Indications for modification of coexisting dual atrioventricular node pathways in patients undergoing surgical ablation of accessory atrioventricular connections.

Concomitant susceptibility to atrioventricular (AV) node reentrant tachycardia has been demonstrated in certain patients having reentrant tachycardia utilizing accessory AV connections. For those patients undergoing accessory connection ablation, AV node surgical modification may be warranted during the same operative procedure. To assess indications for a combined operative procedure, this study evaluated potential predictors of subsequent spontaneous AV node reentrant tachycardia in patients undergoing ablation of accessory AV connections. Among 62 consecutive patients undergoing surgical ablation of an accessory AV connection, 13 (21%) manifested dual AV node pathways. The latter were identified preoperatively in five patients (four with concealed and one with bidirectional accessory connections) and postoperatively in seven (all seven with bidirectional accessory connections). In one patient with a bidirectional accessory connection, dual AV node pathways could not be demonstrated preoperatively, but AV node reentrant tachycardia was induced. Operative ablation of an accessory connection was successful in all patients. However, postoperatively, 2 of the 13 patients had inducible AV node reentrant tachycardia, 5 had AV node "echo" beats and 6 had no inducible arrhythmia. During 26 +/- 7 months of follow-up study, the two patients with inducible AV node reentrant tachycardia postoperatively had symptomatic AV node reentrant tachycardia. In addition, the one patient with inducible AV node reentrant tachycardia preoperatively had recurrence of this tachycardia 4 months after attempted surgical modification of the AV node. Consequently, although dual AV node pathways appear to be common in patients undergoing surgical ablation of an accessory AV connection (21%), only a small group (3 of 13) of these patients are at risk for subsequent clinical AV node reentrant tachycardia.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac and skeletal muscle abnormalities in cardiomyopathy: comparison of patients with ventricular tachycardia or congestive heart failure.

Results of cardiac muscle and skeletal muscle biopsies were compared in 22 patients with cardiomyopathy; 11 patients presented with symptoms secondary to ventricular tachycardia (Group 1) and 11 had symptoms of severe congestive heart failure (Group 2). No patient had structural or ischemic cardiac disease. In Group 1 patients, hemodynamic abnormalities were subtle, but invasive study demonstrated dilated cardiomyopathy in two patients and restrictive cardiomyopathy in nine. In Group 2, eight patients had dilated cardiomyopathy and three had restrictive cardiomyopathy. Cardiac biopsy results were abnormal in all 22 patients and the abnormalities were similar for the two groups. Cardiac histologic study revealed a spectrum of abnormalities including fibrosis, dilated sarcoplasmic reticulum, increased numbers of intercalated discs and mitochondrial abnormalities. Histologic abnormalities of skeletal muscle were similar in each group, consisting of endomysial fibrosis and increased lipid deposits. Slightly more than half of the Group 1 and Group 2 patients also had a low concentration of skeletal muscle long chain acylcarnitine. These data demonstrate that abnormalities of both cardiac and skeletal muscle are common in patients with cardiomyopathy; abnormalities are similar whether initial symptoms are due to ventricular tachycardia or congestive heart failure. It is suggested that these patients with cardiomyopathy may have a generalized myopathy.

Inducible ventricular arrhythmias in a naturally occurring model of cardiomyopathy.

This study examined inducibility of ventricular tachyarrhythmias in turkeys with and without naturally occurring dilated cardiomyopathy. Using a transvenously positioned electrode catheter, 32 cardiomyopathy and 12 control unsedated turkeys aged 2 to 4 months were studied by right ventricular endocardial extrastimulus testing at basic pacing cycle lengths of 200 and 170 ms with both 1 and 2 extrastimuli and burst pacing at progressively shorter cycle lengths (200 to 100 ms). Following study, a dilatation index (determined as the ratio of left ventricular endocardial and epicardial diameter at level of the apex-base midpoint) was utilized to assess the functional severity of cardiomyopathy. All control turkeys had a dilatation index less than 0.3. In cardiomyopathic turkeys, dilatation index was normal (less than 0.3) in 3/32, showed mild to moderate dilatation in 25/32 (0.3 to 0.6), and severe dilatation in 4/32 (greater than 0.6). Results showed no difference in right ventricular effective or functional refractory periods between control and cardiomyopathic turkeys. Control turkeys had no inducible ventricular tachyarrhythmias, but 16/32 cardiomyopathic turkeys (p less than 0.005) had inducible ventricular tachyarrhythmias, consisting most frequently of two beats of rapid ventricular tachycardia supervened by ventricular fibrillation. In the cardiomyopathic turkeys, inducible tachyarrhythmias occurred in 1/3 with normal dilatation index, in 11/25 with mild to moderate dilatation, and in 4/4 with severe dilatation. Thus, inducibility of ventricular tachyarrhythmias in cardiomyopathic turkeys is closely associated with increasing ventricular dilatation, but does not correlate with altered right ventricular refractoriness. This model may be suitable for studying the relationship between ventricular tachyarrhythmias and cardiomyopathy.

