Evaluation and Management of Arrhythmias in the Pediatric Population

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Chapter 75 Evaluation and Management of Arrhythmias in the Pediatric Population

Appropriate evaluation and management of arrhythmias in pediatric patients require an understanding of the presentation, clinical correlations, and unique responses of children to the various potential treatments. The primary focus of this review of arrhythmias in the pediatric population is on arrhythmias that are seen more frequently or behave differently in children than how they do in adults. For arrythmias that have shared characteristics, we refer you to the excellent chapters in this text dealing with each specific rhythm disturbance.

Supraventricular Tachycardia

General Presentation and Evaluation

Supraventricular tachycardias (SVTs), which are arrhythmias that originate above the ventricles and involve the atria, the atrioventricular (AV) node, or the accessory bypass tracts, represent the most common tachycardias seen in infants and children, with an incidence of 1 in 250 to 1000 in the United States.1 SVTs are classified by identifying those components of the heart that are involved in maintaining the tachycardia. SVTs include primary atrial tachycardias (those in which the AV node and the ventricles are not involved), AV reciprocating tachycardias (those that involve both the atrium and the ventricle), and AV nodal re-entrant tachycardias (those that do not involve the atria as part of the circuit). The presentation of the pediatric patient with SVT varies from palpitations to signs of marked cardiac failure. This variability is related to the patient’s age at presentation and his or her ability to communicate symptoms, the duration and rate of the tachycardia, as well as the mechanism of SVT. Parents of infants with SVT occasionally note changes in feeding habits and irritability as symptoms. Symptoms in those old enough to voice complaints include palpitations (noted as skipped or rapid heart beats, or as “heart beeping”), chest pain, shortness of breath, fatigue, dizziness, and syncope.

The evaluation of pediatric patients includes a 12- or 15-lead electrocardiogram (ECG), which may show Wolff-Parkinson-White (WPW) syndrome or other abnormalities such as an ectopic atrial rhythm. While the ECG is often normal when SVT is not present, an ECG during SVT can be very helpful in distinguishing the mechanism of the tachycardia. A recent study suggested that leads V1 and III were the most helpful in this diagnosis.2 Twenty-four-hour ambulatory (Holter) monitoring is useful to screen for occult arrhythmias or to assess heart rate ranges and variability if the patient is having frequent symptoms (i.e., on a daily basis). Transtelephonic event monitors are often helpful in those with fleeting symptoms, allowing the patient or parent to record symptoms as they occur with simultaneous ECG recordings. Exercise tolerance testing is useful in those with palpitations or symptoms associated with activities. More invasive testing such as a transesophageal or intracardiac electrophysiological study (EPS) is used to induce arrhythmia and determine the particular mechanism and often as a prelude to catheter ablation.

Mechanisms

Three primary mechanisms are involved in SVT: (1) enhanced automaticity, (2) triggered automaticity, and (3) re-entry. Automaticity is the ability of cardiac myocytes to spontaneously depolarize, which leads to myocardial contractions. Sinus and AV nodes are the primary sites of automaticity in the heart. Enhanced automaticity occurs when myocytes outside the sinus or the AV node depolarize spontaneously, which leads to atrial or ventricular ectopic beats or SVT and to ventricular tachycardias (VTs) when repetitive ventricular ectopic beats occur.3

Tachycardias arising secondary to enhanced automaticity have characteristics that are distinct from those of re-entry. They are highly catecholamine sensitive, with warm-up and cool-down phases. This leads to variable rates during the tachycardia. These tachycardias are not inducible with programmed stimulation, nor are they terminated with overdrive pacing or with direct-current cardioversion. Automatic tachycardias can arise from all areas of the heart, with those from the atria referred to as ectopic atrial tachycardia, those from the AV junction as junctional ectopic tachycardia, and those from the ventricles as automatic VT.

Triggered automaticity results from spontaneous myocardial contractions that occur secondary to oscillations during repolarization reaching threshold and leading to a depolarization.4,5 These oscillations are referred to as after-depolarizations. Arrhythmias arising from triggered automaticity have characteristics shared by both enhanced automaticity and re-entry. They are highly catecholamine sensitive and have warm-up and cool-down phases with a wide variation in heart rate, similar to other forms of automaticity. They can be induced and terminated with pacing maneuvers and respond to direct-current cardioversion, as do re-entrant arrhythmias. Triggered automaticity is thought to play a role in the arrhythmias seen in digoxin toxicity.6

Re-entry occurs when a wavefront of electrical activation travels through tissue for a distance and then re-enters the original tissue and propagates through the circuit again. Re-entrant SVT is the most common type of tachycardia seen in pediatrics. To have a re-entrant circuit, at least two pathways with distinct conduction properties and refractory periods must be present. The sequence of events in a re-entrant circuit is as follows. A stimulus encounters two distinct pathways, with one pathway refractory to conduction (the pathway with the longer refractory period) and one ready for conduction (the pathway with the shorter refractory period). The impulse is conducted along the pathway, with the shorter refractory period having enough conduction delay to allow the first pathway to recover and to conduct the impulse back to the original site of entry into the circuit. The impulse then re-enters the original pathway and transverses the circuit again. Re-entry can occur in the sinus node, the atrial tissue, the AV node, and the ventricular tissue or between the atrium and the ventricle. Those re-entry circuits between the atrium and the ventricle involve specialized conduction tissue referred to as accessory pathways. In AV nodal re-entrant tachycardia (AVNRT), the accessory pathway is in the AV node or in the AV nodal region. Moe and colleagues initially described the evidence of dual AV node physiology in 1956.7 Re-entry tachycardias have characteristics that are distinct from those seen in automatic tachycardias. They often have sudden onset and termination, with patients frequently feeling that they have been “switched” on and off. The tachycardia is regular, with little variation in rate. Re-entry tachycardias often respond to vagal maneuvers by slowing or terminating the tachycardia. These tachycardias are easily induced and terminated with pacing protocols ranging from single premature stimuli to burst pacing or by direct-current cardioversion.

Specific Mechanisms in Children

The most common specific mechanism of SVT in children, representing 75% to 90% of arrhythmias in children without other cardiac conditions, is re-entry through an accessory pathway between the atrium and the ventricle or within the AV node.8,9 Over two thirds of these pathways are concealed, and one third are manifested as WPW or other forms of pre-excitation. AV nodal re-entry represents around 15% of pediatric SVTs, most commonly seen in adolescents, with up to one third of SVTs presenting in adolescence, the cause being AVNRT.9,10 Automatic atrial ectopic tachycardia represents 10% to 18% of SVTs in children. Atrial flutter and atrial fibrillation (AF) are responsible for 10% and 40% in non-postoperative and postoperative children, respectively, and junctional ectopic tachycardia responsible for less than 1% in non-postoperative patients.

Atrioventricular Reciprocating Tachycardias

Presentation and Treatment

Some variability exists in the presentation of AV reciprocating tachycardia in childhood. This may relate more to the age of the patient than to the specific type of tachycardia. For this reason, the presentation and treatment of pediatric patients with AV nodal re-entry, concealed bypass tract, and WPW re-entrant tachycardia are combined in the following overview, with emphasis on the different age-related presentations and treatment strategies.

Tachycardia may be seen as early as during fetal development. The diagnosis of fetal SVT may be made by auscultation of the baby’s heart rate during maternal examination and is confirmed by fetal ultrasonography or fetal magnetocardiography.11 See Chapter 76 for detailed information on these arrhythmias.

It has been reported that 50% to 60% of SVTs in children present in the first year of life, with accessory pathway–mediated re-entrant tachycardia being the most common mechanism.12,13 The heart rate in infants with SVT is usually 220 to 320 beats/min. Neonates and infants with tachycardia are often very ill, presenting with congestive heart failure, which occurs if the tachycardia has been present for more than 48 to 72 hours before the patient receives medical care. This occurs because of the infant’s inability to communicate and the family’s lack of awareness that the child could have a significant medical condition. Parents may note that the infant is acting somewhat different from normal, is more irritable, or is not eating well. This is often interpreted as colic or some other “normal” childhood problem. At presentation, these babies often are acidotic from decreased cardiac output and may need aggressive resuscitation, including artificial ventilation and rapid termination of the tachycardia. Intravenous (IV) adenosine is effective in the acute termination of SVT in this population, but IV access is often difficult in a 3- to 4-kg baby with congestive heart failure. It has been suggested that in the treatment of infants, the starting doses of adenosine should be higher than those generally recommended as the average effective dose in infants, which is around 200 µg/kg.14 If IV access is not possible, transesophageal overdrive pacing has proven to be effective. Once the rhythm is restored to normal and the cardiac function has begun to recover, IV access becomes easier, and IV medications can be administered. If the infant is hemodynamically compromised and the rhythm cannot be medically converted, electrical synchronized cardioversion is indicated.

Medications for the acute treatment of SVT are shown in Table 75-1. Digoxin is a first-line medication used in the treatment of SVT in infants with decreased myocardial performance. It is contraindicated as a chronic treatment in patients with WPW syndrome but can be used acutely in those with decreased ventricular function with careful monitoring. The authors of this chapters do not discharge patients with WPW syndrome on digoxin because of its potential effect to enhance conduction of atrial impulses to the ventricle but switch them to another medication once the cardiac function has normalized. Other IV medications used acutely to treat SVT include β-blockers such as esmolol, amiodarone, and procainamide. These medications must be used with caution because of their negative inotropic effects, which can lead to worsening of cardiac function. The use of IV calcium channel blockers is contraindicated in infants and children younger than 1 year because as sudden death has been reported in some cases.15,16 In a long-term study from Sweden, the SVT was managed with a single drug in 62% of patients.17

Table 75-1 Pharmacologic Agents for Acute Treatment of Supraventricular Tachycardia

AGENT INITIAL TREATMENT (IV)
Adenosine 50–100 µg/kg, increase by 50-µg/kg increments
Every 2 min to 400 µg/kg or 12 mg maximal dose
Amiodarone IV: 5 mg/kg over 1 hour, followed by IV bolus of 2.5 mg/kg q4–6h
Digoxin Dose is age dependent
Give in 3 doses (image TDD, image TDD, image TDD)
Preterm infant: 10–20 µg/kg TDD
Term newborn to adolescent: 30–40 µg/kg TDD oral to maximal TDD of 1–1.5 mg (IV 3/4 PO)
Oral maintenance: 10 µg/kg/day q12h
Esmolol IV Load: 200–500 µg/kg/min over 2–4 min. May increase in 50–100 µg/kg/min increments (maximum dose = 1000 µg/kg/min)
Maintenance infusion: 50–200 µg/kg/min
Phenylephrine 100 µg/kg bolus
10 µg/kg/min infusion
Procainamide 5 mg/g over 5–10 min or 10–15 mg/kg over 30–45 min
20–100 µg/kg/min infusion
Propranolol 0.05–0.1 mg/kg over 5 min q6h
Verapamil 0.05–0.30 mg/kg over 3–5 min
Maximal dose: 10 mg

IV, Intravenous; TDD, total digitalizing dose.

Presentation in Older Children

After the peak of presentation in the first year of life, the other two common ages of presentation are early childhood (6 to 8 years of age) and adolescence.12,18 SVT in children is predominantly caused by concealed or manifested accessory pathways (WPW) throughout early childhood, with the proportion of patients with AVNRT increasing with age.8 The heart rate in older children is generally in the range of 160 to 280 beats/min. Older children often present with palpitations or dizziness. It is not uncommon to hear a 3- or 4-year-old complain that the heart is “beeping” too fast. These patients are generally not in SVT long enough to develop congestive heart failure, as seen in infants, because they can communicate to their caregivers that they are experiencing something abnormal or unusual. With the exception of those with WPW syndrome with rapid conduction down their accessory pathway during an atrial tachycardia, patients rarely present with syncope during SVT.

Treatment

The long-term treatment of infants with AV reciprocating tachycardia includes the use of oral preparations of the medications listed in Table 75-2, with digoxin or propranolol/β-blocker medications being the most commonly used agents. During initiation of therapy with oral antiarrhythmics in infants, the authors of this chapter monitor the patients in the hospital for at least five half-lives of the medication, allowing a steady state to be reached, watching for side effects, and educating the family about administering the medication and recognizing the tachycardia. The family can also be taught about simple vagal mechanisms to interrupt the tachycardia. When starting therapy with oral β-blockers in infants and young children, the patient must be monitored for hypoglycemia and the caregivers must know how to recognize the symptoms of hypoglycemia. The authors of this chapter ask the family to notify medical personnel if their child is not tolerating the medication or if side effects occur. It is not the authors’ practice to discharge patients with heart rate monitors. Families can assess their children for recurrent SVT without resorting to continuous monitoring. Most infants are treated with oral medications for 10 to 12 months, with the dosage adjusted on the basis of weight gain. At 10 months to 1 year of age, if no recurrences of the SVT have occurred, the patient will be weaned from the medication unless the WPW pattern is still present on the ECG. Approximately one third of all patients who develop SVT in the first 3 months of life will outgrow it by 1 year of age.12,18,19,20

The short- and long-term medical management of children and adolescents is similar to that of infants. The exception is that catheter ablation becomes more of a therapeutic option around 4 to 5 years of age and is more commonly used after 8 years of age. Ablation can and has been performed in younger children, but the overall consensus is that it should only be used for patients 4 years of age or younger who have medically refractory arrhythmias, as the risks of the procedure are higher under 4 years of age.21,22 See Chapter 78 for detailed information on catheter ablation in children.

