Efficacy and safety of intravenous and oral nadolol for supraventricular tachycardia in children.

The efficacy and safety of oral nadolol in supraventricular tachycardia were evaluated prospectively in 27 children (median age 5.5 years). Fifteen patients had an unsuccessful trial of digoxin therapy. Intravenous nadolol was given to seven patients during electrophysiologic study; five of these had an excellent response and two had a partial response (25% decrease in tachycardia rate). Six of these patients had a similar response to oral nadolol. Twelve patients received both propranolol and nadolol. Among six patients, intravenous propranolol was successful in four and unsuccessful in two; all six had a similar response to oral nadolol. With oral propranolol, tachycardia was well controlled in four patients and persistent in two; five of five patients had a similar response to oral nadolol. Twenty-six patients were treated with oral nadolol; the arrhythmia was well controlled in 23, 2 had recurrent tachycardia and 1 patient had tachycardia at a 25% slower rate. The effective dose of nadolol ranged between 0.5 and 2.5 mg/kg body weight once daily (median dose 1 mg/kg per day). During follow-up (3 to 36 months), compliance and tolerance were excellent; excluding 2 patients with reactive airway disease who developed wheezing, only 3 (12%) of 24 had side effects necessitating a change in drug therapy. Once a day nadolol is a safe and effective agent in the management of supraventricular tachycardia in children. Its long-term efficacy can be predicted by the short-term response to intravenous nadolol or propranolol during programmed electrophysiologic study.

Percutaneous transluminal balloon angioplasty of stenotic standard Blalock-Taussig shunts: effect on choice of initial palliation in cyanotic congenital heart disease.

To date, attempted balloon dilation of stenotic standard Blalock-Taussig shunts has been largely disappointing. It has been suggested that this may be due to the use of balloons of insufficient diameter. Balloon dilation of stenotic Blalock-Taussig shunts was attempted with use of relatively large balloons in five patients (11 to 67 months old) with cyanotic heart disease who were becoming progressively cyanotic and polycythemic (hemoglobin 17.9 +/- 1.1 g/dl) because of discrete shunt stenosis at the site of pulmonary anastomosis. Balloon diameters selected were equal to or within 1 mm of the unobstructed proximal shunt diameter. Before balloon dilation the diameter at the site of the stenosis was 2.8 +/- 0.8 mm (range 1.7 to 4); after balloon dilation it was 5.7 +/- 1.1 mm (range 4.5 to 7.5). The diameter increased in all patients (range 2.0 to 3.5 mm); the mean increase was 2.8 +/- 0.2 mm (p less than 0.005). Expressed as a percent, the increase in diameter at the stenosis ranged from 80% to 182.4% (mean 108.2 +/- 16.8%). Before balloon dilation the systemic oxygen saturation was 72.8 +/- 9.2% (range 55% to 80%) and after balloon dilation it was 83.6 +/- 2.9% (range 80% to 87%). A satisfactory increase (range 6% to 25%) in blood oxygen saturation was seen in all patients; the mean increase was 10.8 +/- 3.2% (p less than 0.01). At follow-up, the oxygen saturation by pulse oximetry was 85.8 +/- 2.9% (mean 5.8 +/- 1.7 months after balloon dilation) and the hemoglobin was 15.6 +/- 1.9 g/dl (mean 6.6 +/- 1.5 months after balloon dilation).(ABSTRACT TRUNCATED AT 250 WORDS)

Ectopic automatic atrial tachycardia in children: clinical characteristics, management and follow-up.

Ectopic automatic atrial tachycardia, an uncommon type of supraventricular tachycardia in children and adults, has been reported to be resistant to medical therapy, and surgical or cryoblation has been recommended. This report describes 10 infants and children (median age 6 months; range birth to 7.5 years) with automatic atrial tachycardia and their management and follow-up. Digoxin alone was unsuccessful in controlling tachycardia in all 10 patients but decreased the tachycardia rate by 5 to 20% in 8. Intravenous (0.1 mg/kg body weight per dose) and oral propranolol successfully suppressed tachycardia in three of five patients and oral propranolol successfully controlled tachycardia in two of five other patients. Class I antiarrhythmic agents--quinidine (three patients), procainamide (four patients) and phenytoin (three patients)--did not control tachycardia in any patients but made the tachycardia rate worse in three patients. Intravenous (5 mg/kg per dose) and oral amiodarone suppressed tachycardia in three of four patients and oral amiodarone suppressed it in another patient. Thus, intravenous propranolol and amiodarone were effective in acutely suppressing automatic ectopic atrial tachycardia and predicted the response to long-term oral therapy. One patient had persistent tachycardia after surgical ablation of the high right atrial ectopic focus, and another patient had unsuccessful catheter ablation of the high right atrial ectopic focus (25 J). During follow-up (10 to 28 months), ectopic atrial tachycardia resolved completely in four patients and was well controlled in four patients.