Atrial flutter in infancy: diagnosis, clinical features, and treatment.

The clinical features and treatment of atrial flutter in eight infants (four male and four female) less than 2 months of age are presented. Atrial flutter was noted during the first week of life in six of the infants and between 6 and 8 weeks of life in the other two infants. Four of the eight infants had associated structural or functional cardiovascular disease, and in three infants a central venous pressure catheter was present in the atrium at the time atrial flutter was diagnosed. Classic flutter waves were apparent on 12-lead ECGs in only two infants. In six infants, flutter waves were not obvious on standard ECGs, but transesophageal electrogram recordings demonstrated the presence of atrial flutter with second degree atrioventricular block. The atrial cycle length during flutter ranged from 135 to 180 ms (mean 149 ms; mean atrial rate 403 beats per minute); there was a 2:1 ventricular response to atrial flutter. Successful termination of atrial flutter was accomplished using three modes of electrical cardioversion in seven of the eight infants: direct current cardioversion in one, transvenous atrial pacing in one, and transesophageal atrial pacing in five. One asymptomatic infant converted to normal sinus rhythm 24 hours following digoxin administration. One infant had multiple atrial flutter recurrences and required chronic procainamide therapy. In seven of the eight infants, no recurrences have been noted in 6 months to 3 1/2 years of follow-up. These results demonstrate that atrial flutter may be difficult to diagnose in infants with tachycardia unless transesophageal electrogram recording is utilized for evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiovascular collapse in infants: association with paroxysmal atrial tachycardia.

Four infants, aged 16 to 28 days (mean 23 days), were seen in the emergency room with acute cardiovascular collapse and with normal heart rate and rhythm. During evaluation for cardiovascular collapse, no infant had sepsis; cardiac assessment revealed normal intracardiac anatomy but global cardiac chamber enlargement and poor left ventricular systolic function, which resolved with supportive treatment. However, three of the four infants demonstrated ventricular preexcitation on their surface electrocardiogram and, subsequently, two infants had transient episodes of tachycardia. During a transesophageal pacing study to evaluate inducibility and electrophysiologic characteristics of tachycardia, sustained tachycardia was initiated in all four infants. Reentrant tachycardia used an accessory atrioventricular connection as evidenced by the presence of preexcitation during sinus rhythm (three infants), the ability to initiate and terminate tachycardia by programmed electrical stimulation (four infants), minimum ventriculoatrial interval recorded in the esophagus (V-Aeso) exceeded 70 ms (four infants), transient bundle branch block during tachycardia prolonged the cycle length and the V-Aeso by 30 to 50 ms (three infants). Findings in these infants suggested prior episodes of prolonged tachycardia as the probable etiology of the cardiovascular collapse.

Prediction of digoxin treatment failure in infants with supraventricular tachycardia: role of transesophageal pacing.