Parents should be reassured regarding the generally benign nature of these arrhythmias. Parents should be taught about vagal maneuvers that can be used to interrupt the tachycardia, including applying ice or cold to the face, inducing gagging, breath holding, Valsalva maneuver, and performing a headstand or handstand. Schools should have emergency plans in place regarding whom to contact and what symptoms should prompt a call for emergency help, such as when a student faints and is unresponsive or seems to be experiencing a cardiac arrest.

Wolff-Parkinson-White Syndrome

Presentation

WPW syndrome in children is very similar to that in adults. This syndrome involves an accessory pathway between the atrium and the ventricle that usually has bi-directional conduction properties. The reported incidence of WPW syndrome ranges from 1 to 4 per 1000 live births.23 Of all patients who present with WPW syndrome in childhood, one fifth to one third will have associated cardiac abnormalities. The most common congenital lesions include Ebstein’s anomaly of the tricuspid valve and L-transposition of the great arteries.18 Patients with WPW syndrome present any time from fetal life through adolescence. The patients who present during fetal life and early infancy present with re-entrant SVT. Children and adolescents also present with re-entrant SVT but can occasionally present with atrial fibrillation (AF) with rapid conduction down the accessory pathway. Approximately 10% of children will present with an antidromic tachycardia that uses the accessory pathway as the antegrade limb, with approximately half of these patients having multiple accessory pathways.24 A number of asymptomatic patients are noted to have the pattern of WPW syndrome on ECGs obtained for other reasons. Sudden death can be the first manifestation of WPW, leading some authors to suggest that risk stratification, including an EPS, should be done for all patients with WPW beyond infancy and early childhood.25 The presence of a short refractory period or multiple pathways increases the risk of a subsequent life-threatening event, and catheter ablation is advised.26 Occasionally, an infant presents with a narrow-complex tachycardia with no evidence of a WPW pattern on a baseline ECG, but pre-excitation becomes obvious when the patient is treated with a medication (e.g., digoxin) that slows AV nodal conduction.

Treatment

In the young adolescent or older child who presents with WPW syndrome, the authors of this chapter offer catheter ablation as a first-line therapeutic option. In their practice, in children with known WPW syndrome older than 6 to 8 years of age, the accessory pathway conduction or the refractory period of the accessory pathway is routinely assessed by using exercise testing, esophageal pacing, intracardiac EPS, or combinations of these methods. When given the option, many patients who are old enough to decide or their caregivers agree to an intracardiac EPS so that a catheter ablation procedure can be performed and the WPW addressed definitively. The treatment strategies for patients with WPW syndrome vary on the basis of the patient’s age and symptoms at the time of presentation. The mainstay of treatment in infants and young children with WPW has been the use of antiarrhythmic medications. The authors refrain from the use of digoxin or calcium channel blockers in patients with WPW because conduction down the accessory pathway may be enhanced, along with blockade of the conduction through the AV node. This is thought to lead to an increased risk of rapid conduction of AF or premature atrial contractions through the accessory pathway. The authors recommend β-blockers as a first-line therapy unless a contraindication such as severe reactive airway disease is present. When β-blocker therapy is contraindicated or fails to control the tachycardia, the authors use other medications such as flecainide, amiodarone, or sotalol. Occasionally, a neonate with WPW syndrome presents with significant signs of cardiac decompensation and cardiogenic shock. In those circumstances, to improve ventricular function as well as to suppress the tachycardia, the authors use digoxin while the patient is still in the hospital. The patient is always converted from digoxin to another antiarrhythmic medication before being discharged.

A special consideration in the pediatric population is the patient who presents with asymptomatic WPW syndrome following an ECG obtained for some other indication such as chest pain or for a school physical examination. The authors recommend that patients older than 6 to 8 years of age have the evaluation mentioned above to assess whether they have rapid conduction down their accessory pathways. Generally, these patients are taken to the electrophysiology laboratory to measure their minimum cycle length of pre-excitation both with atrial pacing and during AF. A minimum cycle length of pre-excitation of less than 220 ms is used during AF as a marker that the patient may have a significant risk of sudden death. This is based on studies performed by Bromberg et al and Paul et al, which demonstrated that these values are helpful, though not fully predictive of cardiac arrest and syncope.27,28 Additional studies by Pappone and Santinelli show an increased risk for sudden death with WPW with short accessory pathway effective refractory periods and with multiple pathways and recommend that catheter ablation be performed.26,29,30

Primary Atrial Tachycardia

Ectopic Atrial Tachycardia

Ectopic atrial tachycardia (EAT), or automatic atrial tachycardia, is an arrhythmia arising from both atria with inappropriately fast atrial rates. This tachycardia represents approximately 10% of the SVT seen in the overall population.32 The heart rates seen in EAT vary on the basis of the patient’s age and catecholamine state during the tachycardia. The heart rates in automatic atrial tachycardias will be inappropriately fast for the patient’s level of activity and are generally in the 130 to 250 beats/min range. In general, the rates are not as fast as those seen in re-entrant tachycardias. The pulse rate may not be reflective of the atrial rate because of variable atrial conduction through the AV node.

Tachycardias arising from foci of increased automaticity can be found in all areas of the atria. The automatic foci are more likely to be located in certain areas than in others. In the right atrium, the foci are frequently found in the right atrial appendage, and in the left atrium, they are often mapped to the orifices of the pulmonary veins.3335

Presentation

EAT is not seen frequently in young infants, but the overall incidence of automatic atrial tachycardia reported by Gillette was 18% of all SVT seen in children.36 EATs usually have a “warm-up” phase in which the heart rate will gradually increase to its maximum rate. During termination, it will “cool down” with a gradual slowing of the rate and become indistinguishable from that of sinus rhythm. This gradual increase in heart rate and termination, which is markedly different from the sudden onset and termination seen in re-entrant SVT, makes it difficult for the patient to recognize the tachycardia. Failure to perceive the arrhythmia and its incessant nature can lead to significant depression of myocardial function, which results in a tachycardia-mediated cardiomyopathy. Many patients with EAT present with signs of congestive heart failure with decreased left ventricular (LV) contractility, AV valve regurgitation, and atrial dilation.37 If the tachycardia is not treated aggressively, the myocardial function can continue to decline, resulting in an irreversible cardiomyopathy. Resolution of the cardiomyopathy can take 6 to 12 months after the tachycardia has been terminated.

Evaluation

ECGs in patients with an EAT generally show a P-wave axis distinct from sinus rhythm. When the focus arises from the left atrium, the P wave is negative in lead I; those with the focus in the low right atrium show a negative P-wave axis in lead aVF and a positive P wave in lead I. Occasionally, the focus is in an area close to the sinus node or in the high right atrium with the P-wave axis similar to sinus tachycardia (0 to 90 degrees in the frontal plane). This can lead to a delay in diagnosis and institution of therapy, when the rhythm is thought to be sinus tachycardia. EAT, as well as other primary atrial tachycardias, can show variable degrees of AV conduction on ECG and Holter monitoring, which is often helpful in establishing the diagnosis. Figure 75-1 represents an ECG of a patient with EAT.

Holter monitoring is helpful in establishing the diagnosis and in determining the frequency of EAT. A careful review of the patient’s diary of activities along with the corresponding heart rates allows the determination of inappropriate elevation of heart rate. The overall average heart rate over 24 hours provides an indication of the amount of time the patient is in SVT. Special attention should be given to the heart rate during sleep. These noninvasive evaluations may be the only way to assess the patient with EAT. Exercise testing is frequently not useful in evaluating EAT because as the sinus heart rate increases, the automatic focus is suppressed. In most cases, EAT cannot be induced in the cardiac catheterization laboratory using conventional pacing protocols but may be induced by rapid atrial pacing of the heart (if triggered automaticity is the mechanism) or by isoproterenol infusion.

A patient with EAT should have a complete echocardiographic evaluation to rule out congenital heart disease, especially the types that could lead to an abnormal sinus node (and therefore abnormal P-wave axis), such as heterotaxy syndrome. A careful assessment of ventricular function, as well as the presence of AV valve regurgitation, should be performed. This evaluation should be done as a baseline study, before medical intervention, to document the progression of the disease or the possible deterioration of ventricular function secondary to the negative inotropic properties of the medical management or to inadequate control of the tachycardia.

Treatment

The medical management of EAT may be problematic, as complete control of the tachycardia is difficult to attain. Two strategies are used in the treatment of EAT. One strategy is an attempt to slow the ventricular rate by slowing conduction through the AV node. The primary medication used in this effort is digoxin, which slows the conduction through the AV node by enhancement of vagal activity. Digoxin is a positive inotropic agent and helps improve ventricular performance. Calcium channel blockers can increase AV block but are negative inotropes and should be used with caution. Medications that have the potential to slow the tachycardia include β-blockers and class I and class III antiarrhythmics. β-Blockers have the potential to slow the tachycardia by blocking the effect of catecholamines on the ectopic focus. Class IC medications such as flecainide and propafenone have shown some success in the management of EAT.38,39 Amiodarone and sotalol (class III) have been used with modest success by slowing conduction throughout the myocardium as well as by slowing AV conduction.4042 Class IA antiarrhythmic medications such as procainamide decrease automaticity, prolong refractoriness, and slow conduction velocity.43,44 All of these medications have the potential to decrease myocardial performance and must be used with caution in patients with decreased LV function. Similarly, the side effects must be carefully monitored. The second strategy, nonpharmacologic methods of treatment, is gaining increasing interest because of the difficulty in the medical management of EAT and the sequelae of uncontrolled tachycardia. These procedures include surgical ablation procedures, which have reasonable success rates but require an open chest procedure, frequently including cardiac bypass.45,46 Catheter ablation, using radiofrequency or cryothermal energy, has become the treatment of choice for patients with poorly controlled EAT.35,34,47 Certain technical aspects may make catheter ablation of EAT challenging. It is difficult to induce EAT. If the arrhythmia does not occur spontaneously, it cannot be mapped and ablated in the electrophysiology laboratory. EAT is considerably catecholamine sensitive. It is not uncommon for the tachycardia to “go to sleep” when the patient does, making sedation an issue in the laboratory. See Chapter 78 for information on ablation.

Multifocal Atrial Tachycardia or Chaotic Atrial Tachycardia

Multifocal atrial tachycardia (MAT), or chaotic atrial tachycardia (CAT), is a primary atrial tachycardia arising from multiple areas of enhanced automaticity in the atria. By definition, the tachycardia must have at least three distinct P-wave morphologies to be considered a MAT. These tachycardias are similar to other primary atrial tachycardias in that there may be variable conduction from the atrium to the ventricle. MAT is occasionally confused with AF in that multiple P-wave morphologies and variable P-R and R-R intervals are present. The most common presentation occurs in the newborn, with a large percentage resolving spontaneously over time.48

Atrial Flutter

Atrial flutter is a re-entry circuit confined to the atria. Although atrial flutter is seen fairly commonly in adults with structurally normal hearts, the majority of atrial flutter seen in the pediatric population occurs in patients with congenital heart disease. In “classic” atrial flutter, typical ECG findings include negative flutter waves in the inferior leads (II, III, and aVF) instead of P waves, with atrial rates of 250 to 450 beats/min. This type of atrial flutter usually involves the isthmus between the inferior vena cava and the tricuspid valve as part of its circuit through the atrium.

Neonates present with atrial flutter in utero, at the time of birth, or shortly thereafter. In most cases, the patient has a structurally normal heart, although congenital heart disease may be present. Atrial flutter represents 15% to 50% of all fetal SVTs, often resulting in fetal hydrops (a form of congestive heart failure).5052 Treatment with sotalol has been effective in 80% of fetuses with atrial flutter.53 See Chapter 76 for additional information on fetal atrial arrhythmias.

Atrial flutter seen in the newborn presents most commonly in the first month of life, often within 2 days of birth. A report of 50 cases indicated that heart failure was present in 20% of these infants. The atrial rate range was 340 to 580 beats/min.54 Twenty-two percent developed additional atrial or supraventricular arrhythmias during the follow-up. AF is generally converted to sinus rhythm with medication, transesophageal pacing, or direct current cardioversion. In the remainder of these patients, recurrence is rare.

Patients with congenital heart disease may develop atrial flutter before or following surgical interventions. This is most commonly seen in those lesions with extensive atrial surgery or prior postoperative atrial dilation. The multiple atrial flutter circuits seen after surgery for congenital heart defects results in slower rates and unusual flutter wave axis and morphology. These different “flutter” characteristics have led many experts to refer to this as intra-atrial re-entry tachycardia rather than as atrial flutter. Refer to Chapter 80 for additional information on atrial flutter in association with congenital heart disease.

