Management of atrial flutter after the Fontan procedure.

OBJECTIVES: The purpose of this study was to review the management of atrial flutter occurring after the Fontan procedure. BACKGROUND: Atrial flutter occurs frequently after the Fontan procedure and is often hemodynamically poorly tolerated. METHODS: The patients' charts were reviewed for relevant information. RESULTS: Between 1984 and 1992, 18 patients had atrial flutter after the Fontan procedure. The underlying heart defect was tricuspid atresia in nine, mitral atresia in six and double inlet left ventricle in three. All but three patients had undergone previous palliative surgery. The time interval from Fontan operation to atrial flutter was < 1 day to 16 years (mean 3.7 years). Seven had early atrial flutter before leaving the hospital. Electrophysiologic study in 15 showed sinus node dysfunction in 12. Atrial flutter was inducible in all patients, and 13 had > 1 flutter configuration. Digoxin and a variety of other antiarrhythmic agents (mean 2.7 drugs/patient) were tried with poor results. Only digoxin, amiodarone, flecainide and propafenone showed some benefit when used alone or in combination. Antitachycardia pacemakers were implanted in 16 patients (endocardial 14, epicardial 2) and, with drugs, were useful in 8 (50%). Because atrial flutter was resistant to treatment, right atriectomy was performed in three patients (with benefit in two, one death), successful radiofrequency catheter His bundle ablation in one patient and catheter ablation of atrial flutter in three patients (two failed, one partial success). One patient underwent heart transplantation, and two died suddenly. Another died of complications after an elective epicardial pacemaker replacement procedure. CONCLUSIONS: Atrial flutter after the Fontan procedure is difficult to control. Aggressive drug and antitachycardia pacemaker therapy help about half of the patients. When these measures fail, other options, such as atriectomy, His bundle ablation or catheter ablation of atrial flutter, need consideration. The risk of sudden death justifies the use of such aggressive treatment methods.

Radiofrequency catheter ablation of right ventricular outflow tachycardia in children and adolescents.

OBJECTIVES: The current study reviews the safety and efficacy of radiofrequency catheter ablation for the treatment of right ventricular outflow tachycardia in children and adolescents and describes a modified method for mapping the tachycardia focus. BACKGROUND: Although radiofrequency catheter ablation has proved highly effective for the treatment of supraventricular tachycardia during childhood and adolescence, its application in children with idiopathic right ventricular outflow tachycardia has been limited. METHODS: Six children (mean [+/- SD] age 10.6 +/- 2.4 years, range 6 to 16) with right ventricular outflow tachycardia underwent seven radiofrequency catheter ablation procedures. The mean tachycardia cycle length was 323 +/- 24 ms (range 300 to 360). Two multipolar catheters were positioned in the right ventricular outflow tract to map the tachycardia focus. RESULTS: Radiofrequency catheter ablation was successful in five (83%) of the six children (95% confidence interval 36% to 99%). At successful ablation sites, local endocardial activation time preceded the surface QRS onset by 46 +/- 5 ms (range 37 to 57), and there was concordance of the 12-lead pace map and the electrocardiogram (ECG) in 11 (one patient) to 12 ECG leads (four patients). One patient developed complete right bundle branch block during radiofrequency catheter ablation. There were no additional complications and no clinical recurrences over a mean follow-up period of 12.7 +/- 3.8 months (range 9 to 22). CONCLUSIONS: These results suggest that radiofrequency catheter ablation is a safe and effective treatment for right ventricular outflow tachycardia during childhood and adolescence. In addition, tachycardia mapping may be enhanced by use of a multipolar right ventricular outflow catheter technique.

Radiofrequency catheter ablation of incessant, medically resistant supraventricular tachycardia in infants and small children.

OBJECTIVES: This study retrospectively evaluates initial experience with radiofrequency catheter ablation in a group of seven infants and small children with a history of incessant, medically resistant supraventricular tachycardia. METHODS: Before attempted catheter ablation, all patients had had unsuccessful conventional medical therapy (with digoxin or propranolol, or both) and, in addition, each continued to have daily episodes of supraventricular tachycardia while taking amiodarone or a class IC antiarrhythmic agent alone or in combination. The average patient age was 10 months (range 1 to 27) and the average patient weight was 6 kg (range 3 to 13). Electrophysiologic diagnosis included reentrant supraventricular tachycardia in six patients and atrial ectopic tachycardia in one patient. RESULTS: These seven patients underwent a total of nine catheter ablation procedures. The atrial approach to ablation was employed in eight of the nine procedures. Overall, radiofrequency catheter ablation was totally successful in five of the seven patients, partially successful in one patient and unsuccessful in the remaining patient. The combination of radiofrequency catheter ablation and surgical ablation was successful in controlling tachycardia in all patients; with at least 5 months of follow-up study, no patient has had a recurrence of supraventricular tachycardia or reappearance of a delta wave. CONCLUSIONS: Surgical ablation of arrhythmogenic substrates in the pediatric age group, although rarely indicated, has been found in the past to be safe and effective. Our initial experience with radiofrequency catheter ablation in infants and small children demonstrates that this procedure is a promising nonpharmacologic therapeutic alternative to surgical ablation.

