Pediatric Arrhythmias

Debra Hanisch, RN, MSN, CPNP

During the past decade, our awareness and understanding of arrhythmias in children has expanded immensely. This report
discusses the more commonly encountered pediatric rhythm disturbances, including sinus node dysfunction, the various forms
of supraventricular tachycardia, ventricular tachycardia, long QT syndrome, and the atrioventricular blocks. The electrocardiographic characteristics, electrophysiological mechanisms, clinical presentation, and current acute and chronic management
options for each are described.
Copyright 2001 by W.B. Saunders Company

ORMAL CONDUCTION IS characterized by

an impulse that is initiated by the sinoatrial
(SA) node, located in the right atrium near the
superior vena cava junction, propagated through
the cardiac conduction system across the atria,
converging at the atrioventricular (AV) node, proceeding down the His bundle to the right and left
bundle branches, and finally spreading throughout the Purkinje fibers to depolarize the ventricles
(Figure 1). Characteristic electrocardiographic
(ECG) findings of normal sinus rhythm include
upright P-waves and QRS complexes in leads I and
aVF. Age-appropriate parameters for heart rate, PR
interval, and QRS duration are presented in Table
1 (Liebman, 1982).
The majority of children who are diagnosed with
arrhythmias have structurally normal hearts. For
children with complex congenital heart disease,
advances in surgical and medical management
have resulted in improved survival. However,
these children may be at risk for developing early
and late postoperative arrhythmias. This report discusses the more common pediatric arrhythmias
found in both structurally normal hearts and in
patients with congenital heart disease. ECG characteristics, electrophysiological mechanisms, clinical presentation, and acute and chronic management options for each rhythm abnormality are
described.

age (Table 2). Sinus pauses, sinus arrest, and exit

block produce electrical pauses in the rhythm.
Junctional escape rhythms occur when the atrial
rate slows to the point where the AV nodes automaticity takes over. Slow heart rates may predispose the heart to certain tachyarrhythmias, such as
atrial flutter.
Typically, SND occurs as a result of surgical
injury to the sinoatrial node or its arterial supply.
Surgical procedures associated with a higher incidence of SND include the Mustard or Senning
procedures for d-transposition of the great arteries,
the Fontan procedure for single ventricle physiology, closure of atrial septal defects, and repair of
total anomalous pulmonary venous return. However, whenever cannulation for cardiopulmonary
bypass is done near the superior vena cavaright
atrial junction, there is a risk for SND.
Nonsurgical causes of SND include right atrial
dilation due to pressure or volume overload. SND
may be seen in cardiomyopathies or inflammatory
conditions, such as myocarditis, pericarditis, and
rheumatic fever. Increased vagal tone and certain
drugs, especially antiarrhythmic agents, may result
in SND as well. In anorexia nervosa, resting heart
rates tend to be 20 beats/min slower than in
matched controls (Panagiotopoulos, McCrindle,

SINUS NODE DYSFUNCTION

From the Lucile Packard Childrens Hospital at Stanford,

Palo Alto, CA.
Address reprint requests to Debra Hanisch, RN, MSN,
CPNP, Pediatric Cardiology, Lucile Packard Childrens Hospital at Stanford, 750 Welch Rd., Suite #305, Palo Alto, CA
94304.
Copyright 2001 by W.B. Saunders Company
0882-5963/01/1605-0008$35.00/0
doi:10.1053/jpdn.2001.26571

Sinus node dysfunction (SND) encompasses a

number of arrhythmias including sinus bradycardia, sinus pauses or arrest, sinoatrial exit block,
escape rhythms, and brady-tachy syndromes. On
the ECG, sinus bradycardia has the appearance of
sinus rhythm, but at a slower rate than expected for
Journal of Pediatric Nursing, Vol 16, No 5 (October), 2001

351

352

DEBRA HANISCH

Figure 1.
system.

