Co-burn J. Porter

Atrial flutter in the young: a collaborative study of 380 cases

As children with cardiac disease grow older, atrial flutter becomes more prevalent. A collaborative study was performed in 19 institutions to determine the clinical characteristics of these children and the factors affecting prognosis. There were 380 patients with one or more electrocardiographically documented episodes of atrial flutter that first occurred between ages 1 and 25 years (mean age at onset 10.3). Episodes of flutter continued to occur for a mean of 2.5 years after the onset. Of the 380 patients, 60% had repaired congenital heart disease, 13% palliated congenital heart disease, 8% unoperated congenital heart disease, 8% an otherwise normal heart, 6% cardiomyopathy, 4% rheumatic heart disease and 2% other lesions. Overall, drugs were effective in eliminating atrial flutter in 58% of patients; specifically, amiodarone and digoxin plus quinidine were effective in 53%, digoxin alone in 44% and propranolol in 21%. Amiodarone was effective in seven (78%) of nine patients. Corrective surgery was performed after the onset of atrial flutter in 66 patients; in 52% the atrial flutter was easier to control or it resolved and in only 4% it was worse. At follow-up (mean 6.5 years), 83% of the patients were alive (49% without atrial flutter and 34% with atrial flutter) and 17% died (10% suddenly, 6% of nonsudden cardiac cause and 1% of noncardiac cause). Cardiac death occurred in 20% of those for whom an effective drug could not be found to eliminate atrial flutter compared with 5% of those who were treated with an effective drug (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Amiodarone treatment of critical arrhythmias in children and young adults

The majority of sudden cardiac deaths in children occur in patients with prior arrhythmias and an abnormal heart. Amiodarone was given to 39 young patients (35 with an abnormal heart) with arrhythmias unresponsive to conventional treatment. Their age ranged from 6 weeks to 30 years with nine patients younger than 2 years of age. Atrial flutter was present in 16 patients, ventricular tachycardia in 14 patients and supraventricular tachycardia in 9 patients. The most common diagnosis (14 patients) was postoperative repair of congenital heart disease. The dose ranged from 2.5 to 21.6 mg/kg per day (mean 8.2). Elimination of arrhythmia (on 24 hour electrocardiography) occurred in 15 of 16 patients with atrial flutter, 11 of 14 with ventricular tachycardia and 5 of 9 with supraventricular tachycardia. Symptomatic side effects were: rash (three patients), headache (two patients), nausea (one patient) and peripheral neuropathy (one patient); seven patients had asymptomatic corneal microdeposits which normalized in all after the drug was discontinued. No side effects occurred in patients younger than 10 years of age. The following changed with treatment (p less than 0.05): heart rate decreased (three patients with atrial flutter and sick sinus syndrome required pacemaker implantation for bradycardia) and QTc increased; thyroxine (T4) and serum reverse triiodothyronine (T3) increased. During follow-up study (range 6 months to 3 years), 21 of the 39 patients continued to take amiodarone with complete control of arrhythmias, 9 were no longer taking the drug and 9 died (7 nonsudden and 2 sudden deaths). Amiodarone is an extremely effective treatment for infants and children with tachyarrhythmias resistant to conventional treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital complete atrioventricular block: Clinical and electrophysiologic predictors of need for pacemaker insertion

Because of initial Adams-Stokes attack in the patient with congenital complete atrioventricular (A-V) block may sometimes prove fatal, there is a need to be able to identify the patient at great risk of having such attacks. Twenty-four children with congenital complete A-V block were followed up for 1 to 19 years to determine the efficacy of current methods of predicting risk for Adams-Stokes syncope and the usefulness of pacemaker therapy in relieving symptoms. The heart rate at rest, configuration of surface electrocardiographic complexes, data obtained during intracardiac electrophysiologic study and response to graded treadmill exercise testing were compared in children with and without syncope. One or more Adams-Stokes episodes were experienced by eight children, one of whom died. Only a persistent heart rate at rest of 50 beats/min or less demonstrated any significant (probability [p] less than 0.01) correlation with the incidence of syncope. Intracardiac electrophysiologic study was of little benefit because of site of block did not correlate with syncope. Although the increase in heart rate during treadmill exercise testing showed no correlation with prevalence of syncope or location of block, exercise-induced ventricular ectopic beats may have predictive value in older children and young adults. Ventricular pacemakers were implanted in 10 children. Each child was asymptomatic over a 1 to 10 year follow-up period. Because extreme bradycardia may contribute to the prevalence of Adams- Stokes attacks in children with congenital complete A-V block, careful evaluation of heart rate at rest may be an effective means of differentiating patients at risk of syncope. Pacemaker therapy is a feasible and effective method of treatment in young children and relieves symptoms