Treatment

Before attempting to convert atrial flutter, the presence of atrial thrombi should be ruled out to prevent emboli with conversion of the rhythm. The most effective means to evaluate intracardiac thrombi is a transesophageal echocardiogram. An associated congenital heart defect such an undiagnosed atrial septal defect can be ruled out at the same time. Acute treatment of atrial flutter includes the use of IV medications, overdrive pacing, or direct-current cardioversion. Medications that have been used include those from class IA such as procainamide, class IC (propafenone), and class III (amiodarone, sotalol). Before using these medications, rapid AV nodal conduction should be blocked with medication such as digoxin to slow AV nodal conduction. Digoxin, which is the first-line medication used in the chronic treatment of atrial flutter, works by slowing the conduction through the AV node, preventing rapid ventricular rates, or preventing the initiation of atrial flutter. Frequently, digoxin alone will not control atrial flutter in these patients. Other medications such as class IA agents (procainamide, quinidine, and disopyramide) have been used. These medications have potential deleterious side effects. Each has the propensity to prolong the Q-T interval and can be proarrhythmic with the development of torsades de pointes. Disopyramide is a potent negative inotrope, which may be detrimental to the patient with marginal ventricular function. Class IC medications such as flecainide and propafenone have been used, but they have negative inotropic properties. Class III medications (amiodarone and sotalol) have had varying degrees of success in the management of patients with chronic atrial flutter. These medications have the benefit of slowing conduction through the AV node. Sotalol, which has β-blocking properties, can be a significant negative inotropic agent. As the use of any of these medications can lead to the development of significant bradycardia, patients should be monitored closely for sinus bradycardia, sinus arrest, or junctional escape rhythms. Pacemakers can be used in those patients who develop symptomatic bradycardia.

In patients with recurrent atrial flutter who are beyond early childhood, catheter ablation is an effective treatment with success in 91%.55 See Chapter 78 for details.

Junctional Ectopic Tachycardia

Junctional ectopic tachycardia (JET) is an automatic tachycardia that arises from the AV junction. Two distinct types of JET are seen in childhood. The first is a familial form that occurs in early infancy and may be associated with congenital heart defects in up to 50% of patients.57 The second type is seen in the early postoperative period following repair of congenital heart disease (see Chapter 80).58 In both forms, the tachycardia appears to be secondary to enhanced automaticity. In those patients who present with the familial type of JET, the heart rates will range from 180 to 240 beats/min. The ECG findings in JET show a tachycardia with a ventricular rate that is faster than the atrial rate, with a narrow QRS complex similar to that seen in the patient’s normal sinus rhythm. The ECG findings of JET are shown in Figure 75-2. Rarely, patients with JET develop rate-related aberrancy, and some postoperative patients have a pre-existing bundle branch block that will lead to a wide-complex tachycardia. If the QRS is wide, the diagnosis of VT must be considered and ruled out by comparison with the QRS in normal sinus rhythm or by pacing the atrium faster than the ventricular rate to demonstrate conduction through the His-Purkinje system with persistent wide-complex QRS morphology.

JET tends to be faster and more incessant when it manifests before 6 months of age.59 Patients with JET can present with signs and symptoms of congestive heart failure secondary to the persistently elevated heart rate. Sudden death has been reported in this patient population.59,60

Treatment

Treatment strategies for the familial form of JET include digoxin to slow the rhythm and provide inotropic support. Digoxin alone may not be sufficient to manage this arrhythmia, and the addition of a class IA, class IC, class II, or class III antiarrhythmic agent may be required. It has been the chapter authors’ experience that a combination of these medications is required to control the tachycardia. Class III agents such as IV amiodarone are quickly added to treat individuals who have rapid rates and poor ventricular function, while carefully monitoring blood pressure and cardiac output. The amount of antiarrhythmic medication needed to control the rate to prevent decompensation of cardiac function may suppress the sinus node considerably, necessitating a pacemaker. Amiodarone appears to be the most effective agent in the largest group reported with a 60% success rate. Patients with JET have been treated with catheter ablation with a 80% to 85% success rate but with a risk of AV block, which has led to the more recent recommendations of the use of cryothermal energy (see Chapter 78).61,62 Because of the proximity of the AV node and His bundle to the area of enhanced automaticity that is responsible for JET, catheter ablation in this setting carries a relatively high risk of causing complete heart block. It is possible that, over time, the junctional rate will slow to a point that the patient could be weaned from the chronic medications. Refer to Chapter 80 for additional information on postoperative JET.

Ventricular Arrhythmias

Ventricular arrhythmias include premature ventricular contractions (PVCs), couplets, nonsustained ventricular tachycardia (NS-VT), sustained VT, and ventricular fibrillation (VF). PVCs may be seen in 15% of normal newborns, one third of normal adolescents, and two thirds of adolescents and adults with repaired congenital heart disease.63 PVCs may occur without identifiable cause in children and are often benign. PVCs may be associated with acute or more chronic conditions. A marked difference in prognosis for PVCs is seen between children with normal hearts and those with abnormal hearts, so investigation of children with PVCs for associated conditions should be undertaken.

Evaluation

An echocardiogram should be obtained to look at associated factors and conditions, including structural abnormalities such as hypertrophic cardiomyopathy and abnormalities in cardiac function that might accompany myocarditis or dilated cardiomyopathy. Rarely, cardiac tumors such as rhabdomyomas are identified, or noncompaction of the ventricular myocardium is seen. The evaluation should include a standard ECG on which the QTc (corrected Q-T interval) is carefully measured manually. A 24-hour Holter monitor will determine the amount and complexity of the ectopy. In the presence of a normal heart, less than 20% ectopy usually does not interfere with cardiac function and can be monitored. More than 30% ectopy may result in ventricular dysfunction over time. Patients with this degree of ectopy may have underlying cardiac disease that was not initially diagnosed. Magnetic resonance imaging (MRI) may be indicated if arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is suspected. In patients with more frequent PVCs, an exercise stress test should be performed. Generally, suppression of PVCs during exercise is a positive finding, whereas an increase in ventricular arrhythmia with exercise is not. Prolongation of the corrected Q-T interval, especially in the recovery phase, may be seen in patients with long QT syndrome (LQTS). An EPS would rarely be indicated, unless symptoms suggest more complex arrhythmias or the PVCs are associated with conditions that might predispose the patient to VT or VF.

Prognosis

Long-term follow-up suggests that PVCs and VT disappear over time in 37% to 65% of patients with normal hearts.64 Sudden death is rare in children with PVCs with otherwise normal health, but a few cases have been reported in the literature. In children with abnormal hearts, PVCs may be precursors of more serious arrhythmias, especially if they are complex-multiform, coupled, or associated with VT.

Ventricular Tachycardia

As with PVCs, VT occurs in both acute and chronic situations. Ventricular arrhythmias are less common than supraventricular arrhythmias in children but appear to be occurring more frequently in recent years or are being recognized to a greater extent. An increase in VT in patients after congenital heart surgery is seen, as survival after complex surgery has increased (see Chapter 80).65 Improved methods of surveillance and diagnosis of arrhythmias have made it possible to recognize various etiologies of VT in children, with the most common being congenital LQTS, cathecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic and dilated cardiomyopathies, ARVC, myocarditis, abnormal foci or circuits in structurally normal hearts, and idiopathic etiologies.

Electrocardiographic Manifestations

The electrocardiographic diagnosis of VT is made most easily in the presence of a wide QRS tachycardia with AV dissociation. Many children have ventriculoatrial (VA) conduction with relatively rapid 1 : 1 retrograde VA conduction, and AV dissociation may not occur. VT must be differentiated from all other forms of wide QRS tachycardias, including SVT with bundle branch aberrancy; antidromic SVT using an accessory AV connection; SVT using a nodoventricular, nodofascicular, or atriofascicular connection; or atrial flutter with aberrant conduction. Although other mechanisms of wide QRS tachycardia have been described, until proven otherwise, a wide QRS tachycardia in a child must be considered to be VT. It must be remembered that the normal QRS duration in infants and young children is 40 to 80 ms, so a wide QRS in an infant might only be slightly longer than 80 ms. The rates of VT in pediatrics vary from 120 to 300 beats/min. The T-wave vector is divergent from the QRS vector, but opposite polarity will not occur in every lead. Left bundle branch block (LBBB) is the most common morphology, but right bundle branch block (RBBB) or alternating RBBB and LBBB may occur. The presence of PVCs during sinus rhythm with the same configuration as VT is a suggestive sign of the ventricular origin of the arrhythmia. AV dissociation is suggestive of VT, but 1 : 1 VA conduction is common, especially in young children. Fusion beats are commonly noted at the onset or termination of the VT, which may be sustained (>30 consecutive complexes) or nonsustained (3 to 30 consecutive complexes).

Further differentiation is made according to the morphology, with VT being described as monomorphic or polymorphic. Two types of polymorphic VT have been described, torsades de pointes and bi-directional VT (Figure 75-3). Torsades de pointes is associated with LQTS and so named because of its twisting, undulating nature. Bi-directional VT, with beat-to-beat variation in the QRS axis on ECG, has been associated with digoxin toxicity, familial hyperkalemic paralysis, or CPVT.66

Etiology of Ventricular Tachycardia

Causes of acute VT not associated with congenital heart defects are shown in Box 75-1. These most common causes include metabolic and electrolyte abnormalities; infectious processes such as myocarditis, which may cause LV microaneurysms67; human immunodeficiency virus (HIV) infections68; blunt cardiac trauma, including commotio cordis6971; coronary ischemia, especially in association with Kawasaki disease; and drugs such as caffeine, inhalation anesthetics, and recreational drugs, including amphetamines and cocaine.

The causes of chronic or recurrent VT include congenital heart disease, both preoperatively and postoperatively; acquired heart diseases; metabolic disorders, including disorders of fatty acid metabolism72; neuromuscular disorders such as Duchenne’s muscular dystrophy; cardiomyopathies, including ARVC; hypertrophic cardiomyopathy (HCM); tumors and infiltrates; left ventricular noncompaction (LVNC), VT originating in both the right ventricle and the left ventricle, associated with structurally normal hearts; VT associated with LQTS; and other primary electrophysiological abnormalities such as Brugada syndrome and CPVT. A detailed list is provided in Table 75-3. While failure to identify a specific cause for VT in children is not unusual, persistence in evaluations often identifies the pathology in patients initially thought to have an unidentifiable etiology.7377

Table 75-3 Etiology of Chronic Ventricular Tachycardia

Congenital Heart Disease Ebstein anomaly
Tetralogy of Fallot, absent PV leaflets
Aortic valve disease, AI/AS
Mitral valve prolapse
Hypertrophic cardiomyopathy/IHSS
Coronary artery anomalies
Eisenmenger syndrome, pulmonary hypertension
Postoperative CHD Tetralogy of Fallot, DORV
Ventricular septal defects
AV canal defects
Aortic valve disease, stenosis, and insufficiency
Single ventricle complexes status post–Fontan repair D-TGA status post–intra-atrial repair
Acquired heart disease Rheumatic heart disease
Lyme disease
Myocarditis
Kawasaki disease
Cardiomyopathies Hypertrophic
RV cardiomyopathy/dysplasia
Dilated cardiomyopathy
Postviral
Connective tissue disease: SLE
Marfan syndrome
Muscular dystrophy, Friedrich ataxia
Tumors and infiltrates Rhabdomyoma
Hemosiderosis: Thalassemia, sickle cell disease
Oncocytic cardiomyopathy
Leukemia
Idiopathic/structurally normal heart RV outflow tract VT
LV septal VT/fascicular tachycardia
Primary arrhythmias LQTS
Congenital complete heart block
Familial VT
Other Myocardial ischemia/infarction

AI, Aortic insufficiency; AS, aortic stenosis; AV, atrioventricular; CHD, congenital heart disease; DORV, double outlet left ventricle; D-TGA, D-transposition of the great arteries; IHSS, idiopathic hypertrophic subaortic stenosis; LQTS, long QT syndrome; LV, left ventricular; PV, pulmonary valve; RV, right ventricular; SLE, systemic lupus erythematosus; VT, ventricular tachycardia.

Mechanism of Ventricular Tachycardia

VT has been reported to result from re-entry, triggered automaticity, and abnormal automaticity. An EPS is helpful in differentiating these mechanisms. The mechanisms of VT in children include re-entry in 60% and abnormal automaticity in 40%.78 Re-entry is most often the mechanism in patients with congenital heart disease after surgical repairs, related to re-entry circuits that develop around suture lines and ventriculotomy scars. Triggered automaticity is thought to be the mechanism in CPVT.

Clinical Correlations

Presentation of patients with VT varies and depends, to a large extent, on the underlying etiology and clinical status with regard to myocardial function and structure. In one study of patients with VT with structurally normal hearts, presentation was most common in infancy (48%), with 58% being younger than 6 months.73 Associated findings were heart failure in 30%, hemodynamic compromise or collapse in 23%, and in utero diagnosis in 18%. Diagnosis was incidental in 30%. No specific etiology was found in 50%, with cardiomyopathy or myocarditis (20%) being the most common etiology identified.