Hick, & Katzman, 2000). Conditioned athletes also

have slower-than-average resting heart rates. Modified criteria are used to diagnose SND in these
latter two populations.
Sinus node dysfunction may occur at any age.
As a surgical complication, SND may occur immediately after surgery or may not manifest for up
to 10 years or longer after surgery. Most children
with SND are asymptomatic. Of those who are
symptomatic, the most common symptoms appear
to be fatigue, exercise intolerance, dizziness, and
syncope. Sudden death is rare.
Table 1. Normal Heart Rates, PR Intervals, and QRS Durations
in Children
Heart Rate (bpm)
Age

Range

PR Interval
(ms)

QRS Duration
(ms)

1 day
1-7 days
3-30 days
1-3 months
3-6 months
6-12 months
1-3 years
3-5 years
5-8 years
8-12 years
12-16 years

119
133
163
154
140
140
126
98
96
79
75

94-145
100-175
115-190
124-190
111-179
112-177
98-163
65-132
70-115
55-107
55-102

70-120
70-120
70-110
70-130
70-130
80-130
80-150
90-150
100-160
100-170
110-160

50-84
40-79
40-73
50-80
60-80
50-80
50-80
60-84
50-80
50-84
40-80

Adapted and reprinted with permission (Liebman, 1982).

Normal conduction

Acute management of the bradycardic child with

hemodynamic instability includes adequate ventilation, oxygenation, and administration of epinephrine (0.01 mg/kg) or, if the bradycardia is vagally
mediated, atropine (0.02 mg/kg; minimum dose 0.1
mg). If there is no response to these medications,
temporary pacing should be instituted (Hazinski,
Cummins, & Field, 2000).
For long-term management, permanent pacemaker therapy for SND is indicated when symptoms are associated with bradycardia or chronotropic incompetence (Gregoratos et al., 1998).
Pacing may also be warranted in individuals with
brady-tachy syndrome, in which slowing of the
heart rate may mediate the onset of tachycardia. In
this situation, antibradycardia pacing either alone
or in combination with antitachycardia pacing may
be used. Prophylactic permanent pacing is a consideration for patients with SND who are placed on
antiarrhythmic agents that prolong the QT interval,
such as amiodarone, to prevent further slowing of
the heart rate and/or pause-dependent torsades de
pointes (Martin & Kugler, 1999).
SUPRAVENTRICULAR TACHYCARDIA
Supraventricular tachycardia (SVT) refers to a
sustained tachyarrhythmia that originates above

PEDIATRIC ARRHYTHMIAS

353
Table 2. Sinus Bradycardia

ECG Criteriaa

24-Hour Ambulatory ECG Criteriaa

Age Group

Heart Rate

Age Group

Infants to 3 years
Children 3-9 years
Children 9-16 years
Adolescents 16 years

100 bpm
60 bpm
50 bpm
40 bpm

Infants to 1 year of age

Children 1-6 years
Children 7-11 years
Adolescents, young adults
Highly trained athletes

aBased

Heart Rate

60
60
45
40
30

bpm sleeping; 80 bpm awake

bpm
bpm
bpm
bpm

on data from Kugler, JD (Kugler, 1990).

the bundle of His. SVT is the most common abnormal tachyarrhythmia in children, with an estimated incidence in the pediatric population of
0.1% to 0.4% (Ludomirsky & Garson, 1990). The
rhythm typically appears with an abrupt onset as a
regular, narrow QRS tachycardia. Rates vary from
130 to 300 beats/min depending on the patients
age and the SVT mechanism. On the ECG, the
QRS complex is usually normal in configuration,
but in less than 10% of cases the QRS is wide due
to aberrant conduction to the ventricles (Ludomirsky & Garson, 1990).
Several SVT mechanisms have been identified.

Figure 2.

The more common ones are described here (Figure

2). The vast majority of SVT rhythms are either
due to a re-entrant circuit, with or without an
accessory pathway, or an automatic ectopic focus.
Triggered activity accounts for a very small percentage of SVT.
Atrioventricular reciprocating tachycardia
(AVRT) is a common type of SVT that relies on at
least two electrical connections between the atria
and ventricles: the AV node and an accessory
pathway (more than one accessory pathway may
be present). Conduction proceeds antegrade down
one pathway and retrograde up the other to form a

Common mechanisms of SVT.