Transesophageal atrial pacing was used to initiate and terminate tachycardia in 24 infants (seven female and 17 male, aged 1 to 34 days) with ECG documentation of supraventricular tachycardia. Six infants received no chronic treatment, and chronic oral digoxin prophylaxis was administered to 18 infants in an effort to prevent recurrences of tachycardia. In these 18 infants, the effectiveness of digoxin therapy in preventing the initiation of tachycardia by transesophageal pacing was compared with its ability to prevent spontaneous recurrences of supraventricular tachycardia. While receiving chronic oral digoxin therapy, tachycardia could be reinitiated in 15/18 (83%) infants. In these infants, the cycle length of tachycardia and the atrioventricular interval were the same before and during chronic digoxin treatment. Three infants in whom tachycardia could not be initiated during chronic digoxin therapy had no spontaneous recurrences during 6 months of follow-up, whereas 10/15 (67%) infants in whom tachycardia could be reinitiated had clinically significant recurrences in spite of chronic digoxin therapy. Six infants who received no chronic drug treatment had no documented recurrences during 6 months of follow-up. This study demonstrates that digoxin was effective in preventing significant spontaneous recurrences of supraventricular tachycardia in only 8/18 (44%) infants treated with digoxin. The ability to initiate supraventricular tachycardia with transesophageal pacing may be useful in determining which digoxin-treated infants are at risk for recurrence. Finally, not all infants with supraventricular tachycardia require chronic prophylaxis; six of the untreated infants had no documented recurrences.

Clinical and electrophysiologic features of fetal and neonatal paroxysmal atrial tachycardia resulting in congestive heart failure.

Those clinical and electrophysiologic features of paroxysmal atrial tachycardia (PAT) that appeared to be the cause of prenatal congestive heart failure (CHF) (hydrops fetalis) were evaluated in 12 neonates, ages 1 to 14 days, and compared with those occurring in 12 neonates, ages 2 to 34 days, who developed CHF from PAT diagnosed postnatally. Transesophageal electrophysiologic evaluation was performed after birth or at the time of PAT occurrence in the prenatal CHF group and at the time of CHF diagnosis in the postnatal CHF group. Before the electrophysiologic study, spontaneous PAT onset and termination were observed in all prenatal CHF neonates. In the postnatal CHF neonates, however, a single, prolonged episode of PAT was observed. During PAT, all neonates were observed to have a regular heart rate, normal QRS morphology and ventriculoatrial interval exceeding 80 ms. These findings suggested an orthodromic reciprocating tachycardia using an accessory atrioventricular connection as the tachycardia mechanism. In the 12 neonates with prenatal CHF, the PAT cycle length measured 243 +/- 30 ms, whereas in the 12 neonates with postnatal CHF it measured 208 +/- 19 ms (p less than 0.003). Neonates with prenatal CHF secondary to PAT appear to develop CHF from multiple PATs recurring at relatively long cycle lengths (slow heart rates), whereas postnatal CHF neonates develop heart failure symptoms during a sustained tachycardia episode with relatively short cycle lengths (fast heart rates).

Absent pulmonary valve syndrome in infancy: surgery reconsidered.

Congenital absence of the pulmonary valve leaflets is generally associated with ventricular septal defect, anular pulmonary stenosis and aneurysmal dilatation of the pulmonary arteries: the absent pulmonary valve syndrome. Symptomatic infants with this syndrome suffer primarily from respiratory insufficiency caused by bronchial compression by the dilatated pulmonary arteries, and have a high mortality rate. Asymptomatic infants are thought to do well, and may have elective surgery late in childhood. Findings in four infants with absent pulmonary valve syndrome are reviewed. One symptomatic infant was successfully treated with closure of a ventricular septal defect and pulmonary arterial plication. One previously asymptomatic infant had a fatal respiratory arrest revealing the complexity of management of infants with absent pulmonary valve syndrome.

Modes of onset ("initiating events") for paroxysmal atrial tachycardia in infants and children.

Observations of spontaneous onset of paroxysmal atrial tachycardia (PAT) in infants and children have been infrequently reported. This study reports on modes of spontaneous onset of PAT in 22 infants and 8 children in whom onset of PAT was recorded during continuous electrocardiographic recording. During PAT, all 30 patients used the normal specialized atrioventricular conduction system for ventricular activation, with atrial activation occurring through an accessory atrioventricular connection (orthodromic reciprocating tachycardia). Wolff-Parkinson-White syndrome was present in 7 patients. Analysis of the mode of onset of PAT revealed that infants initiated PAT with atrial extrasystoles or sinus acceleration (a gradual shortening of the P-P interval). In 10 infants more than 10 PAT onsets were recorded, and in these infants the mode of onset was sinus acceleration. In 7 infants, both atrial extrasystole and sinus acceleration were observed to initiate PAT. In the older children, onset of PAT followed atrial extrasystole (3 patients), ventricular extrasystole (2 patients), and sinus pause with junctional escape (3 patients). It has been previously recognized that the natural history of these "initiating events" varies with patient age. Variations in frequency of spontaneous episodes of PAT may relate to chronologic variations in frequency and type of initiating events.

