked beat occurs.
 Mobitz II (non-Wenckebach) AV block
The second form is Mobitz II second-degree AV block, which is
characterized by a constant PR interval followed by sudden failure of
a P wave to be conducted to the ventricles, so that either an
occasional dropped P wave or a regular conduction pattern of 2:1 (2
conducted and 1 blocked), 3:1 (3 conducted and 1 blocked), and so
on is observed. This block is usually located more distally in the His
bundle or bundle branches, or both, and the escape rates are usually
slower and less stable than in Mobitz I block.

P-wave
 Complete/third-degree AVB
Third-degree AV block is diagnosed when no supraventricular impulses are
conducted to the ventricles. P waves on the rhythm strip reflect a sinus node
rhythm independent from QRS wave complexes. The QRS complexes
represent an escape rhythm, either junctional or ventricular. The escape
rhythm originating from the junctional or high septal region is characterized
by narrow QRS complexes at a rate of 40-50 beats/min, whereas escape
rhythm from low ventricular sites is characterized by broad QRS complexes
at a rate of 30-40 beats/min.
No relationship exists between the rhythm of P waves and the rhythm of
QRS complexes in third-degree AV block. The frequency of P waves (atrial
rate) is higher than the frequency of QRS complexes (ventricular rate).
 Etiology
First-degree AV block and Mobitz I (Wenckebach) second-degree AV block may
occur in healthy, well-conditioned people as a physiologic manifestation of high
vagal tone. Mobitz I AV block also may occur physiologically at high heart rates
(especially with pacing) as a result of increased refractoriness of the AVN, which
protects against conducting an accelerated arrhythmia to the ventricles.

The causes of second-degree AV block are:

- autoimmune or inflammatory conditions, such as neonatal lupus erythematosus,
myocarditis, endocarditis, Lyme disease, and rheumatic fever.
- Toxicity from drugs or other substances (excessive doses of digoxin, beta
blockers, calcium channel blockers, and Vaughan Williams class III agents) .
- Hyperthyroidism (e.g., thyrotoxicosis, which is sometimes exacerbated by beta-
blocker
therapy) or hypothyroidism, either primary or drug-induced.
- Congenital long QT syndrome.
- Surgical procedures (e.g., aortic valve replacement and congenital defect repair).
Other therapeutic procedures (eg, AV node ablation and alcohol septal ablation in
patients with obstructive hypertrophic cardiomyopathy).
 Etiology
Causes of complete atrioventricular (AV) block (AVB):
- Postsurgical complete atrioventricular (AV) block (AVB) is the most common cause for
acquired AV block in children, resulting from trauma to the AV node at the time of
surgery (ie, hemorrhage, ischemia, necrosis, inflammation, traumatic disruption).
- Systemic infections that cause myocardial inflammation and infiltration, such as
diphtheria, Borrelia burgdorferi infection (Lyme disease), Chagas disease, Rocky
Mountain spotted fever, Yersinia enterocolitica infection, infectious mononucleosis,
bacterial endocarditis, and viral myocarditis.
- Inflammatory and coronary disease: Rheumatic fever, Reiter syndrome, Sarcoidosis,
Coronary ischemia and degeneration of the conducting system.
- Neuromuscular and neurocutaneous disease: Myotonic dystrophy forms 1 and 2, Kearns-
Sayre syndrome, Tuberous sclerosis.
- Degenerative disease (2 degenerative diseases of the specialized conducting system: Lev
disease and Lenègre disease).
- Intracardiac tumor.
- Abnormal protein deposition: amyloidosis.
- Drugs, chest trauma, and irradiation.
 Clinical Presentation
• First-degree atrioventricular (AV) block is generally not associated with
any symptoms and is usually an incidental finding on
electrocardiography (ECG).
• Second-degree AV block usually is asymptomatic. However, in some
patients, sensed irregularities of the heartbeat, presyncope, or syncope
may occur.
• Third-degree AV block frequently is associated with symptoms such as
fatigue, dizziness, exercise intolerance, failure to thrive (in infants), and
congestive heart failure, presyncope, and syncope most commonly.
Typically, patients with complete AV block have bradycardia. Syncopal
episodes due to slow heart rates are called Morgagni-Adams-Stokes
(MAS) episodes. The clinical presentation of newborns with complete
congenital AV block may range from asymptomatic presentation with
heart rates increasing to 100 bpm upon stimulation (eg, when feeding,
crying) to hydropic presentation due to congestive heart failure, to
stillborn and hydropic presentation.
 Morgagni-Adams-Stokes Syndrome