354

DEBRA HANISCH

re-entrant circuit. Nearly 75% of the SVT rhythms

in children are mediated by accessory pathways.
The most common form of AVRT is orthodromic
reciprocating tachycardia, which involves antegrade conduction down the AV node to the ventricles and retrograde conduction up the accessory
pathway to the atria. Antidromic reciprocating
tachycardia (down the accessory pathway, up the
AV node) occurs in less than 10% of patients.
Wolff-Parkinson-White (WPW) syndrome is a
frequently encountered type of AVRT. WPW is
characterized by a manifest pathway that is evident
on a 12-lead ECG during sinus rhythm. Pre-excitation of the ventricle through the accessory pathway (Kent bundle) appears as a delta wave, or
slurred upstroke into the QRS complex, along with
a shortened PR interval (Figure 3). There is an
increased incidence of WPW in patients with Ebsteins anomaly, tricuspid atresia, double-outlet
right ventricle, and hypertrophy cardiomyopathy
(Van Hare, 1999). Among patients with AVRT,
about half have a concealed accessory pathway,
meaning that pre-excitation is not evident on the
12-lead ECG (Deal, 1998).
The permanent form of junctional reciprocating
tachycardia (PJRT) is a type of AVRT in which
the retrograde conduction through the accessory
pathway is slow, producing retrograde (inverted)
P-waves with a longer RP interval than is seen
in other types of AVRT. This slow conduction
through the accessory pathway contributes to the
incessant nature of this form of SVT.
AV nodal re-entrant tachycardia (AVNRT) is
distinguished from AVRT in that, instead of an
accessory pathway, dual pathways exist within the
AV node. Typically, conduction proceeds antegrade down a slow pathway and retrograde up a
fast pathway to create a re-entrant circuit. On the
ECG, retrograde P-waves are buried within the
QRS complex. AVNRT accounts for nearly 15%
of SVT in the pediatric population, but rarely appears before the age of two years (Ko, Deal, Stras-

Figure 3.

burger, & Benson, 1992). AVNRT is not associated with congenital heart disease (Deal, 1998).
Intra-atrial re-entrant tachycardia (IART),
commonly referred to as atrial flutter, is a reentrant tachycardia that is confined to the atria.
Propagation of IART relies on an electrical pathway within the atria with both an area of slow
conduction and an anatomic obstruction that results in unidirectional block. This milieu for IART
exists after atrial surgery for congenital heart disease, such as the Mustard/Senning operation for
transposition of the great arteries, the Fontan procedure for single ventricle physiology, repair of
total anomalous pulmonary venous return, and
atrial septal defect closure. In fact, almost 95% of
atrial flutter diagnosed beyond infancy is associated with structural heart disease (Deal, 1998). On
ECG, characteristic saw-tooth waves (flutter
waves) are seen at rates of 200 to 400 bpm with
variable AV conduction (Figure 4).
Atrial ectopic tachycardia (AET) is a primary
atrial tachycardia that arises from an automatic
focus in the atria but outside the sinus node. Atrial
rates may range from 90 to 330 bpm with variable
AV block (Sokoloski, 1999). On ECG, distinct
P-waves are seen with a morphology that is different from the normal sinus P-wave. AET is present
in only a small percentage of children with SVT
but has proven to be quite resistant to medical
management. As a rapid, incessant tachycardia,
AET may lead to a dilated cardiomyopathy that is
usually reversible with successful abolition of the
automatic focus.
Junctional ectopic tachycardia (JET) is an automatic tachycardia that originates in the AV junction or His bundle. The ventricular rates generally
range from 150 to 300 bpm. The ECG may reveal
AV dissociation with the ventricular rate being
faster than the atrial rate (Figure 5). However, in
some cases, 1:1 VA conduction occurs, with the
retrograde P-wave superimposed on the QRS complex. JET is encountered more frequently as a

Wolff-Parkinson-White (WPW).

PEDIATRIC ARRHYTHMIAS

355

Figure 4.

Atrial flutter with 2:1 block.

postoperative arrhythmia. The heart rate may start

out more slowly but then rapidly increases within a
few hours after cardiopulmonary bypass. Hemodynamic compromise occurs as a result of the fast
heart rate and loss of the atrial contribution to
ventricular filling (atrial kick). If not adequately
controlled, JET is associated with a high mortality
in the postoperative patient. Infrequently, JET may
present as a congenital arrhythmia with a familial
predilection.
The relative incidence of these different types of
SVT varies with age (Table 3). AVRT has a relatively high incidence among newborns and infants,
with male patients affected more than female patients. In many of these infants SVT will spontaneously resolve by 6 to 12 months of age, but over
30% will have recurrence later in life (Perry &
Garson, 1990). AVNRT is virtually unseen in neonates but occurs more frequently with increasing
age. AVNRT represents over 50% of the SVT in
adults. The incidence of primary atrial tachycardias
remains fairly constant across all age groups.
Infants with SVT tend to present with nonspecific symptoms such as irritability, lethargy, poor
feeding and, after 24 to 48 hours, signs of conges-

Figure 5.