Effect of intravenous propranolol or verapamil on infant orthodromic reciprocating tachycardia.

The effects of intravenous verapamil (0.15 mg/kg) and propranolol (0.2 mg/kg) with regard to atrioventricular (AV) conduction and tachycardia termination during paroxysmal atrial tachycardia were compared in 2 groups of infants (verapamil n = 14, propranolol n = 18, mean age 80 +/- 21 days, range 1 to 364). Using transesophageal recording techniques, tachycardia cycle length, AV intervals and ventriculoatrial intervals were measured before and after drug administration. Both intravenous propranolol and verapamil significantly prolonged tachycardia cycle length and AV interval (cycle length--propranolol 230 +/- 30 to 262 +/- 33 ms, p less than 0.05, verapamil 223 +/- 38 to 245 +/- 32 ms, p less than 0.05; AV interval--propranolol 98 +/- 26 to 126 +/- 38 ms, p less than 0.05, verapamil 96 +/- 19 to 109 +/- 24 ms, p less than 0.05). Neither drug prolonged the ventriculoatrial interval. Tachycardia terminated after intravenous verapamil in 11 of 14 infants (79% efficacy rate). Tachycardia terminated in 0 of 18 after intravenous propranolol (0% efficacy rate). In 8 infants an atrial deflection was recorded on the esophageal electrocardiogram at the time of tachycardia termination after intravenous verapamil, which suggested that tachycardia terminated by block occurring in the AV node. In 2 infants a ventricular deflection was recorded at the time of tachycardia termination after verapamil, which suggested that block occurred in the accessory connection. Both drugs prolonged tachycardia cycle length by prolonging AV conduction to a similar degree.(ABSTRACT TRUNCATED AT 250 WORDS)

Transesophageal atrial pacing threshold: role of interelectrode spacing, pulse width and catheter insertion depth.

This study evaluated the role of interelectrode spacing, pulse widths greater than 10 ms, and depth of esophageal insertion on minimizing transesophageal atrial pacing threshold in 30 patients aged 1 day to 77 years. Interelectrode spacings of 15, 22 and 28 mm were evaluated by establishing strength-duration curves in 2 or more serial studies in 12 patients; electrode spacing had no effect on pacing threshold. In 23 patients studied with 22-mm electrode spacing, pulse widths of 15 and 20 ms had no significant effect on current threshold requirements compared with 10-ms pulse widths. In 20 patients, pacing threshold and esophageal electrograms were obtained at 1.0- to 2.5-cm intervals with a 22-mm lead using a pulse width of 10 ms. Average minimal pacing threshold was 10.2 mA (range 4.5 to 20). The site of minimal pacing threshold was highly correlated with patient height (r = 0.987), and occurred within 1.1 cm (0 to 2.5 cm) of the site of the maximal bipolar atrial electrogram amplitude and 0.95 cm (0 to 3 cm) of the site of the maximal unipolar atrial electrogram. Bipolar electrode spacing of 15, 22 or 28 mm has little effect on transesophageal pacing threshold. In most patients, pulse widths greater than 10 ms do not significantly decrease pacing threshold. Correct catheter insertion depth is critical to minimize pacing threshold and may be predicted by either the site of the maximal atrial electrogram amplitude or patient height.

Bundle branch block during orthodromic reciprocating tachycardia onset in infants.

Transesophageal electrophysiologic studies were performed in 58 infants (age less than or equal to 1 year, median 10 days) with electrocardiographically documented orthodromic reciprocating tachycardia (ORT). The aim was to evaluate the occurrence, type and electrophysiologic effects of bundle branch block (BBB) during ORT onset. Of the 58 infants, 25 (43%) had BBB with pacing-induced tachycardia onset. BBB was initiated by single or double premature atrial extrastimuli and by burst atrial pacing; 4 infants also demonstrated BBB with spontaneous ORT onset during transesophageal study. Two of 25 infants had BBB only after intravenous procainamide. Comparison of the 25 infants exhibiting BBB at ORT onset with the 33 infants not demonstrating BBB revealed that age was not statistically different in the 2 groups, but that severity of illness (based on a 1 to 3 scale) was greater (p less than 0.05) and normal QRS ORT cycle length was shorter (p less than 0.02) in the infants with BBB. Of the 25 infants with BBB at ORT onset, 17 had left BBB, 3 had right BBB and 5 had both left and right BBB. Ventriculoatrial interval or cycle length increases during ORT with BBB in 16 of 25 (64%) infants suggested left free wall-accessory atrioventricular connections.(ABSTRACT TRUNCATED AT 250 WORDS)

Role of transesophageal pacing in evaluation of palpitations in children and adolescents.