Clinical Signs and Symptoms

The type and degree of symptoms appear to be rate related, with symptoms being most common in patients with rates greater than 150 beats/min. Except for those patients with underlying cardiac disease or ventricular dysfunction, patients with VT have symptoms similar to those of SVT, with the severity of symptoms relating more to the rate than to the mechanism of tachycardia. Symptoms include dyspnea, shortness of breath, chest or abdominal pain, palpitations, dizziness, syncope, and cardiac arrest or sudden death. Older children may exhibit exercise intolerance or easy fatigability. Infants may feed poorly and be irritable or lethargic. Patients with VT and heart disease usually have symptoms, whereas only one third with normal hearts and VT have symptoms. The type of symptom relates to both the tachycardia rate and the underlying state of the myocardium. Sudden death occurs most commonly in the presence of an abnormal heart but has also been reported in patients with normal hearts.74,79,80 Children younger than 5 years or those in incessant tachycardia may not have a perception of a fast heart rate or be able to accurately express what they are feeling. Signs include palpitations, sensation of a rapid heart rate, tachypnea, or hypotension with accompanying pallor and diaphoresis as well as signs of congestive heart failure. Although VT usually has a sudden onset, it may occur during exercise and be difficult to perceive. It may gradually “warm up” or increase in rate.

Specific Associated Conditions

Accelerated Ventricular Rhythm

An accelerated ventricular rhythm is a rhythm originating from the ventricle with all the characteristics of VT but with a rate that is only slightly more rapid than the underlying sinus rhythm, usually less than 120 beats/min. It is often seen in children with normal hearts. This arrhythmia is not uncommon in neonates and has been reported in two patients with fetal tachycardia. It is self-limited, resolving in 2 weeks to 3 months after birth.81 These early ventricular arrhythmias are probably related to developmental factors associated with the autonomic nervous system. In older children, these arrhythmias may be related to unidentified viral infections with myocarditis that affects only the conduction system. This arrhythmia is seen around puberty and probably relates to autonomic and hormonally mediated factors. In addition, accelerated ventricular rhythms have been reported in association with metabolic abnormalities, medication, ARVC, and myocardial infarction.82 In pediatric patients, this arrhythmia is generally thought to be benign, even in the occasional patient who has congenital heart disease.82,83 It has been suggested that those rhythm disturbances arising from the right ventricular outflow tract (RVOT) may be a marker for future development of ARVC in some patients.8284

Arrhythmogenic Right Ventricular Cardiomyopathy

An unusual cause of VT known as arrhythmogenic right ventricular cardiomyopathy or arrhythmogenic right ventricular dysplasia (ARVD) was first described in 1978.86 The VT has a LBBB pattern in most instances. One pediatric series reported ARVC in 30% of its patients with VT and an apparently normal heart, although it is much less common in most other pediatric series. ARVC is a familial form of right ventricular (RV) cardiomyopathy associated with sudden death.87 It has an autosomal dominant genetic pattern with variable penetrance and variable expression. The pathologic lesion involves massive replacement of the RV wall by fibrous tissue, fatty tissue, or both. Focal myocardial changes may be present and include necrosis, degeneration, or hypertrophy and chronic inflammatory infiltrates. This is a progressive process that starts with the epicardium or midmyocardium and extends to become transmural. Although many cases are familial, sporadic cases have been reported as well. ARVC has not been commonly reported in young patients, as it usually presents in the second to fourth decade, with a male predominance, but should be considered in previously healthy children or adolescents who present with VT.

Evaluation

Patients who are suspected of having this condition should have an ECG, echocardiogram, and MRI. Because of the localized nature of this condition, echocardiography may not be diagnostic. MRI may be more helpful by demonstrating thinning of the RV myocardium replaced by fatty tissue or showing localized areas of hypokinesis in the infundibulum, free wall, or RV apex, accompanied by RV dilation and decreased contractility. LV free wall and septal involvement in this process has been noted.88 Although the above findings have been helpful in making the diagnosis in adults, standard MRI criteria applied to 81 pediatric patients suspected of having ARVD provided a low diagnostic yield.89 Other potential diagnostic modalities include exercise testing, contrast ventriculography, signal-averaged ECG, and single photon emission computed tomography (SPECT) analysis.9096 Research on genetic identification is still under way. Immunohistochemical analysis of myocardial biopsies has demonstrated reduced levels of plakoglobin at intercalated discs in patients with ARVC and not in other forms of heart-muscle disease.97 This may prove helpful in establishing a diagnosis of ARVC. Currently, no single gold standard exists, and the best strategy consists of combining information from several diagnostic tests.89 Children in affected families should be evaluated by using ECGs, 24-hour Holter monitors, echocardiograms, and MRIs.77,98

Treatment and Follow-Up

Variable medical therapies, including β-blockers and sotalol, have been suggested for patients with frequent, symptomatic, or potentially life-threatening ventricular arrhythmias. Automatic implantable cardioverter-defibrillators (ICDs) have been used and can be life saving in these patients.99,100 Extensive surgical procedures, including a complete electrical disconnection of the RV free wall, have been reported.101 The prognosis and clinical course reported in these patients have been quite variable. Continued surveillance with periodic Holter monitoring and exercise stress testing are important in the follow-up of this patient group, as the incidence of serious arrhythmias increases with age.

Long QT Syndrome

Congenital LQTS is an inherited condition characterized by syncope, seizures, and sudden death, associated in most individuals with a prolongation of the Q-T interval on the ECG.102 An example of the ECG in LQTS is shown in Figure 75-4. In addition to the prolongation of the QTc, these patients often have bizarre or notched T-wave morphology with prominent U waves or T-wave alternans. They develop life-threatening VT, known as torsades de pointes, or VF. This syndrome includes Jervell and Lange-Nielson syndrome (JLNS), described in 1957, associated with congenital deafness caused by an autosomal recessive inheritance and Romano-Ward syndrome, described in 1963 and 1964, demonstrating autosomal dominant inheritance, without hearing deficit.103105

The prevalence of the disorder is estimated to be 1 : 2000 to 1 : 10,000.106108 This condition has variable expression and penetrance within families, leading to a spectrum of severely affected members with repeated cardiac events and arrest to those with the identical mutation but who are totally asymptomatic.109

In 1993, statistics from a group of 287 children were compiled from a number of medical centers.110 The initial presentation was cardiac arrest (9%), syncope (26%), seizures (10%), presyncope, or palpitations (6%). Sixty-seven percent with symptoms had exercise-related symptoms. Thirty-nine percent were identified because of family history or the identification of other family members with the syndrome. Thirty-nine percent of the patients were asymptomatic at presentation; of these, 4% experienced sudden death compared with 8% overall. The strongest predictors of sudden death were QTc longer than 0.60 seconds and noncompliance with taking recommended medication.

Bradycardia is commonly seen in these patients, and some may develop or present with second-degree AV block. This is more common in neonates who may have second-degree or third-degree AV block, but it may be seen in older children, especially during exercise.111

One series reported sudden death occurring in 73% without treatment, and others have reported sudden death in 21% of symptomatic patients in the first year after presenting with syncope.112,113

Diagnosis and Evaluation

The diagnosis of this syndrome is made from a variety of criteria. Schwartz et al provided some criteria and suggested a scale for identifying these patients by categorizing them into high-risk, intermediate-risk, or low-risk groups.114 All criteria involve measurement of the Q-T interval and taking a careful history of syncope, seizures, and arrhythmias in the patients and their families. Commonly, a complete history may reveal syncope or seizures associated with exercise or emotional stress or a family history of sudden death in young relatives, including unexplained automobile accidents or drowning.

Additional studies such as 24-hour monitoring and exercise stress testing may provide helpful information in the form of significantly prolonged Q-T intervals, especially during recovery from exercise, or the occurrence of polymorphic ventricular arrhythmias during or after exercise. The use of provocative tests such as isoproterenol or epinephrine infusions can identify some subclinical cases, especially LQT1 with a prolongation or paradoxical response in these individuals.115,116

A high level of suspicion is needed to diagnose LQTS. Any patient who presents with syncope during or immediately after exercise or with VT, especially of the polymorphic or torsades de pointes type, or in association with physical or emotional stress should have an ECG, with determination of QTc intervals. Evaluation of a resting ECG may not be sufficient as more than 10% to 15% of gene carriers may have normal ECGs.117 A more worrisome study by Priori showed an even lower penetrance of the gene in Italian families, with probands initially thought to have sporadic occurrences of LQTS. Genetic studies revealed multiple family members who were genetic carriers but with normal ECGs.118 ECGs should be obtained in all family members of identified individuals with LQTS. When genetic mutations are identified, this can be used to screen family members more specifically.

Since a variety of drugs can prolong the QTc, a careful history of medication use should be obtained during the evaluation. Those with prolonged QTc with medication often are subclinical LQT cases or have single nucleotide polymorphisms for LQTS genes.

Clinical Implications of Molecular Genetics

The molecular genetic understanding of LQTS began in 1991.119 Many other genetic discoveries have resulted in the current and rapidly expanding knowledge of the etiology of LQTS.120125 Genetic studies have identified repeated mutations in at least 12 genes that encode for proteins that modulate the ion channels, primarily potassium or sodium channels, causing LQTS by altering cardiac repolarization and increasing the risk for ventricular arrhythmias. Mutations in a few other genes have been found in only isolated individuals. It is estimated that 25% of the gene mutations have not yet been identified. The mutations are primarily in the coding regions of the genes. These mutations result in a decrease in repolarizing potassium currents prolonging repolarization or in late entry of sodium or calcium into the cardiac cell prolonging depolarization or repolarization. Both of these mechanisms prolong the Q-T interval. These genes that involve sodium and potassium currents include KCNQ1(IKs), KCNH2(IKr), minK, and MiRP1, and SCN5A and represent LOT1, 2, 5, 6, and 3, respectively.122127 Syndromic LQT genetic disorders include LQT7, otherwise known as Andersen-Tawil syndrome, associated with skeletal malformations and periodic paralysis, caused by mutations in the KCNJ2 gene with reduced Kir2.1 currents and LQT8, or Timothy syndrome, associated with syndactyly, and mutations in the CACNA1C gene with increase in the Cav1.2 current. LQT9 is associated with mutations in the cavelolin-3 gene, and LQT10 is caused by a mutation in the SCN4B gene, both with late increases in sodium currents. Other mutations have been found in the ankyrin-B gene causing malfunction in a cytoskeletal membrane adapter (LQT4). LQT11 is associated with mutations in AKAP9 and LQT12 with mutations in SNTA1. In addition to these genes that affect ionic channels altering the repolarization phase of the cardiac action potential and resulting in the development of ventricular arrhythmias, an imbalance or oversensitivity of the myocardium to sympathetic stimulation appears to play a role in the development of ventricular arrhythmias. The trigger for arrhythmia in the LQTS is believed to be spontaneous secondary depolarizations that arise during or just following the prolonged plateau phase of action potentials, early after-depolarizations (EADs). Increased sympathetic tone may increase EADs, with these spontaneous repolarizations triggering a sustained arrhythmia. The implications of the location of the mutations, coding type, and the resulting biophysical malfunction has been well described for LQT1 mutations.128 Mutations located in the transmembrane portion of the ion channel protein and the degree of channel malfunction independently affect the clinical course of the individual.

Specific Genetic Defects

KvLQT1 (LQT1) and MinK (LQT5)

The KCNQ1 gene encodes the voltage-gated potassium channel α-subunits.126,129 MinK (KCNE1) encodes a much smaller potassium channel β-subunit. MinK subunits assemble with KCNQ1 subunits to form cardiac IKs channels.124,130,131 Abnormalities of either or both of these genes inhibit channel function and prolong repolarization by affecting IKs by a greater than 50% reduction in channel function, which is a dominant-negative effect. Other mutations may reduce repolarizing potassium currents by causing trafficking defects and interfering with the transport of the subunits to the cell membrane, resulting in an up to 50% reduction in channel function (haplo-insufficiency). Current evidence suggests that more than 40% of affected LQTS families have KCNQ1 mutation.132 Approximately 5% of mutations identified to date have involved minK.132,133 It has been reported that a homozygous mutation of KCNQ1 or minK causes JLNS.134,135 Both KCNQ1 and minK are expressed in the inner ear. Homozygous mutations of KCNQ1 or minK have no functional IKs channels. This leads to inadequate endolymph production and deterioration of the organ of Corti with neural deafness.136

KCNH2 (LQT2) and MiRP1 (LQT6)

In 1994, Jiang et al identified the human ether-a-go-go-related (HERG) gene, now referred to as KCNH2.127 These mutations represent 45% of the total number of LQTS mutations found to date. This gene encodes for the α-subunits that form the cardiac potassium channel delayed rectifier IKr channel, the second of two channels responsible for the termination of the plateau phase of the action potential.137,138 These mutations result in decreased outward potassium current, preventing termination of the plateau phase of the action potential. KCNH2 subunits assemble with the MiRP1 (minK-related protein1), also known as KCNE2, to form cardiac IKr channels.125 MiRP1 mutations represent 2% of the identified LQTS mutations. Multiple drugs are known to prolong the Q-T interval and potentially induce arrhythmias. The structure of KCNH2 channels appears to be predisposed to blockage by multiple drugs, making this the channel most commonly blocked by drugs.139

SCN5A (LQT3)

Jiang et al reported an additional group of LQTS families with mutations in the human cardiac sodium channel gene that encodes the subunit of sodium channel responsible for initiating cardiac action potentials.122,140,141 Gain of function mutations, especially in the inactivation gate between domains III and IV of the sodium channel, have been reported with abnormal gain of function, resulting in continued inward sodium current prolonging the action potential and predisposing to ventricular arrhythmias.133,142

Special Considerations in Long QT Syndrome

Vincent’s study in 1992 indicated that some patients may be genetic carriers for this syndrome without significant prolongation of the Q-T interval.117 Some noncarriers (15%) have abnormal prolongation of the Q-T interval above 0.44 seconds. A QTc of more than 0.47 seconds had a 100% positive predictive value in gene carriers. Above 0.47 seconds, no false-positives were seen in this study. Only 6% of gene carriers had a QTc of less than 0.44 seconds.117 These genetic tests may make it possible to identify more specifically many patients with LQTS. Priori identified families with only a 25% penetrance, and 33% of family members considered to be unaffected on clinical grounds were found to be gene carriers.118

Another interesting association that has come from the International Registry relates to the association of asthma and LQTS. The occurrence of asthma in LQTS patients increases with QTc duration. Asthma comorbidity in LQTS patients is associated with an increased risk of cardiac events. This risk is diminished after initiation of β-blocker therapy.144

A special group of LQTS patients are newborns. Studies have suggested that sinus bradycardia is more likely associated with LQT1 and 2 : 1 AV block with LQT2 or LQT3.145,146 2 : 1 AV block has been associated with a high mortality rate,145,147 but more recent studies show this can be moderated.147a An example of 2 : 1 AV block in association with LQTS is shown in Figure 75-5.