Induction of ventricular tachycardia during electrophysiologic study after repair of tetralogy of Fallot

An association among premature ventricular complexes on routine electrocardiogram, elevated right ventricular systolic pressure and sudden death after repair of tetralogy of Fallot was previously reported. To examine this relation further, noninvasive, hemodynamic and invasive electrophysiologic data were studied in 27 patients who had undergone repair of tetralogy of Fallot 7 months to 21 years (mean 1.75 years) previously. Syncope, which had occurred in four patients, was not significantly related to ventricular arrhythmia on rest electrocardiogram, 24 hour electrocardiogram or treadmill test. All four patients with syncope had either nonsustained (two patients) or sustained (two patients) ventricular tachycardia induced at electrophysiologic study. His bundle to ventricle conduction interval was prolonged in two patients and Q to right ventricular apex interval was prolonged in three of the four patients. All four had abnormal anatomic or hemodynamic findings: two had a right ventricular systolic pressure of 70 mm Hg or more, one had right ventricular dysfunction with tricuspid insufficiency and one a septal aneurysm. The 9 patients with induced nonsustained or sustained ventricular tachycardia were then compared with the 15 patients without induced ventricular arrhythmias. Those with ventricular tachycardia had a greater prevalence of: more complex ventricular arrhythmia on 24 hour electrocardiogram (63 versus 0%, p less than 0.001), long His bundle to ventricle interval (44 versus 0%, p less than 0.001), right ventricular systolic pressure of 70 mm Hg or more (56 versus 0%, p less than 0.01) and reduced right ventricular ejection fraction (33 versus 7%, p less than 0.025). It is concluded that: 1) induction of nonsustained or sustained ventricular tachycardia was associated with a history of syncope; 2) all patients at risk for syncope could not be identified by routine electrocardiogram 24 hour electrocardiogram or treadmill test; 3) hemodynamic alterations may interact with intraventricular conduction abnormalities and predispose to ventricular tachycardia.

Transvenous pacing in pediatric patients

Cardiac pacing has been traditionally carried out by the epicardial approach in pediatric patients.1-3 This has been true for several reasons: (1) pacemakers were too large to implant safely in the pectoral area; (2) leads were too large to easily insert into the cephalic vein; (3) leads were considered likely to interfere with the tricuspid valve; (4) lead dislodgement rate was considered unacceptably high. Adults are usually paced by a transvenous approach.4 Because many of these problems appeared to have been solved, we decided to evaluate the feasibility of transvenous pacing in our pediatric patients. Transvenous pacing has been reported in only a small group of children .5 In this report we present the early results of the first year of transvenous pacing in our clinic.

Exercise testing in children with Wolff-Parkinson-white syndrome

Exercise testing using a modified Bruce treadmill protocol was performed by 17 children with Wolff-Parkinson-White (WPW) syndrome. All had intracardiac electrophysiology studies as well. Endurance time, heart rate and blood pressure were normal during exercise. Ventricular premature complexes were seen with exercise in 2 patients and supraventricular tachycardia with exercise testing was seen in 2. Disappearance of the delta wave with exercise correlated with a long anterograde effective refractory period of the Kent bundle (360 to 390 ms). Children with partial normalization of the QRS during exercise had a longer anterograde effective refractory period of the Kent bundle than those in whom preexcitation persisted. In 1 patient, disappearance of the delta wave with exercise confirmed the diagnosis of WPW syndrome. Preexcitation was seen only after exercise in 1 patient. Exercise testing is of value in the evaluation of children with WPW syndrome; children with WPW syndrome who have total normalization of the QRS interval during exercise and few or no symptoms of tachycardia do not require electrophysiologic study.

