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 nally spreading throughout the Purkinje bers to depolarize the ventricles (Figure 1). Characteristic electrocardiographic (ECG) ndings 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. SINUS NODE DYSFUNCTION 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

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 utter. 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 inammatory 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,
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
351

352

DEBRA HANISCH

Figure 1. system.

Normal conduction

Hick, & Katzman, 2000). Conditioned athletes also have slower-than-average resting heart rates. Modied 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 PR Interval (ms) QRS Duration (ms)

Range

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

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

Adapted and reprinted with permission (Liebman, 1982).

PEDIATRIC ARRHYTHMIAS Table 2. Sinus Bradycardia

ECG Criteriaa Age Group Heart Rate Age Group 24-Hour Ambulatory ECG Criteriaa Heart Rate

353

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

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

60 60 45 40 30

bpm sleeping; bpm bpm bpm bpm

80 bpm awake

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 conguration, 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 identied.

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

Figure 2.

Common mechanisms of SVT.

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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-

burger, & Benson, 1992). AVNRT is not associated with congenital heart disease (Deal, 1998). Intra-atrial re-entrant tachycardia (IART), commonly referred to as atrial utter, is a reentrant tachycardia that is conned 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 utter diagnosed beyond infancy is associated with structural heart disease (Deal, 1998). On ECG, characteristic saw-tooth waves (utter 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

Figure 3.

Wolff-Parkinson-White (WPW).

PEDIATRIC ARRHYTHMIAS

355

Figure 4.

Atrial utter 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 lling (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 nonspecic symptoms such as irritability, lethargy, poor feeding and, after 24 to 48 hours, signs of conges-

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

Figure 5.

Junctional ectopic tachycardia (JET).

356 Table 3. Age-Related Incidence of SVTa

Age AVRT AVNRT Primary Atrial Tachycardia (IART, AET)

DEBRA HANISCH

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

aBased

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

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

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

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 reex, 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, ecainide, 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 dened 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

Supraventricular Tachycardia Sinus Tachycardia

History Examination

ECG

Chest radiograph Echocardiogram

Nonspecic; 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 Small or normal heart, clear lung elds (unless congenital heart disease present) Usually normal

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

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

1.0 joules/kg) or, if pulseless, debrillation (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-debrillator (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-

Figure 7.

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 ndings, clinical presentation, and family history. Typically on ECG, abnormal T-wave morphology is present and may reect the specic 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 identied 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 specic types of LQTS that have been identied 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

factors. Typically, drug therapy with beta blocking agents (propranolol, atenolol) is instituted. The use of sodium channel blockers (mexiletine, phenytoin) for patients with identied 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 dened 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 xed ratio of P-waves to QRS complexes (2:1 or 3:1), but occasionally the AV block is variable. Third-degree AV block is dened 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-

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.

PEDIATRIC ARRHYTHMIAS Table 6. Nursing Care of the Pediatric Arrhythmia Patient

Arrhythmia Clinical Symptoms Acute Management Chronic Management Parent/Patient Education

359

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 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 Monitor rhythm and hemodynamic status CPR if needed Synchronized cardioversion (0.5-1.0 joules/kg) If pulseless, debrillation (2-4 joules/kg) IV medications: Amiodarone Lidocaine Procainamide Correct electrolyte imbalance ECMO or VAD for uncontrolled incessant VT Monitor rhythm and hemodynamic status For Torsades de Pointes: Initiate CPR Synchronized cardioversion Administer IV beta blocker (Esmolol) Temporary overdrive pacing to suppress VT

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

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 rst) If symptomatic, go to emergency room Medication teaching as needed Procedural teaching if RF ablation is planned

Ventricular tachycardia

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

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

Long QT syndrome

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

Medications: Beta blockers Propranolol Atenolol ?Alpha blockers ?Na or Ca 2 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 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

AV block (2 or 3)

CHF Fatigue Exercise intolerance Dizziness Syncope

Temporary pacemaker

Pacemaker

360 Table 7. Internet Resources

Organization Website Comments

DEBRA HANISCH

American Heart Association (AHA) North American Society of Pacing and Electrophysiology (NASPE) The Heart of Pediatric Electrophysiology (HOPE)

www.americanheart.org www.NASPE.org 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 www.tchin.org

Physicians Desk Reference

PDR.net

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 Nonprot organization provides information on Long QT Syndrome, including a comprehensive listing of medications that cause QT prolongation Nonprot 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

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). Inammation, 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 rstdegree 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 rst 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

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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 debrillators 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 benecial. 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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