Procainamide elimination kinetics in pediatric patients.

Procainamide kinetics were studied in six children after a single intravenous dose. Two-compartment kinetic analysis of serum concentration-time curves of five children, who received a dose of 5.5 +/- 0.9 mg/kg (mean +/- SD), revealed the following values for kinetic parameters: distribution half-life, 10.3 +/- 3.4 min; elimination half-life, 1.7 +/- 0.1 hr; elimination constant, 1.2 +/- 0.3 hr-1; plasma clearance 19.4 +/- 2.0 ml/min/kg, and steady-state volume of distribution, 2.2 +/- 0.3 l/kg. A sixth patient, who received an accidental overdose of 28 mg/kg, had altered elimination kinetics due to drug-induced hypotension. N-acetylprocainamide (NAPA) was detected in serum samples obtained soon after procainamide dosing and peak concentrations were attained at 1 to 2 hr. NAPA levels were lower than corresponding procainamide concentrations at most sampling periods. The findings of short elimination half-life and rapid plasma clearance of procainamide in children suggest that continuous intravenous infusion may be necessary to maintain therapeutically effective plasma concentrations in these patients.

Ventricular septal defect in the first year of life.

Of approximately 22,000 live births in the region under study during the last 4 years, ventricular septal defect (VSD) was identified as the primary or isolated congenital heart lesion in 124 infants who were followed up from birth for a minimum of 1 year (incidence, 5.7 per 1,000 live births). Doppler color flow mapping was performed in 93 of 124 patients; 47 had a muscular VSD and 46 had a perimembranous VSD. Only 1 patient had 2 muscular VSDs. None had a subpulmonic type of defect. Of 124 patients, 14 were lost to follow-up. Spontaneous closure was seen in 18 patients (42%) in the muscular group, in 9 (23%) in the perimembranous group and in 10 patients (37%) in the unclassified group by the end of the first year. The overall rate of spontaneous closure was 34% by the end of the first year. Congestive heart failure developed in 2 of 46 patients with muscular VSD and in 12 of 47 patients with perimembranous VSD. In the first year, 2 patients with muscular VSD as opposed to 5 with perimembranous VSD required surgery. Doppler color flow mapping is a valuable aid in the diagnosis of VSD and may be one reason for the observed increase in the incidence of VSD. The overall prognosis appeared much better in the muscular than the perimembranous type of VSD.

Characteristics and management of chaotic atrial tachycardia of infancy.

Chaotic atrial tachycardia was observed in 7 infants without underlying structural heart disease. Clinical presentation and approach to management are discussed, with particular attention to the use of propafenone for this uncommon pediatric arrhythmia.

Dual atrioventricular nodal pathways: a benign finding in arrhythmia-free children with heart disease.

The incidence and significance of dual atrioventricular (A-V) nodal pathways are described in 78 children with associated congenital or acquired heart disease. None of these patients had clinical or electrocardiographic evidence of arrhythmia. Dual A-V nodal pathways were observed in 35 percent of the preoperative group and in 33 percent of the postoperative group. Despite this substrate for A-V nodal reentry, supraventricular tachycardia was neither induced during electrophysiologic evaluation nor did it develop clinically over a follow-up period of 1 month to 15 years. It is concluded that dual A-V nodal pathways are common and may be a benign finding in arrhythmia-free children with heart disease.

Hemodynamics and intracardiac conduction after operative repair of tetralogy of Fallot.