Jervell Lange-Nielsen Syndrome

JLNS is the general descriptor applied to LQTS associated with a hearing deficit. It is caused by homozygous or compound heterozygous mutations in the KCNQ1 or KCNE1 genes, resulting in a reduced IK current and associated sensorineural deafness.134,148 Most (85% to 93%) of these patients experience cardiac events, and 50% are symptomatic by age 3 years.149 Events are generally triggered by emotions or exercise. Many have events despite β-blocker therapy, and early consideration of defibrillators has been advised.

Gene-Specific Clinical Correlations

ST-T wave changes have been associated with specific genetic mutations, as shown in Figure 75-6. Because of an overlap, the specificity of this finding is limited.150,151 The influence of genotype on clinical course is being elucidated.150 The frequency of cardiac events is higher among subjects with LQT1 (63%) or LQT2 (46%) than among patients with LQT3 (18%). The likelihood of dying during a cardiac event is higher among patients with LQT3 (20%) than among patients with LQT1 or LQT2 (4%). Cardiac events in patients with LQT1 occur frequently during exercise (62%), especially swimming.152154 Only 3% occurred during sleep. Among patients with LQT1, around 53% experience the first event by age 15 years, with 86% becoming symptomatic by 20 years of age. The probability of a cardiac event increases during adolescence in all three of the major genotypes (LQT1, LQT2, and LQT3), with the risk being higher in males before puberty and higher in females after puberty. In those with LQT2, symptoms are most commonly triggered by emotions (43%) or auditory stimuli (26%). While some events do occur with exercise (37%), more occur during rest (49%) or sleep (29%). The average age of first manifestation of symptoms is 16 years. Pregnancy increases the risk, particularly in women with LQT2, for up to 9 months after delivery. In those with LQT3, symptoms are less commonly triggered by exercise (13%) and occur primarily at rest (39%). The median age of presentation is 16 years. The percentage of patients who are free of recurrence with β-blocker therapy is higher, and the death rate is lower among patients with LQT1 (81% and 4%, respectively) than among those with LQT2 (59% and 4%) and LQT3 (50% and 17%).152 Patients with LQT3 have more cardiac events at rest or during sleep, whereas patients with LQT2 experience more events during exercise or stress. LQT2 events are more likely to be stimulated by loud noises.155

Risk Factors for Long QT Syndrome

High-risk factors for patients with LQTS include a QTc greater than 0.50 seconds to greater than 0.53 seconds, aborted cardiac arrest, torsades de pointes or complex ventricular arrhythmia, recent syncope (>2 times in past 2 years), male gender and ages 10 to 12 years, and noncompliance with prescribed LQTS medications. While younger males with LQT1 have the highest risk, no gender difference exists between young individuals with LQT2 and those with LQT3. In adulthood, a higher risk of cardiac events is present in females than in males with LQT1 and LQT2. The risks of lethal events are highest in males with LQT3 (19% risk) and females with LQT3 (18% risk), higher in males with LQT1 and LQT2 (5% and 6% risk, respectively) than in females with LQT1 and LQT2 (2% risk for either). Additionally, risks are higher in individuals with a QTc greater than 0.53 seconds, in individuals with recent syncope (within 2 years), and in individuals with frequent syncope (>2 times).152,156 The location of the mutation in LQT2 imparts higher risk to those with pore region mutations, whereas the site of the mutation appears to have less impact in LQT1.157 A study evaluating the risks of aborted cardiac arrest and sudden cardiac death in children found that a QTc greater than 0.50 seconds and syncope in males and syncope in females conferred the greatest risks.157

The probability of a first cardiac event before the age of 40 years and before therapy can be classified as high-risk, intermediate-risk, and low-risk by the characteristics illustrated in Figure 75-7.158 In LQT1 and LQT2, the risk of dying during a cardiac event is 4%, with the risk of death being 4% overall. In LQT3, the risk of dying is 17%, with the risk of dying during an event being 20%.

image

FIGURE 75-7 Risk stratification by gender, genotype and QTc.

(From Prior SG, Schwartz PJ, Napolitano C, et al: Risk stratification in the long-QT syndrome, N Engl J Med 348:1866–1873, 2003.)

Sudden Infant Death Syndrome

Schwartz reported on ECGs on 34,442 Italian babies on days 3 or 4 of life; 24 subsequently died because of sudden infant death syndrome (SIDS) and 12 had prolongation of the Q-T interval greater than 0.44 seconds.159 Additional reports have confirmed SCNA5 and HERG mutations in a few babies who died from SIDS.133135 Postmortem molecular studies in infants and children with autopsy-negative sudden unexplained death found that 10% to 30% had genetic mutations for one of the electrical conditions associated with SCA.160

Evaluation and Diagnosis of LQTS

Diagnosis is made by taking a careful history of the affected individual’s episodes as well as a complete family history to identify sudden unexplained death, syncopal episodes in family members, unusual seizure disorders, or hearing deficits. All patients suspected of LQTS should have a standard ECG with careful QTc measurement, 24-hour Holter monitoring, and exercise stress testing, as appropriate for age. The chapter authors do not routinely use other provocative testing such as isoproterenol or epinephrine infusions, reserving it instead for isolated cases with a high index of suspicion but negative testing otherwise.

Careful evaluation of the ECG and 24-hour Holter monitoring is essential. The QTc is measured manually by using Bazett’s formula.161 The longest Q-T interval in any lead is divided by the square root of the preceding R-R interval. The measurement should be made manually and calculated, as computerized values are frequently incorrect. The authors consider a value greater than 0.46 seconds in any lead in children as abnormal on the resting or exercise ECG. Although a number of methods have been proposed for calculating the QTc in the presence of sinus arrhythmia, the authors make every attempt to record an ECG not in sinus arrhythmia.162 In addition to the Q-T interval, each lead of a standard 12- or 15-lead ECG should be evaluated for abnormal T-wave morphology and ST morphology. As different filters are used on the Holter monitor, the authors consider a value greater than 0.50 seconds as abnormal. In addition to Q-T interval prolongation, the Holter monitor may be helpful in illustrating T-wave abnormalities, R-on-T phenomenon, short runs of nonsustained VT, sustained VT, or torsades de pointes.

Provocative testing should include exercise stress testing in children who are able to exercise. Exercise will generally obliterate sinus arrhythmia; a strip can be obtained and a reasonable QTc calculation made. The recovery period with heart rates around 120 to 130 beats/min seems to demonstrate the greatest degree of QTc prolongation in many patients. Exercise may uncover abnormal T waves, polymorphic PVCs, or VT. If suspicion for LQTS is high and other testing has not been definitive, isoproterenol or epinephrine infusion may help identify LQTS. During these provocative tests, T-wave abnormalities may occur in addition to QTc prolongation or the development of ventricular arrhythmias.157

Efforts to identify patients at high risk for syncope and sudden death continue. High-risk ECG markers have included a QTc greater than 0.53 seconds, T-wave alternans, and QTc dispersion.163,164 Dispersion of the Q-T interval has been correlated with high risk in patients with LQTS.163,165,166 QT dispersion, which indicates heterogeneity of repolarization, could predispose to the development of torsades de pointes. In Priori’s study, patients not responding to β-blockers had a significantly higher dispersion of repolarization than did responders.163 In Shah’s study, patients with LQTS at high risk for developing critical ventricular arrhythmias had a QT or JT dispersion more than 55 ms.166 Little information is available on microvolt T-wave alternans in patients with LQTS, although visible T-wave alternans is known to be a high-risk factor.

Treatment

Emergent treatment of these patients includes lidocaine and cardioversion. Magnesium may be used to treat torsades de pointes.167,168 Intravenous propranolol and phenytoin have also been successfully used in these patients.169 Class I agents, which are known to prolong the Q-T interval in normal patients, should be avoided in patients with LQTS. This is felt to be related to QTc prolongation with associated bradycardia, ventricular arrhythmias, or both. Temporary pacing and removal of the offending agent are effective measures in this situation. Sudden death is secondary to the ventricular arrhythmias (torsades de pointes) of the type shown in Figure 75-8, which frequently degenerate to VF. The standard long-term treatment in this condition is the use of β-blockers. Those most commonly used are propranolol and nadolol. Some authors have suggested long-acting propranolol or atenolol. A concern about once-daily dosing relates to the lowest levels of the medication being present in the early morning hours, a particularly high-risk time for some patients. Therefore, we suggest twice-daily dosing at least in this group of patients. One study did suggest a less-than-favorable result with atenolol.170 In patients with compliance issues, once-daily β-blocker would be preferable to no medication. The dose of β-blocker required is variable and is usually greater per kilogram in younger patients. Most teenagers only require 10 to 20 mg of nadolol twice daily. The authors titrate the appropriate dose on the basis of the heart rate response to maximal exercise testing, aiming for a blunted maximal heart rate response of 150 to 160 beats/min on therapy. The prevalence of sudden death decreases from more than 50% to 1% to 2% with β-blockers.171,172 Other series indicate that β-blockers are not protective in every segment of this population and are most effective in patients with LOT1.173 While β-blockers are less effective in LQT2 and even less in LQT3, as no harm has been proven with this regimen, the authors continue to use them in all patients, with ICD backup when indicated clinically. The authors have added mexiletine to the therapeutic regimen of many of their patients with LQT3. Their patients are followed up yearly or twice yearly with exercise stress tests and Holter monitoring to establish the adequacy of treatment, the development of significant ventricular arrhythmias, or both. Patients who do not respond to β-blockers may be treated with mexiletine, phenytoin, or pacing. Rarely, other antiarrhythmics may be used, but those known to prolong the Q-T interval should be avoided. Potassium therapy may be helpful, especially in patients with LQT2, in which case potassium should be maintained above 4 mmol/L.

Left cervico-thoracic sympathetic denervation (LCSD) is a controversial treatment with variable success but may be useful in individuals whose condition is refractory to drug or ICD therapy.174177 Permanent pacing, often in association with an ICD, has been shown to be an effective adjunctive treatment in these patients, especially those with severe bradycardia either from the syndrome itself or from the β-blocker therapy.178,179 The rate of the pacing should be at least 10% to 20% higher than the sinus rate and in severe cases should control the rhythm as much of the time as possible. Pauses should be avoided. Episodes of torsades de pointes may be reduced or eliminated with this treatment. In patients known to have had a cardiac arrest or frequent or significant syncope associated with ventricular arrhythmias, the chapter authors recommend the implantation of an automatic ICD. These devices can recognize VT or VF according to programmed criteria and provide a series of shocks to convert the patient’s rhythm to normal (sinus rhythm). Some devices can provide backup pacing, but at present, these are not appropriate for continuous higher rate pacing, as the battery is depleted prematurely. The sizes of the devices led to limited use in smaller children, but improved technology now allows even small children to benefit from this technology. This is not a therapy to be undertaken lightly at this time, as the need for constant follow-up and inappropriate discharges from the device can significantly affect a child’s life and lifestyle.180 Groh reported on 35 patients with LQTS who had ICDs and were followed up for a mean of 31 months.94 The major indication was aborting of sudden death. Sixty percent of patients had at least one appropriate discharge in the follow-up period. Two patients had multiple discharges and required additional therapies. None of the patients died. These results were similar to those reported earlier by Silka.181

A greater understanding of the molecular mechanisms of LQTS has prompted a number of studies to identify more specific or gene-directed therapies.182 The sodium channel blocker mexiletine has been used in some patients.183 The Q-T interval was noted to decrease with mexiletine treatment in patients with LQT3. Sato et al had reported using a potassium channel opener, nicorandil, in a patient with LQTS.184 Trials are under way with potassium supplementation and spironolactone. An LCSD has been recommended in patients with recurrent cardiac events and repeated appropriate ICD discharges. The benefit of this is still being debated.