tive heart failure (CHF) (Table 4). In the presence

of associated congenital heart disease, CHF may
develop more quickly. In severe cases, hemodynamic compromise may occur and lead to respiratory distress, hypotension, and shock. The older
child with SVT may describe palpitations, a fast
heart rate, chest discomfort, or dizziness. In rare
instances, syncope or cardiac arrest may ensue.
Acute management of SVT depends on the patients age and condition. In the presence of shock
or cardiovascular collapse, immediate synchronized cardioversion with 0.5 to 1.0 joule/kg is
warranted. In the more stable patient, 0.1 to 0.2
mg/kg of adenosine may be administered intravenously by rapid bolus to induce transient AV block
(Hazinski et al., 2000). If the SVT is a re-entrant
tachycardia that uses the AV node as part of its
circuit, adenosine will break the tachycardia. If not,
adenosine may at least be helpful in unveiling the
tachycardia mechanism during the brief time AV
conduction is interrupted (Figure 4). IART responds to either DC cardioversion or atrial overdrive pacing. Automatic tachycardias (AET, JET)
do not respond to DC cardioversion or overdrive
pacing. Intravenous amiodarone has proven to be

Junctional ectopic tachycardia (JET).

356

DEBRA HANISCH
Table 3. Age-Related Incidence of SVTa
Age

AVRT

AVNRT

Primary Atrial Tachycardia

(IART, AET)

Prenatal/Neonate
Infant
1-5 years
6-10 years
Adolescent

80-85%
80%
65%
60%
65-70%

0%
5%
20-25%
30%
20%

15-20%
15%
10-15%
10-15%
15%

aBased

on data from Ko et al. (Ko et al., 1992).

the most effective agent for the acute management

of these challenging automatic tachycardias. For
JET, atrial or dual-chamber pacing at a rate above
the JET rate can improve hemodynamics by establishing AV synchrony. Maintaining normothermia,
or even mild hypothermia, may lower the JET rate
to facilitate pacing therapy. For the very stable
pediatric patient with SVT, vagal maneuvers may
be attempted initially to terminate the tachycardia,
such as placing a bag of ice water over the face to
elicit the diving reflex, inducing rectal stimulation
with a thermometer (infants), or performing a Valsalva maneuver (older child).
Chronic management of SVT also varies depending on the patients age, symptoms, frequency
of tachycardia episodes, presence of structural
heart disease, and risk for sudden death. Most SVT
in infants involves an accessory pathway and can
usually be controlled with oral digoxin or propranolol to slow conduction across the AV node. If the
SVT is refractory to these agents, flecainide, sotalol, or amiodarone may be effective but carry a
greater risk of adverse effects. Combinations of
drugs may be needed in some cases. More than
90% of infants diagnosed with SVT before two
months of age will have spontaneous resolution of
their arrhythmia by eight months of age (Perry &
Garson, 1990). These patients are usually weaned
off their antiarrhythmic medications after 6 to 12
months of therapy but are monitored for late re-

currence. In the older child with infrequent episodes and mild symptoms, a management option
may be to do nothing. For others with more problematic tachycardia, chronic antiarrhythmic drug
therapy may be indicated. Radiofrequency (RF)
catheter ablation becomes a reasonable therapeutic
option in the school-aged child or adolescent. RF
ablation is performed in the cardiac catheterization
or electrophysiology laboratory with percutaneously inserted electrode catheters to map the electrical pathways and an ablation catheter to create
strategically placed thermal lesions to interrupt impulse conduction. The success rate with RF ablation varies according to the type of SVT and institutional experience, but has been reported to be
83% to 96% (Kugler et al., 1994; Kugler, Danford,
Houston, & Felix, 1997). RF ablation has been
performed successfully in infants with SVT refractory to medical management but, because of their
small size, is associated with a much greater risk
for complications, such as AV block or perforation
of the heart.
VENTRICULAR TACHYCARDIA
Ventricular tachycardia (VT) is defined as three
or more consecutive premature ventricular complexes (PVCs) at a rate greater than 120 beats/min
but usually less than 250 beats/min. The QRS
complexes are typically wide with AV dissociation
or, in rare cases, 1:1 retrograde VA conduction. VT
may be nonsustained (lasting less than 10 seconds)
or sustained (lasting 10 seconds or longer). Monomorphic VT refers to tachycardia in which all the
QRS complexes have a similar morphology, in
contrast to polymorphic VT with multiform complexes. Torsades de pointes (twisting of the
points) is a type of polymorphic VT characterized
by rapid, wide, undulating QRS complexes that
appear to be spiraling around an axis (Figure 6).