Electrophysiologic studies were performed in 47 children aged 3 to 18 years, 15 of whom had cardiac arrhythmias 1 to 15 years after repair of tetralogy of Fallot. Six exhibited sinus or atrioventricular nodal dysfunction, 8 had ventricular extrasystoles, and 1 had supraventricular tachycardia. Hemodynamic and electrophysiologic data were obtained at postoperative catheterization. Although electrophysiologic responses were abnormal in a proportion of both the children with and those without arrhythmia, hemodynamic values were similar. Three of 6 children with impaired sinus impulse generation or atrioventricular nodal conduction had a prolonged A-H interval, and in 3 Wenckebach heart block developed at low pacing rates. Ventricular ectopic rhythm was not associated with any particular abnormality of basic intracardiac conduction intervals. Thus, arrhythmias and conduction abnormalities are not consistently related to residual right ventricular hypertension. Abnormalities in electrophysiologic function are common after repair of tetralogy of Fallot in patients with sinus rhythm and may have prognostic implications for these patients.

Currarino-Silverman syndrome (pectus carinatum type 2 deformity) and mitral valve disease.

Currarino-Silverman syndrome is a rare disorder characterized by premature fusion of manubrio-sternal joint and the sternal segments, resulting in a high carinate chest deformity; it is frequently associated with congenital heart disease. Among the various heart lesions reported in this syndrome, mitral valve disease and coarctation of the aorta have not yet been described (to our knowledge). Our report consists of five children with this syndrome, four of whom had mitral valve disease, with an associated coarctation of the aorta in one patient. The fifth patient had an innocent heart murmur.

Radiologic abnormalities of the sternum in Turner's syndrome.

Various skeletal abnormalities, including chest deformity, have been reported with Turner's syndrome. We report radiologic findings of the sternum on lateral chest roentgenograms in 15 children and adolescents with Turner's syndrome, whose ages ranged between 2 weeks and 20 years. Eight had associated congenital heart disease (CHD). Twelve patients (80 percent) had various sternal abnormalities; 5 had short sternum, 3 had premature fusion of the manubrio-sternal junction, and 4 had premature fusion of the mesosternum. Five had decreased ratio of sternal body to manubrium. Three patients had two ossification centers of the manubrium. Four patients had bowing of the mesosternum; three of these had mild pectus excavatum also. In this series, children with and without CHD had similar sternal abnormalities. Although not pathognomonic, sternal abnormalities on a lateral chest roentgenogram are common skeletal abnormalities associated with Turner's syndrome and are independent of associated CHD. In our series of 15 patients, 10 had monosomy (45,X) on blood karyotype; 7 of them had associated CHD. This is the first systematic analysis of radiologic abnormalities of the sternum in Turner's syndrome and includes findings not previously reported.

Recurrent obstruction after subclavian flap repair of coarctation of the aorta in infants. Can it be predicted or prevented?

Recoarctation is a problem in some patients after subclavian flap aortoplasty. To investigate the reason for recoarctation, we reviewed the records of 26 infants who underwent subclavian flap repair for symptomatic coarctation of the aorta at less than 3 months of age between June, 1979, and December, 1983. Age at repair ranged from 2 to 65 days (median 16 days) and weight from 2.1 to 4.9 kg (median 3.4 kg). In 14 patients the coarctation was associated with significant intracardiac defects (complex in six). There were two intraoperative deaths and one early death (surgical mortality 12%). The survivors were followed from 6 weeks to 66 months (median 12 months). Five survivors (22%), all operated on at less than 14 days of age, developed severe recoarctation 6 weeks to 6 months (median 5 months) after repair. The obstruction appeared to be due to lumen obliteration by shelf-life posterior wall tissue. Morphometric analysis of preoperative angiograms showed no correlation between recoarctation and distance between the left subclavian artery and the site of coarctation, length of the isthmus, diameter of the isthmus, combined cross-sectional area of the left subclavian artery and isthmus, or the ratio of the combined cross-sectional area of the left subclavian artery and isthmus to the cross-sectional area of the descending thoracic aorta. Recoarctation did not correlate with weight at operation, but it correlated significantly with age at aortoplasty (p = 0.02). The results suggest that intrinsic abnormalities of the periductal aortic wall are responsible for recoarctation after subclavian flap aortoplasty. Particular attention to this abnormal tissue at repair may prevent early recurrence in young infants.

Congenital systemic arteriovenous fistula between the descending aorta, azygos vein, and superior vena cava.

Various congenital systemic arteriovenous fistulas have been described. An unusual case of congenital systemic arteriovenous fistula arising from the descending aorta and draining separately into the superior vena cava, the azygos vein, and the innominate vein is reported. Clinically, this case simulated a patent ductus arteriosus. To our knowledge, this particular anomaly has not been described before.

Sinus and atrioventricular nodal function: Preoperative and early postoperative assessment in children.