Pulmonary/systemic flow ratio in children after cavopulmonary anastomosis.

OBJECTIVES: This study attempted to provide a formula for calculation of the pulmonary/systemic flow ratio in children after bidirectional cavopulmonary anastomosis. BACKGROUND: With the bidirectional cavopulmonary anastomosis, only the superior vena cava blood is oxygenated by the lungs. The inferior vena cava flow recirculates into the systemic circulation. The ratio of these flows will determine systemic arterial saturation. METHODS: According to the Fick principle, 1) Systemic cardiac output (liters/min) = Pulmonary venous flow + Inferior vena cava flow; 2) Systemic blood oxygen transport (ml/min) = Pulmonary venous blood oxygen transport + Inferior vena cava blood oxygen transport. By substituting the first equation into the second, Pulmonary/systemic flow ratio = (Systemic saturation - Inferior vena cava saturation)/(Pulmonary venous saturation - Inferior vena cava saturation). RESULTS: We applied the third formula to data obtained from 34 catheterizations in 29 patients after bidirectional cavopulmonary anastomosis. Mean [+/- SD] age at operation was 1.70 +/- 1.43 years, and mean age at catheterization was 2.95 +/- 1.65 years. The pulmonary/systemic flow ratio calculated for all 29 patients was 0.58 +/- 0.09. Of 17 patients with aortography, 10 had systemic to pulmonary collateral vessels. Patients with collateral vessels had a significantly higher pulmonary/systemic flow ratio (0.61 +/- 0.07 vs. 0.53 +/- 0.07, respectively, p < 0.02) and systemic saturation (88 +/- 4% vs. 82 +/- 4%, respectively, p < 0.002) than those without collateral vessels. The pulmonary/systemic flow ratio in those patients with no collateral vessels was similar to the previously reported echocardiographically derived superior vena cava/systemic flow ratio in normal children. CONCLUSIONS: The pulmonary/systemic flow ratio after bidirectional cavopulmonary anastomosis can be calculated. Pulmonary blood flow in these patients determines systemic saturation and accounts for the majority of venous return in young children.

QRS prolongation is associated with inducible ventricular tachycardia after repair of tetralogy of Fallot.

Prolonged QRS duration on the electrocardiogram has been found to predict adverse arrhythmic events in patients late after repair of tetralogy of Fallot. Whether QRS duration can also predict inducible ventricular tachycardia (VT) at electrophysiologic study is unknown. Between 1984 and 1995 we studied 135 survivors of tetralogy of Fallot surgery whose age at surgery was 34 days to 37 years (3.7 +/- 3.9, median 2.5) and age at electrophysiologic study was 1.4 to 43 years (9.7 +/- 8.2, median 6.7). QRS duration was 80 to 240 ms (137 +/- 29) and > or = 180 ms in 9 patients. Sustained VT was induced in 22 patients (monomorphic in 17). Induced sustained monomorphic VT was related to QRS duration, right ventricular dimension, H-V interval, and presence of symptoms. QRS duration was also related to induced sustained monomorphic VT by multivariate analysis. QRS duration > or = 180 ms was 35% sensitive and 97% specific for induced sustained monomorphic VT. QRS duration was related to induced sustained monomorphic VT even when only asymptomatic patients were analyzed. A QRS duration > or = 180 ms was 100% sensitive and 96% specific for detecting clinical VT. Prolonged QRS duration on the electrocardiogram is associated with induced sustained monomorphic VT on electrophysiologic study. The finding of prolonged QRS duration should suggest the need for further testing to determine the risk of adverse arrhythmic events in patients after repair of tetralogy of Fallot, even if they are asymptomatic.

Management strategies for surgical treatment of dysrhythmias in infants and children.