Table 4. Differentiating Supraventricular Tachycardia From Sinus Tachycardia

History
Examination

ECG

Chest radiograph
Echocardiogram

Supraventricular Tachycardia

Sinus Tachycardia

Nonspecific; lethargy or irritability, poor

feeding, tachypnea, diaphoresis, pallor
Signs of congestive heart failure: tachypnea,
moist crackles, increased respiratory
effort, poor perfusion, hepatomegaly
Abrupt onset, heart rate 220 bpm,
regular R-R intervals, P-waves seen in
50-60% with abnormal axis, narrow
QRS in 90% of SVTs
May have an enlarged heart, signs of
pulmonary edema
May have ventricular dilation or dysfunction

Suggestive of volume loss, vomiting,

diarrhea, blood loss, or febrile illness
Consistent with dehydration, fever, or blood
loss; clear lungs, normal liver; may be
due to CHF in congenital heart disease
Gradual onset, heart rate usually less than
200 bpm, variable R-R intervals, normal
P-wave axis, narrow QRS

Adapted and reprinted with permission from Hanisch, D. (Hanisch & Perron, 1992).

Small or normal heart, clear lung fields

(unless congenital heart disease present)
Usually normal

PEDIATRIC ARRHYTHMIAS

357

Figure 6.

Torsades de pointes.

Mechanisms for VT are not as well described as

for SVT, but appear to include re-entry (similar to
IART), abnormal automaticity, and triggered activity.
The incidence of VT in the pediatric population
is unknown, but accounts for about 6% of patients
followed for tachyarrhythmia (Dick & Russell,
1998). The causes of VT in children are similar to
those for PVCs. VT may be associated with severe
electrolyte or metabolic abnormalities, hypoxia,
hypothermia, or drug toxicity. Cardiac conditions
such as cardiomyopathy, myocarditis, arrhythmogenic right ventricular dysplasia, and ventricular
tumors (rhabdomyomas, hamartomas) may create a
substrate for VT. Surgery for congenital heart disease, particularly procedures that involve a ventriculotomy (i.e., tetralogy of Fallot repair) have been
associated with an increased risk for early and late
postoperative VT. Another recognized cause of VT
is congenital or acquired long QT syndrome.
VT may present at any age and with varying
degrees of symptoms. Some children may present
with minimal symptoms despite their tachycardia;
however, most children will be symptomatic. Infants may be lethargic, tachypneic, and pale, and
may feed poorly. Mottling or cyanosis may be
present as well. Older children report palpitations,
chest discomfort, dizziness, nausea, or syncope. In
many instances, the child initially presents after
resuscitation from sudden cardiac death.
Acute management of VT in the unstable patient
should be focused on rapid termination of the
arrhythmia with synchronized cardioversion (0.5 to

Figure 7.

1.0 joules/kg) or, if pulseless, defibrillation (2 to 4

joules/kg) (Hazinski et al., 2000). Intravenous lidocaine, procainamide, or amiodarone may be
used to supress PVCs and further occurrences of
VT. Careful attention to maintaining normal electrolyte values is necessary in the postresuscitation
period. In extreme cases of uncontrollable incessant VT, extracorporeal membrane oxygenation
(ECMO) support or a ventricular assist device may
be required.
Chronic management options for VT are based
on the childs age and clinical condition. Antiarrhythmic drug therapy may be successful in suppressing ventricular ectopy in postoperative patients; however, the potential for proarrhythmia
and depression of ventricular function must be
recognized. An implantable pacemaker/cardioverter-defibrillator (ICD) device, with or without
concomitant drug therapy, is indicated for selected
patients with cardiomyopathy, long QT syndrome,
life-threatening VT, and resuscitated sudden cardiac death. RF ablation has been employed successfully in some patients with discrete arrhythmogenic foci (Silka & Garson, 1999). For others,
surgical intervention either cryoablation, revision of previous congenital heart surgery, removal
of a cardiac tumor, or cardiac transplantmay be
necessary (Dick & Russell, 1998).
LONG QT SYNDROME
Congenital long QT syndrome (LQTS) is an
inherited disorder that affects the ion channels in
the heart, resulting in abnormal ventricular repo-

Mobitz I AV block (Wenckebach).