Sudden collapse into unconsciousness due to a disorder

of heart rhythm in which there is a slow or absent pulse
resulting in syncope (fainting) with or without
convulsions. Once the heart stops beating, loss of
oxygen to the brain (cerebral anoxia) results in fainting.
Convulsions may occur if the heart stops for longer than
15 seconds. In rare cases, the attendant oxygen loss may
cause permanent brain damage.
Clinical sings: dizziness, heart rates about 20-30 b/min,
syncope, convulsions.
 Treatment
Long-term medical therapy is not indicated in atrioventricular
(AV) block. Pacemaker implantation is a routine surgical
procedure. Asymptomatic patients with Wenckebach or non-
Wenckebach AV block do not require immediate pacemaker
therapy. However, they should be monitored periodically. In
symptomatic patients, initiation of short-term therapy with a
chronotropic agent, such as isoproterenol and atropine, may be
helpful.
 Emergent treatment of Morgagni-Adams-
Stokes Syndrome

1. Closed-chest cardiac massage.

2. Mouth-to-mouth resuscitation.
3. Endocardiac administration: 0,1% solution of adrenaline and
0,1% solution of atropin (0,05 ml/1 year of life + 10% of
calcium chloride (3-5 ml/1 year of life).
4. Cardiac stimulation.
5. In short episodes of asystolia isoproterenol 0,0025-0,005 g
sublingual, IV 0,2% solution of noradrenaline 0,5-1,0 ml +
200 ml solution of glucose (10-12 drops/min with ECG
control).
 Isoproterenol

• Adams-Stokes Attacks (Off-label)

• Initial: 0.1 mcg/kg/min IV infusion
• Usual dose: 0.1-1 mcg/kg/min IV infusion
• Cardiac Arrest (Off-label)
• Initial: 0.1 mcg/kg/min IV infusion
• Usual dose: 0.2 to 1 mcg/kg/min
• Heart Block (Off-label)
• Initial: 0.1 mcg/kg/min IV infusion
• Usual dose: 0.3 to 1 mcg/kg/min IV infusion
• Postoperative Cardiac Patients with Bradycardia
• IV infusion: 0.029 mcg/kg/min
 Atropine

Sialorrhea, Pylorospasm & Other Spastic Conditions of the

Gastrointestinal Tract
• 3-7 kg: 0.1 mg
• 8-11 kg: 0.15 mg
• 11-18 kg: 0.2 mg
• 18-29 kg: 0.3 mg
• ≥30 kg: 0.4 mg
Alternate Dosing
• <5 kg: 0.02 mg/kg/dose initially, then q4-6hr PRN
• ≥5 kg: 0.01-0.02 mg/kg PO q4-6hr PRN; not less than 0.1
mg/dose, not to exceed 0.4 mg/dose
CARDIOMYOPATHIES

Patients at increased risk of malignant VT and sudden death.

Risk factors: family history of sudden death, history of
syncope, dramatic septal thickness, non-sustained VT and
inappropriate blood pressure response to exercise.

Patients at risk of ventricular tachycardia, supraventricular

tachycardia. High-degree AV block are not uncommon.

Is a genetic myopathy involving fibrofatty degeneration of the right ventricle

and is commonly associated with VT and sudden death. Inheritance is mainly
autosomal dominant but sporadic cases have been described. Peak age of
presentation is late teenage years to the 20s, and a common presenting
symptom is syncope with exercise.
Congenital heart disease and associated
arrhythmias
Congenital heart disease
Tetralogy of Fallot
Double outlet right ventricle
Transposition of the great arteries
Ebstein's anomaly
Ventricular septal defect repair
Atrialseptal defect
Atrial septal defect repair
Associated arrhythmia
Atrial tachycardia
Ventricular tachycardia
Sinus node dysfunction
Ventricular arrhythmias
Atrioventricular block
Supraventricular tachycardia
Heart block
Ventricular arrhythmias
Atrial tachycardia
Sinus node dysfunction