Transesophageal atrial pacing was used to evaluate the cause of palpitations in 28 patients ages 3 to 18 years (mean 11). Palpitations were defined as the sustained (seconds to minutes) sensation of rapid heart beating. Each patient had had greater than 2 episodes of palpitations. No patient had other evidence of heart disease. Standard electrocardiogram was normal (23 of 28 patients), demonstrated ventricular preexcitation (3 of 28 patients) or demonstrated short PR interval (2 of 28 patients). In selected patients, ambulatory monitoring (11 patients) or exercise testing (3 patients) was performed but failed to demonstrate a cause of palpitations. In an effort to initiate tachycardia, a similar transesophageal atrial pacing protocol was performed in each patient. The protocol consisted of: (1) single extrastimuli at progressively closer intervals during sinus rhythm and after an 8-beat pacing train at greater than or equal to 1 cycle lengths and (2) incremental atrial pacing to the point of second-degree atrioventricular block. If this pacing regimen failed to initiate tachycardia, it was repeated during isoproterenol infusion (0.02, 0.05 and 0.1 micrograms/kg/min) and then following intravenous atropine (0.04 mg/kg) administration. During the study, tachycardia was initiated in 20 of 28 patients (71%) (14 of 15 patients greater than 10 years, 6 of 13 patients less than or equal to 10 years; p less than 0.01, Fisher's exact test). Electrophysiologic characteristics of induced tachycardia suggested reentry within the atrioventricular node (8 of 20 patients) or orthodromic reciprocating tachycardia (12 of 20 patients). In 3 of 12 patients with orthodromic reciprocating tachycardia, a transition to atrial fibrillation was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac and skeletal myopathy associated with cardiac dysrhythmias.

Electrophysiologic studies, echocardiograms, cardiac catheterizations and histologic and biochemical analyses of skeletal muscle biopsies were performed in 10 patients (aged 10 to 37 years, mean 21) who had dysrhythmias as the initial manifestation of cardiomyopathy. Presenting symptoms and signs attributable to dysrhythmias included sudden cardiac arrest in 2 patients, syncope in 3, presyncope in 3 and palpitations in 2. There was no clinical evidence of skeletal muscle weakness in any patient. Multicatheter electrophysiologic evaluation established diagnoses of ventricular tachycardia in 6 patients, primary atrial tachycardia in 2 and third degree infra-Hisian heart block in 1 patient. One patient presenting with palpitations had no inducible arrhythmia or conduction disturbance. Echocardiographic, angiographic and hemodynamic studies demonstrated previously unsuspected dilated cardiomyopathy in 7 patients and restrictive cardiomyopathy in 3. Skeletal muscle histologic characteristics were abnormal in all 10 patients; increases in lipid droplets and endomysial fibrosis were the characteristic findings. Serum free carnitine and short- and long-chain acylcarnitine were normal in 9 patients. However, skeletal muscle long-chain acylcarnitine was reduced in 9 patients. These findings support the concept that in certain patients presenting with dysrhythmias, the dysrhythmia may be a manifestation of cardiac and skeletal (that is, generalized) myopathy.

Usefulness of combined propranolol and verapamil for evaluation of surgical ablation of accessory atrioventricular connections in patients without structural heart disease.