358

DEBRA HANISCH

larization and an increased risk for life-threatening

arrhythmias. LQTS is characterized by prolongation of the QT interval on ECG, usually measuring
greater than 440 to 460 ms. The diagnosis of
LQTS, however, is not based solely on the presence of a prolonged QT interval, but on additional
ECG findings, clinical presentation, and family
history. Typically on ECG, abnormal T-wave morphology is present and may reflect the specific ion
channel that is affected. Patients with LQTS tend
to have abnormally low resting heart rates for their
age. A documented episode of torsades de pointes
lends further support to the diagnosis of LQTS.
Clinically, 60% of patients present with symptoms.
These symptoms include syncope or presyncope
(often associated with exercise, noise, or stress),
palpitations, seizure activity, and cardiac arrest.
Also, in 60% of cases, the family history is positive
for either a family member diagnosed with LQTS
or a sudden, unexplained premature death.
The incidence of LQTS is estimated to be 1 in
5,000 to 10,000, with a higher prevalence in female
patients in the adult age group, but a nearly equal
gender distribution in children. Male patients tend
to present at an earlier age and more often with
sudden cardiac death (Locati et al., 1998). Several
clinical associations have been identified with
LQTS, including congenital AV block (5%), congenital deafness (4.5%), congenital heart disease
(12%), and syndactyly (Garson et al., 1993; Marks,
Trippel, & Keating, 1995). Jervell and LangeNielson syndrome represents the autosomal recessive form of LQTS and is associated with congenital deafness. Romano-Ward syndrome refers to the
autosomal dominant form of LQTS. The specific
types of LQTS that have been identified are listed
in Table 5. Approximately 50% of patients have
LQT1, 45% have LQT2, and 5% have LQT3. The
remaining types are relatively rare.
Management of LQTS is individualized, with
consideration given to the patients age and risk
Table 5. Genes Associated With Low QT Syndrome
(Ackerman, 1998)
LQTS

Gene

Chromosome

Ion Channel

LQT1
LQT2
LQT3
LQT4
LQT5
LQT6
aJLN1
aJLN2

KVLQT1
HERG
SCN5A
Unknown
KCNE1 (MinK)
MiRPI
KVLQT1
KCNE1 (MinK)

11p15.5
7q35-36
3q21-24
4q25-27
21q22.1-22.2
21
11p15.5
21q22.1-22.2

K
K
Na
Unknown
K
K
K
K

Data from Ackerman, 1998.

aAssociated with congenital deafness.

factors. Typically, drug therapy with beta blocking

agents (propranolol, atenolol) is instituted. The use
of sodium channel blockers (mexiletine, phenytoin) for patients with identified LQT3 has been
advocated (Schwartz et al., 1995). Limited success
has been reported with potassium supplements and
spironolactone in selected patients with LQT2
(Compton et al., 1996). Pacemaker therapy is useful in combination with beta blockade to prevent bradycardia and pause-dependent torsades de
pointes. Increasing the heart rate with pacing also
helps to shorten the QT interval in these patients.
ICD placement is indicated for those who have
experienced a previous cardiac arrest or have failed
drug therapy. Tiered therapy consisting of beta
blockade and implantation of a pacemaker/ICD is
considered appropriate for high-risk patients. In
addition, the LQTS patient is instructed to avoid
triggers such as competitive athletics (LQT1), loud
noises (LQT2), hypokalemia, and drugs that cause
QT prolongation.
ATRIOVENTRICULAR BLOCKS
Atrioventricular (AV) block describes delayed or
incomplete conduction of impulses through the AV
node. Three degrees of AV block are recognized.
First-degree AV block is defined as prolonged
conduction through the AV node; this produces a
prolonged PR interval on the ECG, but there is
consistent 1:1 AV conduction. Second-degree AV
block has two forms: Mobitz I and Mobitz II.
Mobitz type I AV block, also known as Wenckebach, is recognized on the ECG by its characteristic pattern of a gradually lengthening PR interval followed by a nonconducted P-wave (dropped
beat) (Figure 7). In Mobitz type II AV block, there
is intermittent failure of the P-wave to be conducted, but wherever measurable PR intervals occur, they are consistent and do not lengthen. Generally, Mobitz II AV block produces a fixed ratio
of P-waves to QRS complexes (2:1 or 3:1), but
occasionally the AV block is variable. Third-degree AV block is defined as complete failure of the
atrial impulses to be conducted to the ventricles.
On the ECG, AV dissociation is seen in which the
atrial rate is faster than the ventricular rate.
AV block may be congenital or acquired. Congenital AV block is estimated to have an incidence
of 1 in 22,000 live births (Ross & Gillette, 1999).
Approximately 25% to 30% of these children have
associated congenital heart disease, most commonly 1-transposition of the great arteries with
ventricular inversion. In addition, there appears to
be a strong association between SS-A/Ro or SS-