Effects of verapamil on supraventricular tachycardia in children

Abstract Thirteen patients, aged 6 weeks to 16 years, with uncontrolled recurrent Supraventricular tachycardia were given intravenous verapamil in an attempt to abolish an episode of Supraventricular tachycardia. All patients had had intracardiac electrophysiologic studies to define the mechanism of their tachycardia. In seven patients conversion to sinus rhythm occurred after administration of verapamil: Five of the seven had atrioventricular (A-V) nodal reentry as the mechanism of their supraventricular tachycardia; the other two had reentrant tachycardia involving an accessory pathway. Verapamil was effective in abolishing the Supraventricular tachycardia in these patients, probably by prolonging the A-V nodal refractory period and conduction, thus breaking the reentrant circuit. In six patients there was no conversion to sinus rhythm: Four of the six had automatic atrial ectopic tachycardia and two had automatic junctional ectopic tachycardia. Among the four patients with automatic atrial ectopic tachycardia, a junctional escape rhythm developed in one, and second degree A-V block developed in the others. The two patients with junctional ectopic tachycardia had severe symptomatic arterial hypotension after verapamil and required resuscitation with intravenous calcium chloride. In spite of the good response to intravenous verapamil in the seven patients with reentrant tachycardia, only four of the seven could be maintained successfully on long-term oral therapy. The patients who experienced conversion to sinus rhythm with an intravenous bolus dose of verapamil but in whom Supraventricular tachycardia could still be induced with programmed stimulation could not be maintained successfully on oral therapy. It is concluded that verapamil is an effective antiarrhythmic agent that can (1) abolish the acute episode of Supraventricular tachycardia only in cases due to reentrant mechanisms, and (2) be used as an oral medication to prevent recurrences of supraventricular tachycardia in patients in whom the arrhythmia cannot be induced with programmed stimulation after intravenous doses of the drug.

Symptomatic sick sinus syndrome in children and adolescents as the only manifestation of cardiac abnormality or associated with unoperated congenital heart disease

Sick sinus syndrome (SSS) occurs infrequently in children who have not undergone cardiac surgery. The symptoms, electrocardiograms, and electrophysiologic data in 11 patients aged 2 to 17 years who had nonsurgical SSS were reviewed. Syncope occurred in 5 patients and sinus bradycardia in 9. Sinus nodal recovery times were prolonged in 6 patients. The atrial effective refractory period was prolonged in 2 patients and the atrioventricular nodal functional or the effective refractory period, or both, was prolonged in 5 patients. Because patients with nonsurgical SSS may have abnormalities not only of the sinus node but also of the atrium and the atrioventricular node, it is recommended that patients with symptomatic SSS be evaluated by electrophysiologic study. The proper choice of antiarrhythmic drug therapy or permanent pacing procedure depends on a complete analysis of the cardiac conduction system.

Effect of ouabain on the anterograde effective refractory period of accessory atrioventricular connections in children

The anterograde effective refractory period of the accessory connection was determined before and after the administration of ouabain (0.015 mg/kg intravenously) during electrophysiologic studies in 21 patients with Wolff-Parkinson-White syndrome. The mean age (+/- standard deviation) was 10 +/- 2 years (range 1 month to 31 years). Each patient had stopped taking all cardiac drugs for more than 36 hours. Determination of the anterograde effective refractory period of the accessory connection was made using the atrial extrastimulus technique. A change in the anterograde refractory period of the accessory connection was defined as an increase or decrease of greater than 10 ms from the value before ouabain administration. The post-ouabain anterograde effective refractory period of the accessory connection increased in 2 (9%) of the 21 patients, decreased in 9 (43%) and was unchanged in 10 (48%). This study demonstrated a decrease in the anterograde effective refractory period of the accessory connection of 43% of patients with Wolff-Parkinson-White syndrome after the administration of ouabain.

Cryosurgical modification of atrioventricular conduction for treatment of atrioventricular node reentrant tachycardia.

Cryosurgical modification of atrioventricular (AV) node conduction was performed in five patients with AV node reentrant tachycardia that used dual AV nodal pathways and was refractory to drug therapy. The procedure alleviated the tachycardia in all patients without the development of complete heart block and without any associated surgical morbidity or mortality. These results suggest that cryosurgical modification of AV node conduction is a promising and potentially curative method of treating AV node reentrant tachycardia.