To elucidate the role of surgery in the management of refractory pediatric dysrhythmias, the investigators' 1987 experience with this therapy was reviewed. Sixteen patients (8 male, 8 female) had surgery. Age ranged from 3 months to 21 years (mean 7.7 years) with 2 being younger than 1 year of age. Eight patients had Wolff-Parkinson-White syndrome with supraventricular tachycardia (SVT), 6 had SVT alone and 2 had ventricular tachycardia. Patient symptoms fell into 3 distinct categories. Nine patients had frequent non-life-threatening tachycardia episodes despite aggressive medical management, 4 patients had life-threatening symptoms either related to frequent tachycardia episodes or due to side effects of drugs and 3 patients had minimal tachycardia symptoms but were judged to be at risk for sudden death. The mechanisms of the tachycardias included reentrant SVT using an accessory atrioventricular connection in 9, the permanent form of junctional reciprocating tachycardia in 2, atrioventricular node reentrant SVT in 2, atrial ectopic focus in 1 and ventricular ectopic foci in 2. Ablation techniques involved either surgical dissection or cryoablation of the arrhythmogenic substrates. There was no surgical mortality and follow-up shows 15 of 16 patients to have had no recurrence of symptomatic tachycardia without any therapy. Based on this experience, management strategies for surgical control of recalcitrant pediatric dysrhythmias are proposed. Surgery for infant dysrhythmias should be performed only for life-threatening symptoms. Beyond the first year of life, after failing 2 medications, surgical options for SVT should be considered. For patients with ventricular tachycardia, aggressive medical management with class II, IC and III agents should be attempted before surgical therapy.

Catheter ablation treatment of supraventricular tachycardia-induced cardiomyopathy.

OBJECTIVE: To investigate the efficacy of radiofrequency catheter ablation (RFCA) as an alternative nonpharmacological therapy for tachycardia-induced cardiomyopathy. DESIGN: A retrospective study of 8 pediatric patients (age range, 10 months to 21 years) who underwent RFCA for an incessant supraventricular tachycardia-induced cardiomyopathy. A patient's tachycardia was considered incessant if the tachycardia was present more than 75% of the time. The left ventricular shortening fraction, as measured by echocardiography, before and after ablation, was used as the index of cardiac function. Cardiomyopathy was defined as a left ventricular shortening fraction of 28% or less. RESULTS: Following RFCA, 7 patients had total resolution of their tachycardia and were discharged from the hospital with no antiarrhythmic medications. The remaining patient's tachycardia was modified by the catheter ablation and was subsequently controlled with flecainide acetate. With follow-up ranging from 9 months to 3 years, all patients have normal cardiac function as documented by echocardiography. No significant morbidity resulted from the catheter ablations. CONCLUSIONS: Tachycardia-induced cardiomyopathy is amenable to "curative" therapy with RFCA. Ventricular function returns to normal after the successful catheter ablation procedure.

The developmental electrophysiologic effects of moricizine HCl on the canine cardiac Purkinje fiber.

Using standard microelectrode techniques, the developmental cellular electrophysiologic effects of moricizine HCl on adult and neonatal canine Purkinje fibers were studied. Steady state and rate-related changes in the transmembrane action potentials produced by moricizine HCl in both age groups were characterized and compared. Also, the rate of barium-induced abnormal automaticity before and after drug was also investigated in neonatal and adult Purkinje fibers. The major findings of this study are as follows. (1) The steady state and rate-related depressant effects of moricizine HCl on Vmax were similar in both age groups. (2) Moricizine HCl shortened APD90 in the adult fibers to a greater extent than in the neonate. (3) The concentration of moricizine HCl required to significantly reduce the rate of abnormal automaticity was less in the neonate than in the adult. The effect of moricizine HCl on APD90 of individual Purkinje fibers is influenced both by their control APD90 value as well as by maturational factors. It is less clear whether developmental differences in the effects of moricizine HCl on abnormal automaticity are solely a result of differences in control rates of abnormal automaticity between the two age groups.

Pediatric cardiac pacing.

Cardiac pacing in children has undergone many improvements in the last decade. The differences between adult and pediatric pacing have narrowed. Children are no longer being denied pacemakers because of size. This article discusses techniques for pacing pediatric patients successfully that will allow them to lead normal lives.

Diagnosis and treatment of pediatric arrhythmias.

The work up of arrhythmias encountered in the pediatric patient is usually initiated by primary care providers. Proper treatment of pediatric arrhythmias necessitates recording the suspected rhythm disturbance and an evolution of the underlying cardiac structure and function.

Surgical treatment of dysrhythmias in infants and children.