PEDIATRIC ARRHYTHMIAS

359
Table 6. Nursing Care of the Pediatric Arrhythmia Patient

Arrhythmia

Clinical Symptoms

Acute Management

Chronic Management

Parent/Patient Education

Sinus node
dysfunction

Slow heart rate

Irregular heart beat
Fatigue
Exercise intolerance
Dizziness
Syncope

Monitor rhythm and

hemodynamic status
If unstable:
Ventilate, oxygenate
Epinephrine
Atropine
Isoproterenolol
Temporary pacing

Pacemaker

How to check pulse rate

Report symptoms to cardiologist
Pacemaker education if device
is implanted

Supraventricular
tachycardia

Infant:
Irritability,
lethargy
Poor feeding
Pallor
Sweating
CHF
Older child:
Palpitations
Fast heart rate
Chest discomfort
Dizziness

Monitor rhythm and

hemodynamic status
If stable:
Vagal maneuvers
Overdrive pacing
Adenosinemay be diagnostic
and/or therapeutic (0.10.2 mg rapid IV bolus)
If unstable:
Synchronized cardioversion
(0.5-1.0 joule/kg)
For JET,
IV amiodarone
Overdrive pacing

Medications:
Digoxin for non-WPW only
Propranolol
Atenolol
Flecainide
Sotalol
Amiodarone
RF ablation

How to check pulse rate

Report symptoms to cardiologist
How to perform vagal
maneuvers (check with
cardiologist first)
If symptomatic, go to
emergency room
Medication teaching as needed
Procedural teaching if RF
ablation is planned

Ventricular
tachycardia

Cardiac arrest

Monitor rhythm and

hemodynamic status
CPR if needed
Synchronized cardioversion
(0.5-1.0 joules/kg)
If pulseless, defibrillation (2-4
joules/kg)
IV medications:
Amiodarone
Lidocaine
Procainamide
Correct electrolyte imbalance
ECMO or VAD for uncontrolled
incessant VT

Medications:
Amiodarone
Beta-blockers
Verapamil (in Verapamilsensitive VT)
RF ablation
ICD

How to check pulse rate

How to perform CPR
Call 911 in the event of
syncope or arrest
Medication teaching as needed
Avoid medications that cause
QT prolongation
Procedural teaching if ICD is
planned
Activity restrictions as
prescribed by cardiologist

Infant:
Lethargy
Tachypnea
Pallor
Poor feeding
Older child:
Palpitations
Chest discomfort
Dizziness
Nausea
Syncope

Long QT
syndrome

Syncope/presyncope
often
associated with
exercise, noise,
or stress
Palpitations
Seizures
Cardiac arrest

Monitor rhythm and

hemodynamic status
For Torsades de Pointes:
Initiate CPR
Synchronized cardioversion
Administer IV beta blocker
(Esmolol)
Temporary overdrive pacing
to suppress VT

Medications:
Beta blockers
Propranolol
Atenolol
?Alpha blockers
?Na or Ca2 ion-channel
blockers
Pacemaker
ICD

How to check pulse rate

How to perform CPR
Call 911 in the event of
syncope or arrest
Family members need ECGs to
measure QT (hereditary)
Medication teaching
Procedural teaching if
pacemaker or ICD is
planned
Avoid triggers:
Competitive athletics
Loud noises (LQT2)
Hypokalemia
Medications that cause QT
prolongation

AV block
(2 or 3)

CHF
Fatigue
Exercise intolerance
Dizziness
Syncope

Temporary pacemaker

Pacemaker

How to check pulse rate

How to perform CPR if
pacemaker dependent
Procedural teaching for
pacemaker implant
Pacemaker education,
emphasize no contact
sports
Call 911 in the event of
syncope or arrest

