Supraventricular Arrhythmias in Children
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122B JACC Vol. 5, No.6 June 1985:122B-129B Supraventricular Arrhythmias in Children PAUL C. GILLETIE, MD, FACC Charleston , South Carolina Sudden death may occur in children with supraventric• in the electrophysiology laboratory. Sudden death due ular arrhythmias. Sick sinus syndrome, particularly if to complete AV block shoUld be preventable using pace• associated with tachycardia, may result in sudden death makers. Neonates with a ventricular rate less than SS in children who have had open heart surgery and rarely beats/min or children with It rll~e less than 4S beats/min in children with a normal heart. Children witt, supra• should receive pacemaker therapy because of tlie statis• ventricular tachycardia rarely die. Only those with junc• tical probability of death or syncope. Ventricular ectopic tional automatic tachycardia or Wolff-Parkinson-White beats, particularly if frequent or multiform, may be an syndrome have died. Pati~nts with Ii short anterograde indication for pacemaker insertion. Patients with sur• refractory period may be at risk f?f sudden death. Sur• gical complete AV block that persists for more than 7 gical division of the accessory ennneetion cart prevent to 10 days should receive physiologic pacemakers for the sudden death. Digitalis may accelerate atrioventricular prevention of sudden death and hemodynamic benefit. (AV) conduction in patients with WoltT-Parkinson-White (J Am Coli CtirdioI1985;5:122B-129B) syndrome and, thus , should be used only after testing In this review, I will discuss the role that supraventricular There is overlap between this topic and sudden death due arrhythmias play in sudden death in children. In doing this, to ventricular arrhythmias. Some cases of death in patients I am discussing the rare causes of a rare event. The ar• with primary bradyarrhythmias are due to ventricular fi• rhythmias I will include are: I) sick sinus syndrome, both brillation. In addition, death in patients with supraventric• naturally occurring and acquired after open heart surgery; ular tachycardia and Wolff-Parkinson-White syndrome is 2) the bradycardia-tachycardia syndrome; 3) supraventric• probably often due to ventricular fibrillation. ular tachycardia, emphasizing junctional automatic tachy• cardia arid the Wolff-Parkinson-White syndrome; and 4) complete atrioventricular (AV) block, The inclusion of com• Sick Sinus Syndrome plete AV block is somewhat arbitrary, but I decided to Death from sick Sinus syndrome was extremely rare in include it in this discussion so that the use of pacemakers the pediatric age group until complex intraatrial surgery could be kept in one place. began to be performed (1-4), Even now, death in "pure" For each of these entities, I will discuss the etiology, sick sinus syndrome is unusual, most instances occurring pathogenesis, electrophysiologic mechanisms, natural his• in patients who also had atrial tachyarrhythmias. tory, association with sudden death, evaluation and treat• Isolated sick sinus syndrome. Instances of sick sinus ment. The exact association of an arrhythmia with sudden syndrome in children who did not have congenital heart death is often assumed because, by definition, sudden death disease or cardiac surgery have been reported. At least one is usually not monitored. The definition of sudden death is death in this group has been noted (I ). We have reported an event that occurs suddenly resulting in unconsciousness eight instances of sick sinus syndrome in children with an from which the patient either never recovers or is resusci• otherwise normal heart and three in children without surgical tated. Thus, I will include among patients with sudden death treatment for congenital heart disease (5). those who lived after active resuscitation, an event that is The etiology of these cases remains unknown. In seven being seen with increasing frequency. of our nine symptomatic patients, abnormalities of sinus node automaticity were confirmed by an abnormal degree From the South Carolina Children 's Heart Center, Medical University of overdrive suppression. In three of five, sinoatrial coil• of South Carolina, Charleston, South Carolina . duction was also prolonged, The atrial muscle refractory Address for reprints: Paul C. Gillette , MD, South Carolina Children 's Heart Center , Medical University of South Carolina, 171 Ashley Avenue, period was prolonged in two of eight. Atrioventricular node Charleston, South Carolina 29425. and His-Purkinje function was usually normal, being pro- ©1985 by the American College of Cardiology 0735-1097/85/$3.30 JACCVol. 