It is generally recommended that competitive athletic activities be avoided by patients with LQTS. With regard to those with documented LQTS and symptoms or arrhythmias, the chapter authors would certainly agree with this recommendation. However, as more “carriers” or asymptomatic patients who have only a prolonged Q-T interval, no arrhythmias, and no family history of sudden death or ventricular arrhythmias are being identified, individual exercise and sports participation recommendations may be made. The most important aspect of the care of these patients is continued surveillance. This is true for young family members who appear to have normal Q-T intervals on initial evaluation. The authors have seen the Q-T interval change with age and would recommend periodic ECGs and appropriate 24-hour and exercise ECG in children and adolescent members of families with LQTS who have initial negative results of evaluation unless genetic testing has definitively ruled out LQTS.

In the past, it was considered that adults were at extremely low risk and did not need medication, but this has been shown to be incorrect. Therapy is now recommended in most adults, especially in those who have had symptoms throughout their lives.185,186 It is recommended that patients with LQTS avoid caffeine, adrenergic stimulants such as epinephrine, and over-the-counter stimulants such as decongestants. Medications that prolong the Q-T interval should be avoided. A list of these medications can be found at the website www.qtdrugs.org.

Short QT Syndrome

In 2000, Gussak reported a familial short Q-T interval associated with AF. Subsequent reports associated a short Q-T interval with sudden death. In 2004, Brugada found mutations in KCNH2.187 Short QT syndrome (SQTS) is diagnosed by a short Q-T interval of less than 0.32 seconds or QTc less than 0.34 seconds associated with AF, syncope, and sudden death. Additionally, tall symmetric T waves are present, as illustrated in Figure 75-9. Five forms of SQTS have been found with mutations in KCNH2, KCNQ1, and KCNJ2 predominantly resulting in gain of function and shortened repolarization. Additional mutations in the CACNB2B and CACN1C genes encoding the subunits of cardiac L-type calcium channels have been reported. ICD implantation has been recommended as the treatment.187189 Quinidine has been suggested as an effective therapy, particularly in the young.190

Brugada Syndrome

The association of an ECG pattern of RBBB and ST segment elevation in ECG leads V1 to V3 with sudden death, which was reported in 1992, has been labeled Brugada syndrome.191 Patients die during sleep, which is presumed to occur secondary to VF. The average age of diagnosis is 44 years, although this condition has been reported in a 2-day-old and linked to SIDS.192,193 The syndrome is both sporadic and inherited, with an autosomal dominant mode. A mutation of SCNA5 causes loss of function with slowing of conduction velocity.194 Mutations in the SCN5A gene leading to loss of function account for approximately 18% to 30% of Brugada syndrome cases.192,195 A newly identified gene called glycerol-3-phosphate dehydrogenase 1-like gene (GPD1L), which results in a partial reduction of INa, has been associated with the syndrome.196 The syndrome occurs most commonly in males (3 : 1) and Asians. Presentation in childhood is uncommon, with a series of 30 children finding symptoms in only one third, most identified during a family evaluation.197,198 Fever was the most common precipitating factor. Patients with unexplained syncope or aborted sudden death or a family history of these occurrences should be evaluated for this condition. More recent reports indicate that approximately 20% of patients with Brugada syndrome develop supraventricular arrhythmias.198a A meta-analysis by Gehi suggested that a prior history of syncope or aborted sudden cardiac death, male gender, and a spontaneous typical type I Brugada ECG (coved ST-segment elevation >2 mm) are predictors of high risk.198b

Evaluation and Treatment

Patients with this condition have normal Q-T intervals, ST-segment elevation with a “coved” appearance, RV conduction delay, and a propensity for sudden death. Sodium channel blocking agents such as procainamide or flecainide have been used to unmask this condition.197,199 Placement of the precordial leads several intercostal spaces higher than usual may unmask the condition as well. In children, the condition also may be unmasked by fever; vagotonic agents or events; or medications, including tricyclics or β-blockers; and recreational drugs such as cocaine.192,198 Antiarrhythmics have not decreased the incidence of sudden death in these patients, and implanted defibrillators are often recommended, although this remains controversial, as does the use of inducible VT in the electrophysiology laboratory.192,195,200,201 Some evidence exists that quinidine may be effective in children too young for ICD implantation who present with potentially threatening symptoms.202

Cathecholaminergic Polymorphic Ventricular Tachycardia

Cathecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder associated with stress-induced, bi-directional VT that may degenerate into VF and result in sudden death. This condition usually occurs in childhood, adolescence, or young adulthood, with an estimated prevalence of 1 : 10,000.203 Reports of mutation in the cardiac ryanodine receptor gene RyR2 with autosomal dominant inheritance have been found in 70% of families with catecholamine-induced VT.204 Abnormalities of this gene would result in an abnormality of intracellular calcium handling, which leads to calcium overload and tachyarrhythmias. An autosomal recessive form of CPVT has been reported to be associated with mutations in the CASQ2 gene, which encodes calsequestrin, a protein that is the major calcium (Ca++) reservoir within the sarcoplasmic reticulum.205 Mutations can be identified in 50% to 70% of cases.206 The proposed mechanism is triggered automaticity. The initial symptom is usually syncope triggered by emotional or physical stress. The mean age of onset is between 7 and 9 years.206 Approximately 30% have a positive family history of syncope or sudden death. Over 70% also have supraventricular arrhythmias.203 Patients with CPVT frequently have associated sinus node dysfunction and inducible atrial tachyarrhythmias, which indicates that the pathogenesis of CPVT is associated with not only the ventricular myocardium but also with broad regions of the heart, including the sinus node and atrial muscle.207

Myocarditis

Patients with myocarditis present another special problem. Of patients with ventricular ectopy, 14% to 50% have been shown to have histologic evidence of myocarditis.210,211 The most common causes of viral myocarditis include coxsackie A and B viruses and adenovirus. A large number of these patients present with ventricular arrhythmias, usually single PVCs or nonsustained VT and only mild or no impairment of ventricular function.

Evaluation and Treatment

Resting ECGs may show ST-T wave changes or low-voltage QRS in addition to PVCs or VT. 24-hour Holter monitoring will indicate the extent and complexity of the arrhythmia. In a hospitalized patient, telemetry monitoring may pick up runs of sustained or nonsustained VT or periods of AV block.

Steroid therapy has been beneficial in some patients.212 The use of IV immunoglobulin may help in the recovery of LV function and improve survival in the first year after presentation in these patients.213 In instances of simple ventricular arrhythmias, no treatment may be needed. Patients with more complex or frequent arrhythmias may need treatment. The chapter authors have used β-blockers or mexiletine successfully in these patients. Some patients have slow rates from sinus node dysfunction or AV block and develop rapid ventricular arrhythmias as the heart rate slows. Temporary pacing may be necessary in these patients and may result in the control of the ventricular arrhythmia without the use of pharmacologic agents. When ventricular arrhythmias in these patients are potentially life threatening or impair ventricular function, immediate treatment may be needed. Often, these patients with diminished myocardial function require inotropic support to maintain cardiac output. Although each patient has unique sensitivities, a supportive agent with the least arrhythmogenic characteristics should be chosen, if possible. For example, dobutamine is usually less arrhythmogenic than dopamine, which, in turn, is less arrhythmogenic than epinephrine or isoproterenol. Ventricular arrhythmias may occur in patients with myocarditis and associated complete heart block and slow escape rhythms. In these instances, an increase in the heart rate with a temporary transvenous pacemaker may be all that is needed to control the ventricular arrhythmia. In general, pressor agents should not be used just to increase the heart rate because of their arrhythmogenic potential in this subset of patients.

Postoperative Ventricular Tachycardia

With few exceptions, patients who undergo intracardiac surgery risk the development of postoperative arrhythmias and conduction defects.214 A history of previous surgery on the ventricle or previous elevation of LV pressure before surgery is seen in most patients with VT after surgery for congenital heart disease. Most of the instances of sudden death in these postoperative patients are seen after repair of aortic stenosis, coarctation, transposition of the great arteries, or tetralogy of Fallot.215

Management and Treatment of Patients with Postoperative Ventricular Tachycardia

Because of their high incidence in postoperative patients, the precise role of ventricular arrhythmias in the occurrence of sudden death is unclear. It is known that after repair of tetralogy of Fallot, exercise stress testing or Holter monitoring will uncover a 25% to 70% incidence of ventricular arrhythmias. In 1985, Garson reported that treatment of more than 10 PVCs per hour on Holter monitoring decreased the incidence of sudden death in their study population.218a In contrast, Sullivan and others have reported no increase in the incidence of sudden death by not treating similar patients.219 A definitive conclusion is still pending as no large controlled study of these patients has been performed yet. The presence of frequent or complex ventricular ectopy probably identifies a high-risk group, but at present, the ability to further identify patients at highest risk is limited. It appears that patients with QRS duration above 180 ms and severe pulmonary regurgitation represent the high-risk group. The best time for valve replacement in children has not yet been determined.

Although no complete agreement as to the indications for treatment exists, the chapter authors have adopted a policy of treating patients with clinical episodes of VT or with symptoms and inducible VT. They generally treat patients with complex ventricular arrhythmias and abnormal hemodynamics or patients with significant symptoms, abnormal hemodynamics, or both. We use the EPS to determine the efficacy of specific drug regimens or the need for ICD implantation in patients with ineffective drug therapy or hemodynamic deterioration.

Treatment can include a combination of pharmacologic agents, radiofrequency ablation, surgical repair, and ablation or implantation of a pacemaker or an ICD. The most commonly used drug regimens include β-blockers, mexiletine, or class IA or IC agents. In the 1970s, phenytoin was found to be an effective drug in this population, but mexiletine is more commonly used at present. Amiodarone has been found to be an effective drug in some patients with refractory disease. Recently, surgical or catheter ablation has been used effectively in these patients. ICDs are used in patients who have had syncopal events or aborted sudden death or those in whom unstable VT or VF is induced in the electrophysiology laboratory.

Ventricular Tachycardia and Tumors

An association is known to exist between VT and cardiac rhabdomyomas. An incessant form of VT has been reported in infants and young children secondary to myocardial hamartomas. These patients can be treated successfully with surgical ablation.220 The chapter authors have found that aggressive medical management, including combination drug therapy, may be used to control this type of VT. These tumors and rhabdomyomas may regress over time. A more diffuse infiltrative type of disease known as histiocytoid or oncocytic cardiomyopathy of infancy is known to manifest as incessant VT in infancy and is usually fatal.221 Any patient with a diagnosis of tuberous sclerosis, which is known to be associated with cardiac rhabdomyoma, should have an ECG and echocardiogram done. If tumors are noted, 24-hour Holter monitoring is necessary.

Ventricular Tachycardia and Mitral Valve Prolapse

In adults, the incidence of sudden death associated with mitral valve prolapse (MVP) is 1.4%.222 Sudden death has been reported in children and adolescents, especially in athletes with MVP.223 The sudden death is proposed to be secondary to ventricular arrhythmias.224 One study found MVP in 25% of patients with idiopathic VT. On follow-up, sudden death did not occur in any patient.224a Twenty-four-hour monitoring has revealed PVCs or more complex ventricular arrhythmias in as many as 46% of children with MVP.225 Twenty-three percent of these arrhythmias were considered life threatening. Exercise stress testing revealed serious ventricular arrhythmias in 20% of patients. β-Blockers have been the drugs of choice for the control of these arrhythmias. The incidence of these events was 0.32% per year. Predictors of adverse outcome included an enlarged left ventricle or left atrium, MVP with thickened mitral leaflets, or cardiovascular symptoms.225

Although no large or long-term follow-up studies have been performed in pediatric patients with MVP, studies in relatively young adults have been performed and have shown a small but significant risk. Four hundred four U.S. Air Force aviators with a diagnosis of MVP were followed up for a mean of 8.6 years and evaluated for “suddenly incapacitating events” described as sudden cardiac death, syncope, pre-syncope, and cerebral ischemic episodes.225a

Ventricular Tachycardia and Cardiomyopathies

Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is the most common inherited cardiovascular disorder affecting 1 in 500 individuals in the general population.227,228 A high incidence of sudden death is seen in children with hypertrophic cardiomyopathy (HCM), also known as idiopathic hypertrophic subaortic stenosis (IHSS), which is presumed secondary to ventricular arrhythmias.229232 HCM is the most common cause of sudden death in young competitive athletes.229,233 The clinical manifestations are a result of cellular disarray, fibrosis, and hypertrophy of the myocyte leading to diastolic dysfunction, myocardial ischemia, and arrhythmias.234,235