Successful surgical ablation of atrioventricular (AV) accessory connections may be confirmed during postoperative electrophysiologic testing by the absence of accessory connection conduction in both the anterograde and retrograde directions. Whereas the former may be readily apparent by examination of the surface electrocardiogram during sinus rhythm or atrial pacing, assessment of the latter may be complicated by the frequent presence of enhanced retrograde AV nodal conduction in the postoperative period. Consequently, availability of interventions that selectively affect AV nodal conduction and refractoriness without concomitant effects on accessory connections may be helpful for assessing the success of the surgical procedure. In this study the effects of combined propranolol and verapamil administration on electrophysiologic properties of the AV node and the accessory AV connection were assessed both pre- and postoperatively in 17 patients (12 men and 5 women, mean age 33 years) undergoing surgical ablation of accessory connections. Preoperatively, electrophysiologic characteristics of all but 1 of the accessory AV connections were unaffected by propranolol and verapamil administration. Postoperatively, on the other hand, propranolol and verapamil significantly prolonged both the retrograde AV node effective refractory period (baseline: 272 +/- 34 ms vs after drugs: 384 +/- 70 ms [p less than 0.0001]) and the shortest cycle length maintaining 1:1 ventriculoatrial conduction (baseline: 357 +/- 99 ms vs after drugs: 485 +/- 64 ms [p less than 0.0001]). Late postoperative electrophysiologic evaluation (7 +/- 3 weeks) revealed no evidence of residual accessory AV connection conduction, and all patients remain asymptomatic at 21 +/- 10 months follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Success of re-use of cardiac electrode catheters.

The feasibility of cardiac electrode catheter re-use was prospectively evaluated over a 5-year period (1981 to 1986), during which time 178 catheters were used 1,526 times for 847 electrophysiologic procedures. Detailed records of catheter testing and use were maintained. No complications were encountered during the study period. All re-used catheters functioned for cardiac pacing and electrographic recording. Surveillance cultures and biologic indicators revealed that adequate sterilization procedures were used. Thus, electrode catheters may be safely re-used provided a thorough cleaning, testing and record-keeping system is instituted. This may result in lower patient cost (approximately $30 per re-use vs [200 per single use) for electrophysiologic catheterization.

Mechanisms of regular, wide QRS tachycardia in infants and children.

A regular wide QRS tachycardia was electrocardiographically documented in 32 patients aged 1 month to 18 years. The mechanisms of the tachycardia were evaluated using standard multicatheter electrophysiologic techniques. These mechanisms included (1) orthodromic reciprocating tachycardia with bundle branch aberration (seven patients), (2) antidromic reciprocating tachycardia using single (three patients), or multiple (three patients) atrioventricular connections (Kent bundles), (3) atrial flutter with ventricular preexcitation over accessory connections (eight patients), (4) reciprocating tachycardia using a nodoventricular connection (Mahaim fiber) (five patients), and (5) ventricular tachycardia (six patients). Regular side QRS tachycardias are not rare in pediatric patients. Their mechanisms can be quite complex, and electrocardiographic analysis with respect to QRS configuration, heart rate, or the presence or absence of ventriculoatrial dissociation is not sufficient for diagnostic purposes. Our results show that considerable understanding of the mechanism of regular, wide QRS tachycardias can be obtained by multicatheter electrophysiologic study. Understanding the mechanism is essential in order to make rational use of available therapeutic options.

Usefulness of disopyramide for prevention of upright tilt-induced hypotension-bradycardia.

Susceptibility to transient hypotension-bradycardia of neurally mediated origin has been attributed in part to accentuated afferent neural traffic arising from cardiopulmonary mechanoreceptors, and consequently, may be diminished by agents with anticholinergic and negative inotropic effects, such as disopyramide phosphate. This study assessed electrocardiographic and hemodynamic responses to upright tilt testing (alone or during isoproterenol infusion) before and after disopyramide therapy in 10 patients (age range 16 to 74 years) with recurrent syncopal episodes of neurally mediated origin. Untreated, syncope occurred at less than or equal to 7 minutes of tilt alone (6 patients) or tilt plus isoproterenol at less than or equal to 3 micrograms/min (4 patients) and was associated with hypotension (mean arterial pressure, 40 +/- 16 mm Hg vs baseline 76 +/- 10 mm Hg, p less than 0.001) and inappropriate heart rate slowing (mean heart rate, 59 +/- 39 beats/min vs baseline 88 +/- 18 beats/min, p less than 0.005). After oral disopyramide 150 mg 3 times daily (mean plasma level, 3.0 +/- 0.64 micrograms/ml), all patients tolerated 10 minutes of both tilt and tilt plus isoproterenol (maximum dose, 3 micrograms/min) without symptoms, hypotension (mean arterial pressure; tilt 1 min, 79 +/- 7 mm Hg vs tilt 10 min, 77 +/- 8 mm Hg, difference not significant) or bradycardia (mean heart rate; tilt 1 min, 81 +/- 12 beats/min vs tilt 10 min, 83 +/- 11 beats/min, difference not significant). Furthermore, during subsequent 20 +/- 5 months of disopyramide therapy, all but 1 patient remain asymptomatic. Thus, oral disopyramide may be effective for preventing inducible and spontaneous neurally mediated syncope.