5. No.6 GILLEITE 123B June 1985:122B- 1298 SUPRA VENTRICULAR ARRHYTHMIAS IN CHILDREN longed in I of II and 1 of 10, respectively. Tachyarrhyth• septal defect repaired using hypothermia without cardio• mias were inducible in 4 of 11, although none of these pulmonary bypass , suggesting that cannulation of the su• patients had clinical tachyarrhythmias. perior vena cava might be a major cause of sinus node Each ofthese patients had episodes ofsyncope, but none damage. Some histologic studies have shown sutures in the was documented to be caused by additional bradycardia. sinus node and other investigations have found hemorrhage The infrequency of the episodes made their recording by or occlusion of the sinus node artery (6,8). ambulatory electrocardiographic monitoring unlikely. In most Atrial septal defect repair. Bricker (personal commu• of these patients, ambulatory monitoring demonstrated more nication), in a review of 269 cases of atrial septal defect severe bradycardia during sleep than during the awake state. repair, found that 7.5% of patients had symptomatic sinus Ventricular ectopic activity was rare in this group. Exercise node dysfunction on postoperative electrocardiography. Two testing showed a blunted increase in sinus rate in most of had received a pacemaker, but none had died postopera• these patients, although two had a normal response. tively. The prevalence of atrial tachycardias in this series Role of pacemaker implantation. Each of these pa• was low. tients was symptomatic and 8 of II , therefore, received a Mustard operation for transposition. EI-Said et al. (9), permanent pacemaker. In patients with syncope, if the heart in a review of postoperative patients who had undergone rate decreases to less than 50 beats/min in an infant, 40 the Mustard procedure, found that 2% were victims of sud• beats/min in a toddler or 30 beats/min in a teenager, it may den death and 3% required an implanted pacemaker. Mod• be assumed that bradycardia is the cause of symptoms. All ifications of the surgical procedure decreased the early in• of our patients in this group became asymptomatic after cidence of arrhythmias, but after 5 years the incidence rate pacemaker implantation , with the exception of one patient was the same. The rate of incidence remained the same in who also had an atrial-endocardial cushion septal defect and recent years when patients were operated in the first year cardiomyopathy. After a ventricular demand pacemaker was of life. implanted, her major symptom, syncope with grand mal Electrophysiologic studies show that slightly more than seizures, was abolished. She continued to have dizzy spells. 50% of patients who undergo the Mustard procedure have Ambulatory electrocardiographic monitoring showed that sinus node dysfunction as indicated by decreased automa• these occurred when she had sinus bradycardia and her ticity (Fig. I) (10-13). A few have an abnormally prolonged pacemaker was turned on. Because retrograde conduction sinoatrial conduction time and atrial conduction to the low was present, "pacemaker syndrome" was suspected. An lateral right atrium (13) . Conduction from the sinus node AV sequential pacemaker was implanted, and her symptoms to the AV node and through the AV node and His-Purkinje were relieved. system to the ventricles is universally normal. Induction Thus. cardiac pacing can relieve symptoms of syncope protocols frequently produce supraventricular tachycardias in patients with pure sinus node dysfunction. Whether any but have thus far failed to produce ventricular tachycardia, of these patients would have died is unclear. Very few although fewer patients have been studied with ventricular reports of sudden death in children with a normal heart and protocols. sick sinus syndrome are available. It seems wise to use a Natural history. The natural history of postoperative si• pacemaker in these children to relieve symptoms and pos• nus node dysfunction seems to be progressively worse. Some sibly prevent sudden death. Atrial or universal AV sequen• sudden deaths occur in the first postoperative year, but new tial pacemakers should be used to prevent pacemaker syn• sudden deaths continue to occur up to 15 years postopera• drome and provide the most physiologic response. Our policy tively. The direct association of sudden death with sinus is to use an atrial pacemaker if AV node function is normal bradycardia remains unproven . Deaths have occurred in pa• or near normal. This is best determined by the rate of atrial tients with a normally functioning pacemaker. It is likely pacing at which type I second degree AV block occurs . If that many deaths are due to atrial flutter with I : I ventricular it occurs at a rate of 110 beats/min or greater, we believe response. A few may be due to ventricular arrhythmias. that atrial pacing is indicated. This , of course, presumes Indications for digitalis. We have observed several in• that the patient has not already had second