Clinical Correlations

A family history of ventricular arrhythmias leading to sudden death, including nonsustained VT on 24-hour Holter monitors, previous syncope, extensive generalized hypertrophy, or ventricular systolic or diastolic dysfunction, may identify a high-risk population.232 Asymptomatic VT on Holter monitoring may or may not be predictive.236 Abnormal blood pressure response to exercise may predict sudden death. Exercise stress testing may uncover ST-segment depression or an abnormal systolic blood pressure response, including a small difference between peak and resting systolic blood pressure. Predictors of sudden cardiac death include higher left ventricular outflow tract (LVOT) pressure gradient at rest and failure of systolic blood pressure to increase during exercise testing.237 Two events that predict subsequent sudden death include a combination of inducible sustained VT at EPS and a history of cardiac arrest or syncope.235 A multi-center study evaluating 1511 consecutive patients with HCM demonstrated a relative risk of sudden death of 1.78 in patients with unexplained syncope and 0.91 with neurally mediated syncope compared with patients without syncope. A fivefold increased risk was seen in patients with unexplained syncope within 6 months of their initial evaluation. Of the 1511 subjects, 147 were younger than 18 years of age. During 6.5 years of follow-up, 15 sudden deaths occurred, that is, an incidence of 15.7 per 1000 person-years. Seven of the 147 patients had experienced unexplained syncope before their initial evaluation, with 3 dying suddenly. In those with syncope, the mortality rate was 120 per 1000 person-years compared with 13 per 1000 person-years in those without unexplained syncope.236 More than one third of patients who experience syncope with VT/VF or resuscitated VF will die within 7 years from sudden cardiac death or progressive heart failure.237 Unfortunately, many high-risk patients will not have inducible VT at EPS but may still be at high risk. It has been suggested that fractionation of electrograms with programmed stimulation may be a marker for high-risk patients.238 Recent studies suggest that myocardial bridging of the left anterior descending coronary arteries may cause myocardial ischemia in children with HCM.239 The primary problem with regard to treatment is the divergence of opinion regarding the bridging or compression of branches from LV hypertrophy.240

HCM is a genetic disease caused by mutations in contractile sarcomeric proteins. The penetrance is often incomplete and 20% to 30% of adults with these gene mutations do not express the phenotype.241 It is hoped that the recent genetic identification of HCM mutations will increase understanding and help identify high-risk patients.240 The first gene identified for HCM is the β-myosin heavy chain MHC located on the long arm of chromosome 14.242 The MHC gene (MyHC) is responsible for 35% of familial HCM.243 Multiple other genes that code the proteins of the sarcomere have been found to be responsible for HCM. These include the gene of cardiac T-troponin (cTnT),241 the gene of α-tropomyosin, and the gene of binding protein C (MyBP-C).244 Specific mutations have been reported to be associated with a higher incidence of sudden death, but this is not fully agreed upon.242

Evaluation

All family members and identified patients should be monitored with periodic ECGs and echocardiograms. ECG findings have been identified as major criteria in the diagnosis of hypertrophic cardiomyopathy, including left ventricular overload, deep Q waves more than 40 ms in the LV inferior lateral wall, and T-wave inversion 3 mm or more in V3 to V6, L1, and aVL and 5 mm or more in L2, L3, and aVF (Figure 75-10).244 In identified patients, yearly Holter monitoring should be performed. Exercise stress testing should be performed in those who are of an appropriate age. EPS should be performed in those who have had syncope, documented complex arrhythmias, or symptoms suggesting ventricular arrhythmias. It has been noted that atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) are elevated up to 5 and 85 times the normal levels, respectively, in patients with HCM.245 These values may be useful in the initial evaluation as well as follow-up in those with a diagnosis of HCM.

Treatment

Treatment includes antiarrhythmic therapy, “pacemakers,” myotomy or myectomy, and ICDs.170,246251 Maron reported a retrospective multi-center study of efficacy of ICDs in preventing sudden death in 128 patients with HCM who were judged to be at higher risk for sudden death and had ICDs implanted. Twenty-three percent had appropriate shocks or anti-tachycardia pacing, and 7% had inappropriate shocks per year. In those in whom secondary prevention was provided after cardiac arrest or VT, 11% had appropriate activation. The interval between implantation and the first appropriate discharge was substantially prolonged by 4 to 9 years in six patients. The defibrillators were highly effective in terminating life-threatening ventricular arrhythmias.251a The recommendations of the 36th Bethesda Conference are that athletes with a diagnosis of HCM or probable HCM abstain from competitive sports and vigorous training, with the exception of low-intensity activities.252

Dilated Cardiomyopathy

The most common etiology of dilated cardiomyopathy (DCM) in children is idiopathic,253 but other causes include antecedent myocarditis, familial or genetic associations, and immune regulatory abnormalities.253256 The prognosis for children with DCM is poor, and the reported mortality has been estimated at 24% to 50%. In a study of 62 children and adolescents (followed up for 3.9 to 4.5 years), 50% died, 16% recovered, 27% had residual decreased LV function, and 7% underwent orthotopic heart transplantation.257 In another study of 41 children followed up for a median of 2.5 years, 30% died, 32% recovered, and 38%survived with decreased LV function.258 Mortality rates at follow-up were 24% at 1 year and 29% at 5 years. Poorer prognosis was associated with clinical severity at presentation, lower mean shortening fraction, and severe arrhythmias.258 Little information is available on risk stratification in children with dilated cardiomyopathy.

Treatment

Treatment includes antiarrhythmic therapy, biventricular pacing, and implantable ICDs. Ten percent of patients will require heart transplantation. In the past decade, a number of pediatric studies have looked at the effect of β-blocker therapy in the treatment of pediatric patients with dilated cardiomyopathy.260262 These studies have primarily evaluated the safety and effectiveness of carvedilol in this population. Each of these has demonstrated a significant improvement in systemic ventricular function as assessed by shortening, ejection fraction, or both. Carvedilol was added to other standard therapeutic modalities in each of these studies. The use of carvedilol, other β-blockers, or both in patients who develop significant arrhythmias may result in an interaction with other antiarrhythmics such as amiodarone.

Biventricular pacing has been reported recently as a successful treatment modality in adults with symptomatic drug-refractory heart failure secondary to dilated cardiomyopathy and associated interventricular conduction delay.263,264 Several electrophysiologists are recommending a combination of biventricular pacing, implantable ICDs, or both for adults.251,265 The usefulness of biventricular pacing in pediatric patients with a dilated cardiomyopathy, interventricular conduction delays, ventricular asynchrony, and congestive heart failure has not been studied in large clinical trials but may be a new treatment option for these patients. Several large multi-center trials of biventricular pacing are ongoing, including trials to evaluate the effects of biventricular pacing on ventricular arrhythmias.263,264,266 Although early results in adults are encouraging, many questions still remain to be answered.

Ventricular Tachycardia and Structurally Normal Hearts

Two sites have been associated with VT and structurally normal hearts. The first is located in the right ventricular outflow tract (RVOT), usually in the anterior portion, most commonly anteroseptal, but also in the anterolateral and anterior regions.267 The morphology of VT on ECG is LBBB, most commonly with an inferior axis but also with a superior or rightward axis (Figure 75-11). An RBBB pattern with superior or rightward axis is also seen. Both sustained and nonsustained VT are seen clinically. It has been suggested that RVOT tachycardias may resolve over time. However, Drago reported biopsies positive for acute myocarditis, ARVD fatty infiltration, and other histologic abnormalities in 67% of patients without obvious heart disease and RVOT tachycardia.269 The tachycardia may be induced as nonsustained or sustained VT by programmed electrical stimulation and isoproterenol in 40% to 80% of selected patients. Pacemapping can help identify the specific site of VT in the RVOT.

The second form of VT in patients without heart disease originates from the left ventricle, most commonly in the septum in the mid-to-inferior position. The morphology of the VT is generally RBBB with left axis deviation (Figure 75-12). It can be induced in a similar fashion to VT in the RVOT. These VTs are often sensitive to verapamil. VT from the basal aspect of the superior LV septum can appear as repetitive monomorphic VT, revealing a dominant R-wave pattern in V1, an inferior axis, and a precordial R-wave transition at or before lead V2.270 These VTs may be responsive to verapamil or adenosine, which suggests a triggered mechanism.271 Some LV VTs originate in the LVOT with an LBBB pattern.

Acute Treatment of Ventricular Tachycardia

VT should be treated emergently unless the rate is slow and the patient is clinically stable. If an extracardiac cause such as an electrolyte abnormality or acidosis has been identified, the underlying abnormality should be corrected. The correction usually results in the conversion of the ventricular arrhythmia to sinus rhythm. In patients with cardiac compromise, IV lidocaine at 1 mg/kg should be given immediately. If the lidocaine is effective, a continuous infusion of lidocaine at 10 to 50 µg/kg should be started to maintain an adequate level of lidocaine. Lidocaine levels should be monitored carefully to prevent toxicity. Although lidocaine has proven to be a safe and effective medication in the treatment of VT, current advanced cardiac life support (ACLS) protocols also recommend the use of IV amiodarone in this population.

Synchronized cardioversion at 2 to 5 watt-sec/kg should be performed if the lidocaine does not result in immediate conversion or if an IV site is not available. The energy requirements in biphasic cardioversion and defibrillation are less than those in monophasic cardioversion and defibrillation. Current ACLS recommendations call for no reduction in the energy delivered during cardioversion or defibrillation.

Procainamide IV has been used to treat acute episodes of VT. Because of the associated negative inotropic effects, patients must be monitored very carefully during the infusion. More recently, amiodarone has been shown to be effective for VT when given intravenously. Other drugs used in acute therapy are listed in Table 75-4. Rapid ventricular pacing may be used to convert the rhythm to sinus if pharmacologic therapy fails or is contraindicated.

Table 75-4 Acute Treatment of Ventricular Tachycardia

INITIAL TREATMENT DOSAGE LEVEL
Lidocaine 1-2 mg/kg IV bolus q5-15 min 1.5-5.0 mg/L
  IV infusion: 20-50 µg/kg/min  
Cardioversion 1-5 watt-sec/kg; double if ineffective  
SECONDARY TREATMENT IV DOSAGE LEVEL
Amiodarone 5 mg/kg over 1 hour, followed by 2.5 mg/kg IV bolus q4-6 h  
Magnesium 0.25 mEq/kg over 1 min, followed by 1 mEq/kg over 5 hours to achieve Mg++ level of 3-4 mg/dL  
Phenytoin 3-5 mg/kg over 5 min, not to exceed 1 mg/kg/min 10-20 µg/mL
Procainamide 5 mg/kg over 5-10 min or 15 mg/kg over 30-45 min NAPA: 4-10 mg/L
  Infusion: 20-100 µg/kg/min PA: 4-8 mg/L
Propranolol 0.05-0.1 mg/kg over 5 min q6h  

NAPA, N-acetylprocainamide; PA, procainamide.

Long-Term Treatment of Ventricular Tachycardia

Once the arrhythmia has been converted, choice of an appropriate long-term regimen is essential to maintaining the stability of the patient. The drugs most commonly used are listed in Table 75-1. If lidocaine has been successful, it may be continued until adequate levels of a chronic regimen have been reached or the acute causative agent is no longer present. When switching to mexiletine, the lidocaine should be gradually weaned as the mexiletine is initiated to prevent the combined toxicity of these two drugs, as their side effects are similar. Propranolol or other β-blockers are especially effective in patients whose arrhythmia is sensitive to adrenergic stimuli.274 Class I agents and amiodarone are effective in more refractory cases.274,275 A combination of amiodarone and propranolol has been effective in infants and children.276

As sudden death occurs in up to 30% of patients with VT and congenital heart defects, these patients should be placed on a long-term regimen. This type of arrhythmia is poorly tolerated by patients in the immediate postoperative period, but the arrhythmia generally responds to lidocaine or IV amiodarone and to correction of other underlying hemodynamic and metabolic abnormalities. Studies have shown that patients with early postoperative VT are likely to develop this arrhythmia in the late postoperative period, so long-term therapy is often recommended. The patient who presents months to years after surgery also requires long-term therapy. A thorough investigation is needed to rule out underlying hemodynamic abnormalities, as VT is tolerated less well by this group of patients. Residual or new hemodynamic lesions may be contributing to the arrhythmia. Mexiletine has been shown to be an effective long-term drug in patients after tetralogy of Fallot repair, as is the β-blocker class of drugs.261 Class I agents as well as amiodarone may be effective in refractory cases. The EPS may be a helpful guide to medical therapy.216,217 In cases refractory to drug therapy, the EPS can determine the site of origin of the tachycardia and direct treatment by surgical or catheter ablation. Patients with life-threatening episodes or those not responding to medical or ablative therapy may require ICDs. See Chapter 80 for more details.

Electrophysiological Study of Ventricular Tachycardia

Specific indications for the performance of EPS in children with VT are included in Box 75-2. Intracardiac recordings during VT show the absence of His-bundle deflections consistently preceding ventricular depolarizations. Atrial capture at rates more rapid than the tachycardia normalizes the QRS complex. One of the most significant differences between adults and children is the mechanism responsible for VT.78 In adults, more than 90% have inducible VT. In children, only 30% to 60% have inducible or re-entrant VT, and the rest have triggered or automatic VT. Inducible re-entrant VT in children is more commonly associated with structural heart disease.