Transesophageal study of recurrent atrial tachycardia after atrial baffle procedures for complete transposition of the great arteries.

Transesophageal study was used for diagnosis and treatment of 51 episodes of tachycardia in 13 patients with complete transposition of the great arteries who had undergone atrial baffle procedure. At the time of atrial baffle procedure, patients were 6 to 36 months old (mean 23). Tachycardia (1 to 17 episodes per patient) first occurred 1 to 23 days (4 patients) or 1.8 to 12 years (9 patients) after atrial baffle. Transesophageal study was performed using a bipolar silicone rubber-coated catheter. Tachycardia conversion was accomplished with stimulation bursts using 4 to 10 stimuli 9.9 ms in duration at 20 to 28 mA and an interstimulus interval of 50 to 100 ms less than the atrial cycle length. All tachycardia episodes had regular atrial cycle lengths ranging from 200 to 350 ms. In 12 patients, second-degree atrioventricular (AV) block was observed during tachycardia, suggesting primary atrial tachycardia. However, in 1 patient, occurrence of AV block always resulted in tachycardia termination, suggesting the presence of AV reentrant tachycardia. Transesophageal stimulation converted 48 of 51 tachycardia episodes to sinus/junctional rhythm. Ten tachycardia episodes in 6 patients were transiently converted to atrial fibrillation lasting 3 seconds to 28 minutes before spontaneous conversion to sinus junctional rhythm. Conversion attempts were unsuccessful on 3 occasions. Acceleration of ventricular rate after stimulation necessitated DC cardioversion on 1 occasion. Conversion was not achieved in 2 tachycardia episodes using stimuli less than 10 mA. Transesophageal study is a safe and effective minimally invasive technique for diagnosis and treatment of tachycardia in infants and children who have had atrial baffle for transposition of the great arteries.

Therapeutic and diagnostic utility of adenosine during tachycardia evaluation in children.

Adenosine has become the drug of choice for termination of regular, normal QRS tachycardia. Initial studies in adult and pediatric patients have shown that the drug is effective for tachycardias using the atrioventricular (AV) node as an integral part of the tachycardia circuit and has few serious side effects. Experience with adenosine administration in children was reviewed to examine the diagnostic and therapeutic usefulness, effective dose, and adverse effects of adenosine. Adenosine was administered to 38 children during 50 separate electrophysiologic evaluations. Eleven patients had structural or acquired heart disease. Tachycardia mechanisms included orthodromic-reciprocating tachycardia using an accessory AV connection (23 patients), primary atrial tachycardia (6 patients), AV node reentrant tachycardia (3 patients), ventricular tachycardia (2 patients), postoperative junctional tachycardia (1 patient), and antidromic-reciprocating tachycardia (1 patient). Adenosine successfully terminated 51 of 53 episodes (96%) of tachycardia using the AV node, 5 of 10 primary atrial tachycardias, 1 of 1 junctional tachycardia, and 1 of 3 ventricular tachycardias. Reinitiation of tachycardia was seen after 16 of 58 successful terminations (28%), reducing the effectiveness to 39 of 53 (74%) for tachycardia requiring the AV node. Average effective dose was 132 micrograms/kg, range 50 to 250 micrograms/kg, and was slightly higher for peripheral (147 micrograms/kg) than for central (120 micrograms/kg) administration. Significant complications occurred in 4 of 38 patients, including atrial fibrillation, accelerated ventricular tachycardia, apnea, and 1 minute of asystole. Although adenosine is useful therapeutically and diagnostically in children with tachycardia, its effectiveness is limited by tachycardia reinitiation and adverse effects. Higher doses may be required for peripheral intravenous administration.