Ninety-three rapid atrial pacing studies were performed in 38 children to compare preoperative and early postoperative function of the sinus and atrioventricular (AV) nodes. The interval between the preoperative and postoperative studies was under 6 months in the majority of patients. Postoperative studies were performed within 48 hours of operation and between 4 and 8 days after operation. Sinus nodal function as measured by sinus nodal recovery time (SNRT) was an unreliable index in determining depression since the number who improved postoperatively (10/55) was nearly equal to the number that worsened (12/55). The majority who had abnormal function postoperatively demonstrated a junctional rather than sinus recovery focus. This finding appears a more definitive and more reproducible indicator of sinus node depression in the postoperative patient. Postoperative AV nodal function was decreased (as measured by the cycle length [CL] at which Wenckebach periodicity occurred) in 15 of 55 studies (27%) of the entire group. There was nearly an equal chance for improvement (24%) in function. This also applied to those patients who had sequential studies. Therefore, this method of assessment for AV nodal function was unreliable, or else the operation did not affect the node significantly. The latter is unlikely in view of late postoperative data. The greatest utility of this test was to determine the capability for AV conduction in certain patients with slow escape rhythms in the absence of surface P waves, and to differentiate complete heart block from AV dissociation when atrial activity was absent. Despite the variability of effects on the sinus and AV nodes in these patients, those who demonstrated depression had a significantly higher incidence of dysrhythmias (80% of patients with sinus nodal depression and 100% of patients with AV nodal depression).

Pharmacokinetics of nadolol in children with supraventricular tachycardia.

The pharmacokinetics of intravenous and oral nadolol, a long-acting beta-adrenoceptor blocking agent, were investigated in six children receiving the drug for treatment of supraventricular tachycardia. In the youngest patient (age 3 months), no distribution phase was seen. In children younger than 22 months of age, nadolol is more rapidly eliminated (t1/2 = 4.3 hours or less) than in older children, in whom elimination is more similar to that in adults (t1/2 approximately 7.3-15.7 hours). After intravenous administration, nadolol displayed two-compartment pharmacokinetics with a distribution phase (t1/2 = 0.2-1.1 hours) followed by elimination. Large changes in nadolol pharmacokinetics may occur during the first year of life. Nadolol should be used cautiously in infants.

Pharmacokinetics of moricizine in young patients.

Moricizine is a novel phenothiazine antiarrhythmic agent that depresses the activity of ectopic foci, has a low incidence of adverse effects relative to other agents, and is useful in treating pediatric atrial ectopic tachycardia. A study was conducted to determine the pharmacokinetics of moricizine in children after oral administration. Moricizine was isolated from frozen serum obtained from four male patients (ages 7, 8, 9, and 18 years) receiving the drug for supraventricular tachycardia and analyzed by high-performance liquid chromatography with ultraviolet detection according to an established protocol. Peak serum levels were between 400 and 2000 ng/mL. Elimination of moricizine did not follow simple single-compartment pharmacokinetics. In three patients we observed an increase or slower decline in blood level occurring after 4 hours. Because of the paroxysmal nature of the tachycardias, decreases in patient heart rate could not be correlated with moricizine blood level. These results suggest that the pediatric pharmacokinetics of moricizine excretion are complex and may differ from those seen in adults.

Experience with moricizine HCl in children with supraventricular tachycardia.

Eight children, age between 4.5 and 19 years were treated with moricizine for supraventricular tachycardia during the last 3 years. The tachycardia was documented by surface electrocardiogram (ECG), and/or by ambulatory ECG in all the children and the mechanism of tachycardia was determined by previously published surface ECG and electrophysiologic criteria in all but one child. Of the eight children, three had atrial ectopic tachycardia, three had automatic junctional ectopic tachycardia, one had atrioventricular (AV) nodal reentry tachycardia and one had atrial reentry. All the children except one had failed trial of two or more antiarrhythmic drugs prior to moricizine therapy. The duration of moricizine therapy ranged from 4 days to 25 months. In three of the eight children (patients 3, 5 and 7), who presented with AV nodal reentrant tachycardia, automatic junctional ectopic tachycardia and atrial ectopic tachycardia, respectively, moricizine therapy was effective in restoring sinus rhythm and controlling the clinical tachycardia. Only one child (patient 1) developed proarrhythmia, an episode of fast, narrow-QRS supraventricular tachycardia lasting for 30 s, on the third day of therapy. This was subsequently confirmed by electrophysiologic study to be AV nodal reentrant tachycardia. The other side effects noted were non-cardiac, not dose-dependant and did not require dis-continuation of therapy. Based on our small series and those of others, moricizine, a newer class I anti-arrhythmic agent, has a limited but useful role in the management of recalcitrant type of supraventricular tachycardia, such as ectopic atrial and junctional tachycardia in children.

Natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy.

BACKGROUND AND HYPOTHESIS: Valvar pulmonary stenosis is a common congenital heart defect. Progression of stenosis over time, even when mild initially, has been shown by serial cardiac catheterization studies in children and adults. We studied the natural history of asymptomatic valvar pulmonary stenosis diagnosed in infancy with two-dimensional echocardiography and Doppler method. METHODS: Between November 1986 and March 1993, 51 infants in the Northeast Tennessee and Southwest Virginia region were clinically diagnosed to have isolated valvar pulmonary stenosis. In 40 patients, the diagnosis was confirmed by two-dimensional echocardiogram/Doppler and color-flow mapping study at the time of presentation, and only their course is reported. Of 40 infants, six asymptomatic infants (15%) showed rapid progression of pulmonary stenosis over a relatively short period of time. Within the first 6 months of life, three of the six infants showed worsening of the stenosis needing intervention (one had surgical valvectomy and the others had percutaneous balloon valvuloplasty). The three other infants showed a more gradual increase of pulmonary stenosis over the first 2 years of life. RESULTS: Pulmonary stenosis even when mild can worsen in infancy, and it is not possible to predict which patients will follow this course. In our group of asymptomatic infants with initial mild pulmonary stenosis, 15% developed significant stenosis that needed intervention. CONCLUSION: We recommend frequent follow-up of asymptomatic infants with mild pulmonary stenosis during the first 2 years of life to detect rapid progression that may need intervention.

Petit mal seizure in a child with Marfan's syndrome.

We report a case of a 17-year-old white girl with Marfan's syndrome and generalized absence-type seizures since 11 years of age. A video EEG recording for six hours demonstrated 52 episodes of clinical generalized absence-type seizures and three-per-second spike and wave epileptiform discharges, characteristic of petit mal epilepsy. Sodium valproate therapy was successful in controlling her seizures. In this article, we review various possible causes of epilepsy in patients with Marfan's syndrome.

An unusual peripheral vascular response to dopamine in a neonate.

We report a case of a neonate who developed hypotension immediately after birth, and needed dopamine infusion to sustain his blood pressure and tissue perfusion. He developed cyanosis of his extremity immediately after dopamine was started via peripheral line and improved spontaneously after dopamine was stopped. This happened repeatedly at various sites and at lower concentrations of dopamine. Subsequently, dopamine was replaced by dobutamine and the patient did well. We conclude that some neonates can show heightened alpha-adrenergic response to dopamine and this can lead to ischemic vascular events. Dopamine infusion in neonates should be started at a low-dose via central line.

Natural history of isolated ventricular septal defect in the first five years of life.

BACKGROUND: Isolated ventricular septal defect (VSD) is the most common congenital heart defect. Incidence, prevalence, and clinical outcomes of VSD have been reported to vary significantly in different geographic areas. Spontaneous closure of VSD, in children, by various methods has been described. HYPOTHESIS: This prospective study was undertaken to evaluate natural history of patients with VSD in the first five years of life in the Northeast Tennessee and Southwest Virginia region. METHODS: Between December 1, 1998 and October 31, 1990, 124 infants were diagnosed clinically with isolated VSD. VSDs were classified as muscular, perimembranous, malalignment, or subpulmonic types by 2-dimensional echocardiogram with color flow mapping. Cardiac catheterization and angiocardiography were performed in 14 patients when clinically indicated. These patients were followed for at least five years. RESULTS: Overall spontaneous closure of VSD was 34% at one year and 67% at five years. Twenty-five percent of perimembranous and 4% of muscular VSDs required surgery by five years. The spontaneous closure rate of muscular VSD was twice that of the perimembranous type, though the relative distribution of both types was almost equal. Overall, 22% of children with VSD need follow-up after the fifth year of life. CONCLUSION: The overall clinical outcome of muscular VSD was consistently better than that of the perimembranous type, though 17% of muscular VSDs, irrespective of size, were open at 5 years of age and needed long-term follow-up.