360

DEBRA HANISCH
Table 7. Internet Resources
Organization

Website

Comments

American Heart Association (AHA)

www.americanheart.org

North American Society of Pacing

and Electrophysiology (NASPE)
The Heart of Pediatric
Electrophysiology (HOPE)

www.NASPE.org

Professional and lay information on many

cardiac conditions, including arrhythmias
Professional and lay information on arrhythmias,
RF ablation, medications, and device therapy
Organization was formed in 1999 to provide
education and support to families and health
professionals.
Website not yet available.
Toll-free phone number: 877-394-HOPE
Nonprofit organization provides information on
Long QT Syndrome, including a
comprehensive listing of medications that
cause QT prolongation
Nonprofit organization provides information on
Long QT Syndrome
Internet resource for professionals and families
providing information on various aspects of
congenital heart disease, including
arrhythmias
Professional and lay information on medications,
including antiarrhythmic drugs

www.rhythmsofhope.org

SADS Foundation (Sudden

Arrhythmic Death Syndrome)

www.sads.org

Cardiac Arrhythmia Research and

Education Foundation, Inc. (CARE)
The Childrens Health Information
Network

www.longqt.com

Physicians Desk Reference

PDR.net

www.tchin.org

B/La autoantibodies in the mother (present in collagen vascular diseases such as lupus erythematosis) and the development of congenital AV block
in the child (Waltuck & Buyon, 1994). Surgical
AV block occurs as a complication in congenital
heart surgery because of injury to the AV node or
His bundle. Certain procedures, such as closure of
an AV septal defect or ventricular septal defect,
tetralogy of Fallot repair, subaortic resection or
aortic valve replacement, carry a higher risk for
surgical AV block. In the current era of congenital
heart surgery, the incidence of permanent AV
block is 3% or less for these procedures (Friedman,
1998). Inflammation, as seen with myocarditis,
rheumatic fever, or Lyme disease, is another cause
for acquired AV block.
Symptoms seen in children with AV block depend on the ventricular rate. Children with firstdegree or second-degree Mobitz I AV block are
generally asymptomatic. However, the fetus with
complete AV block may present with hydrops and
necessitate early delivery and intervention. CHF
may be seen in infants with slow ventricular rates,
especially in the presence of associated congenital
heart defects. Older children may complain of fatigue, exercise intolerance, dizziness, or, in some
cases, syncope. Sudden death has been reported. A
chest radiograph may reveal cardiomegaly in patients with long-standing AV block due to the
chronically slow hearts attempt to compensate by
augmenting the stroke volume.
Pacemaker therapy is clearly indicated for

symptomatic children with second-degree Mobitz

II and third-degree complete AV block. In postoperative patients, the AV block may be transient, so
temporary pacing is employed for the first 10 to 14
days. If the AV block persists beyond this period,
permanent pacing is warranted. Much controversy
exists over the proper time to intervene with pacing
in the asymptomatic child with congenital AV
block (Friedman, 1995). With advances in device
technology and pacing lead design, implanting
pacemakers in young infants and children has become much safer and more practical.
NURSING RESPONSIBILITIES
The bedside nurse is in a crucial position to
identify rhythm disturbances in pediatric patients.
Quick determination of the childs hemodynamic
status at the onset of an abnormal rhythm with
ongoing assessments throughout the course of the
arrhythmia is key to guiding therapy. Monitoring
the ECG and obtaining clear rhythm strips to document both normal and abnormal rhythms contribute to making an accurate arrhythmia diagnosis.
When antiarrhythmic drugs are used, the nurse
must be aware of possible adverse effects, including the potential for proarrhythmia. An awareness
and understanding of newer treatment modalities,
including RF ablation techniques and device therapy, has important implications for patient care as
well (Tables 6 and 7).
Patient education is a vital component of the
nurses role in caring for these children and their

PEDIATRIC ARRHYTHMIAS

361

families. Parents and patients need to learn how to

check a pulse rate, how to recognize signs and
symptoms associated with arrhythmias and side
effects of prescribed antiarrhythmic agents, what
to do if signs or symptoms occur, what types of
activity should be restricted, and, in some cases,
how to perform cardiopulmonary resuscitation
(CPR). Patients at risk for syncope or cardiac arrest
should be encouraged to obtain a MedicAlert
bracelet (MedicAlert, Turlock, CA). Psychosocial
issues need to be addressed as well. Parents and
patients may be afraid of a cardiac arrest, especially if there is pacemaker-dependency or a family

history of sudden cardiac death. Children and adolescents with implanted pacemakers or defibrillators often express concerns related to repeated surgical procedures and the resultant scars and
visibility of the implanted device. These patients
frequently express a need to be accepted by their
peers and be treated normally (Zeigler & Corbett,
1995). In some cases, referral to professional counseling may be beneficial. Effective and safe management of young patients with arrhythmias is
contingent upon a comprehensive team approach
that includes not only the health care professionals,
but also the caretakers of these special children.

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