Sinus Node Dysfunction

Sinus node dysfunction is uncommon in the pediatric patient. Patients can present with significant bradycardia with long pauses secondary to sinus arrest, with chronotropic incompetence leading to exercise intolerance. Infrequent presentations include intermittent periods of tachycardia and bradycardia (sick sinus syndrome). Etiologies of sinus node dysfunction include congenital defects of the sinus node, traumatic damage to the sinus node or its blood supply, inflammatory processes, or idiopathic causes. Relatively hemodynamically insignificant congenital heart abnormalities can lead to sinus node dysfunction, such as the absence of the right superior vena cava with persistent left superior vena cava or other abnormalities in the venous drainage to the heart (heterotaxy syndromes). In some instances, the patient may present with signs and symptoms consistent with sinus node dysfunction wherein the sinus node is normal but the autonomic input to the sinus node is abnormal.

Atrioventricular Block

Abnormalities of AV conduction are uncommon in children, but they do occur. Etiologies include congenital abnormalities, inflammatory processes, and trauma. Occasionally, no distinct etiology can be identified, and the block may be related to increased vagal tone.

First-Degree and Second-Degree Atrioventricular Block

First-degree and second-degree heart block may be congenital or acquired. Acquired etiologies include traumatic damage associated with surgery for congenital heart defects and inflammatory processes such as rheumatic heart disease or Lyme carditis. The EPS in pediatric patients has generally localized the delay to the AV node.269 Delay in the His-Purkinje system with prolongation of the H-V interval may be more significant, indicating a predisposition to the development of complete heart block.279,280 Patients with first-degree and second-degree AV block are generally asymptomatic unless the ventricular rate is significantly decreased. It is not uncommon to see first-degree and Mobitz I AV block during 24-hour Holter monitoring in teenagers, especially during sleep. The low heart rates are especially significant in patients with compromised myocardial function, and thus the cardiac output may be insufficient to meet the patient’s needs.

Complete Heart Block

Complete heart block is the most common cause of significant bradycardia in children. The ventricular rate is usually 40 to 80 beats/min, depending on the patient’s age. The QRS morphology and heart rate are related to the location of the escape pacemaker. The higher the origin of the pacemaker within the AV conduction system, the faster is the ventricular rate and the narrower the QRS complex (Figure 75-13). Wider QRS escape complexes usually originate from the His bundle or below. The usual rate in the neonate is 60 to 80 beats/min.

Complete heart block may be either congenital or acquired and occurs in 1 per 20,000 live births. The same causes of acquired heart block that cause second-degree AV block discussed earlier can cause complete heart block. In a series of 599 patients with congenital complete heart block, followed up for more than 10 years, 92% survival was seen in patients without associated structural heart disease.281 The greatest risk of mortality was during the first weeks of life, especially in those delivered prematurely secondary to fetal hydrops, with half of the deaths occurring during the first year. The highest risk was indicated by associated cardiac anomalies, cardiomegaly, ventricular bradycardia less than 55 beats/min, and atrial tachycardia greater than 140 beats/min in infants.

A strong association has been noted with connective tissue disease in the mother and congenital complete heart block. The prevalence has been reported to be as high as 85% in mothers of affected infants.282,283 Only one half of these affected mothers are symptomatic, whereas the other half, although asymptomatic, are serologically positive. A mother with systemic lupus erythematosus has a 1 : 20 risk of having a child with complete heart block if she is anti-Ro positive.284 Maternal immunoglobulin G antibodies to soluble tissue ribonucleoprotein antigens, found in the cytoplasm or nucleus of human cells (anti-Ro:SS-A and anti-La:SS-B), cross the placenta after 12 to 16 weeks of gestation. This results in an inflammatory response in the fetal heart, particularly in the conduction system, leading to the destruction of the AV node.285287

Buyon demonstrated an increased risk when the maternal antibodies target specific portions of the ribonuclear complex, particularly the 48-kd La (SS-B), the 52-kd Ro (SS-A), and the 60-kd Ro (SS-A). The highest risk was reactivity to both the 48-kd La (SS-B) and the 52-kd Ro (SS-A) components.288 Because of this immunologic factor, dexamethasone and plasmapheresis have been recommended as effective treatments for the mother of an affected fetus.288,289 Although the heart block has not been shown to resolve with these treatments, the clinical course of the fetus has improved, with decreased pleuro-pericardial effusions and other signs of fetal hydrops.

Presentation

Some cases will be diagnosed in utero because of low fetal heart rates, which usually develop after the seventeenth week of gestation. Fetal echocardiography may show fetal hydrops in 15% to 61%, with a higher incidence being seen in association with structural heart disease, especially AV valve regurgitation. The survival with hydrops is less than 10% unless the fetus can be delivered immediately. Even with early delivery, these infants are often very ill and difficult to manage and require an aggressive team approach by neonatologists, cardiologists, and cardiothoracic surgeons. With this aggressive management, the outlook is relatively good if the fetal heart rate is more than 55 beats/min and if no structural heart disease is present.

Structural heart disease is present in one third to one fourth of infants with congenital heart block. The associated congenital heart defects most commonly include those with L-transposition of the great arteries (L-TGA) (physiologically corrected transposition) or the heterotaxy syndromes. With associated heart disease, mortality in the first year has been reported to be 29% in one study.281 Complex postoperative congenital heart lesions or those with an unusual course of the AV conduction system may develop postoperative block.291 After ventricular septal defect repair, the presence of RBBB and left anterior hemiblock is 7% to 17%, with a 1% incidence of complete heart block.292 Complete heart block has been reported to occur as late as 14 years after surgical repair.293 Complete heart block occurs more commonly after repair of AV canal defects, probably because of the unusual course of the conduction system in these lesions, and may be seen in up to 7% of patients.294 Physiologically corrected L-TGA is associated with AV conduction disturbances ranging from first-degree to complete heart block in 30% to 60% of patients.

These conduction disturbances may be present at birth, develop insidiously, or occur during or after the surgical correction of associated defects. The AV bundle and conduction system have been found to cross the pulmonary outflow tract and descend along the anterior rim of the ventricular septal defect or along the right margin of the foramen ovale between the main and outflow chambers in a single ventricle with an outflow chamber. Careful attention to these facts and intraoperative mapping have decreased the incidence of this form of postoperative block.

When low fetal heart rates are noted, fetal echocardiography should be performed. Signs of cardiac decompensation include evidence of fetal hydrops with pleural or pericardial effusions or ascites. Cardiac function may be decreased, and tricuspid regurgitation is often present.

In a study of patients with congenital heart block who were followed up for more than 30 years, only 10% did not require a pacemaker, and a 20% incidence of sudden death was noted. Mitral regurgitation and prolonged QTc were poor prognostic findings. Pacemakers were recommended even in asymptomatic adults.295

Evaluation and Treatment

Twenty-four-hour Holter monitoring may be used to determine the average heart rate, heart rate ranges, QRS duration, Q-T intervals, and the presence of ventricular arrhythmias or long pauses.

Although many infants with congenital complete heart block are asymptomatic at birth, others show findings typical of congestive heart failure and require treatment. A subset may develop severe congestive heart failure and cardiovascular collapse. These distressed infants may require intubation and ventilation, treatment of acidosis, and catecholamine support of heart rate and blood pressure.

In emergencies, immediate transthoracic pacing can be accomplished. The transcutaneous pacemaker may be effective in short-term emergency situations but should be replaced with another pacing method as soon as possible. Placement of a temporary transvenous pacemaker, either through the umbilical vein or the femoral vein under direct fluoroscopic observation, is preferred. Although infants with rates less than 50 beats/min or slightly higher rates and associated congenital heart defects or cardiomyopathies may require pacing, this decision should not be made on the basis of heart rate alone. Older children with congenital heart block may develop evidence of exercise intolerance, easy fatigability, or syncope. Not all patients with congenital complete heart block need a pacemaker, and many do not require one until they reach an older age.295

Pacemakers may be placed because of associated ventricular arrhythmias, either during sleep or exercise, easy fatigability or exercise intolerance, syncope, or presyncope.296298 Other indicators for pacing that have been associated with sudden death include severe bradycardia, ventricular ectopy especially with exercise, prolonged pauses, increased QRS width, prolongation of QTc, and a junctional recovery time of more than 3 seconds.299,300 Another important indicator for pacer placement is cardiac enlargement shown by chest x-ray or echocardiogram. With cardiac output being the product of heart rate and stroke volume, the LV will dilate, indicating that the heart rate is not adequate to meet the metabolic demand.

The acquired heart block associated with inflammatory disease such as viral myocarditis, Lyme disease, or rheumatic fever may be transient and require only temporary pacing.

With postoperative heart block, temporary pacing is usually performed 7 to 10 days after surgery. Permanent pacing should be performed if sinus rhythm does not return because of the high incidence of sudden death in this group of postoperative patients if they are not paced.301 As the return of sinus rhythm does not ensure that heart block will not recur at a later time, close follow-up is indicated.

Abnormalities in intraventricular conduction may be congenital or acquired. Congenital RBBB may be inherited or found in association with Ebstein’s anomaly of the tricuspid valve.302,303 Children with Kearns-Sayre syndrome are likely to develop RBBB that progresses to complete heart block.304 Prophylactic placement of a pacemaker is recommended in this group of patients to prevent sudden death. Postoperative RBBB is common after repair of many cardiac anomalies and is either central or peripheral.305,306

Implantable Pacemakers

The use of permanent pacemakers in children was first reported in the early 1960s and predated many recent advances in lead and generator technology.307310 These advances, including programmability and miniaturization, have significantly increased pacemaker use in the pediatric population.311314 See Chapter 79 for more detailed information on the use of pacemakers in children.

Key References

Benson DWJr, Smith WM, Dunnigan A, et al. Mechanisms of regular, wide QRS tachycardia in infants and children. Am J Cardiol. 1982;49(7):1778-1788.

Collins KK, Van Hare GF, Kertesz NJ, et al. Pediatric nonpost-operative junctional ectopic tachycardia medical management and interventional therapies. J Am Coll Cardiol. 2009;53(8):690-697.

Daubert JP, Zareba W, Rosero SZ, et al. Role of implantable cardioverter defibrillator therapy in patients with long QT syndrome. Am Heart J. 2007;153(4 Suppl):53-58.

Davis AM, Gow RM, McCrindle BW, Hamilton RM. Clinical spectrum, therapeutic management, and follow-up of ventricular tachycardia in infants and young children. Am Heart J. 1996;131(1):186-191.

Elliott P, McKenna WJ. Hypertrophic cardiomyopathy. Lancet. 2004;363(9424):1881-1891.

Garson AJr, Dick M, Fournier A, et al. The long QT syndrome in children. An international study of 287 patients. Circulation. 1993;87(6):1866-1872.

Goldenberg I, Moss AJ. Long QT syndrome. J Am Coll Cardiol. 2008;51(24):2291-2300.

Hanisch D. Pediatric arrhythmias. J Pediatr Nurs. 2001;16(5):351-362.

Ko JK, Deal BJ, Strasburger JF, Benson DWJr. Supraventricular tachycardia mechanisms and their age distribution in pediatric patients. Am J Cardiol. 1992;69(12):1028-1032.

Maron BJ, Ackerman MJ, Nishimura RA, et al. Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol. 2005;45(8):1340-1345.

Maron BJ, McKenna WJ, Danielson GK, et al. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. Eur Heart J. 2003;24(21):1965-1991.

Michaelsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation. 1995;92(3):442-449.

Pfammatter JP, Paul T. Idiopathic ventricular tachycardia in infancy and childhood: A multicenter study on clinical profile and outcome. Working Group on Dysrhythmias and Electrophysiology of the Association for European Pediatric Cardiology. J Am Coll Cardiol. 1999;33(7):2067-2072.

Roden DM. Clinical practice. Long-QT syndrome. N Engl J Med. 2008;358(2):169-176.

Santinelli V, Radinovic A, Manguso F, et al. The natural history of asymptomatic ventricular pre-excitation: A long-term prospective follow-up study of 184 asymptomatic children. J Am Coll Cardiol. 2009;53(3):275-280.

Schwartz PJ, Spazzolini C, Crotti L, et al. The Jervell and Lange-Nielsen syndrome: Natural history, molecular basis, and clinical outcome. Circulation. 2006;113(6):783-790.

Vetter VL. Ventricular arrhythmias in pediatric patients with and without congenital heart disease. In: Horowitz LN, editor. Current management of arrhythmias. Philadelphia: BC Decker, 1990.

Wilde AA, Bezzina CR. Genetics of cardiac arrhythmias. Heart. 2005;91(10):1352-1358.

Zareba W. Genotype-specific ECG patterns in long QT syndrome. J Electrocardiol. 2006;39(4 Suppl):S101-S106.

Zipes DP, Ackerman MJ, Estes NAIII, et al. Task Force 7: Arrhythmias. J Am Coll Cardiol. 2005;45(8):1354-1363.

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