The recognition of medically refractory dysrhythmias in children has necessitated the use of more invasive nonpharmacologic therapies. The role of ablative surgery in the management of pediatric rhythm disturbances is presented.

New antiarrhythmic agents in pediatrics.

This article reviews recent developments in the pharmacologic management of arrhythmias in children and provides specific information about six newer antiarrhythmic agents. With the current increase in recognition, frequency, and complexity of rhythm disturbances in children, pediatricians can expect to encounter children on these antiarrhythmic medications in their practices with increasing frequency.

High-dose narcotic anesthesia using sufentanil in swine for cardiac catheterization and electrophysiologic studies.

A protocol for high-dose narcotic anesthesia using sufentanil by intravenous infusion was developed in swine undergoing cardiac catheterization and intracardiac electrophysiologic studies. In experiments involving cardiac conduction system ablation, nine swine received intramuscular ketamine, 33 mg/kg, and intramuscular acepromazine, 1.1 mg/kg, as premedication. An infusion of sufentanil (15 mcg/kg/h) was started followed by a loading dose of sufentanil (7 mcg/kg) given as a bolus intravenously. A continuous infusion of sufentanil (15-30 mcg/kg/h) was used for maintenance anesthesia. Since the development of this protocol, we have been able to achieve stable hemodynamics and cardiac rhythms for intracardiac electrophysiologic studies.

High prevalence of repolarization abnormalities in children with simple ventricular ectopy.

BACKGROUND: Premature ventricular contractions (PVCs) may occur in 0.3 to 2.2% of routine resting electrocardiograms (ECGs) in children with structurally normal hearts. HYPOTHESIS: This study tests the hypothesis that repolarization abnormalities are present more frequently on the surface ECG in pediatric patients with benign ventricular ectopy. METHODS: A retrospective study was performed examining 32 children with benign ventricular ectopy and structurally normal hearts. The surface ECG was carefully reviewed, concentrating on repolarization abnormalities as evidenced by a prolonged QTc, a prolonged JTc, and/or an abnormal T-wave vector. QTc was calculated using Bazett's formula and considered prolonged if > 440 ms, and the JTc was calculated using a formula analogous to Bazett's formula and considered prolonged if > 340 ms. The T-wave vector was considered normal if between 0 to +90 degrees, concordant with the frontal QRS axis and within 60 degrees of the QRS axis. There were 13 females (41%) and 19 males (59%) with an average age of 9.1 years (range 1-16.1 years). These patients were then compared with age- and gender-matched controls. Fourteen of 32 study patients (44%) and 2 of 32 controls (6%) had evidence of repolarization abnormalities. RESULTS: Using chi 2 analysis, there was a significant difference between groups (p = 0.0005). No patient had an abnormal T-wave vector and there were no other significant ECG abnormalities. There was no significant relationship between repolarization abnormalities and patient gender or age. CONCLUSION: A significant percentage (44%; P = 0.0005) of children with benign ventricular ectopy have associated repolarization abnormalities as evidenced by a prolonged QTc and/or JTc. This finding suggests that children with "benign PVCs" may have repolarization changes indicative of underlying substrate abnormalities.

Adenosine's effectiveness in long RP' re-entrant tachycardia: additional evidence of the decremental qualities of the retrograde limb.

Adenosine's ability to terminate atrioventricular (AV) re-entrant supraventricular tachycardia is well documented. Typically, termination occurs as a consequence of transient conduction block in the atrioventricular node, a tissue with decremental qualities. However, the atrioventricular node is not always the site of action when adenosine is used on the re-entrant types of long RP' tachycardias. These tachycardias are, in part, characterized by the decremental qualities of the retrograde limb of the tachycardia circuit, which, in turn, are typically exemplified by retrograde Wenckebach during ventricular (VVI) pacing during intracardiac electrophysiology studies. This case report involves adenosine's ability to block conduction in the retrograde limb of the permanent form of junctional reciprocating tachycardia to provide further evidence as to the AV "nodelike" decremental qualities of this limb.

Successful radiofrequency catheter ablation of atrial muscle reentry tachycardia in a young adult.

Atrial muscle reentry as a mechanism of tachycardia has been well illustrated in isolated animal atrial muscle. It has infrequently been reported as an etiology of supraventricular tachycardia in young patients. A case of atrial muscle reentry tachycardia and its successful elimination using radiofrequency catheter ablation is reported.

Continuity of care. Development and implementation of a shared patient data base.

Although the inpatient Oncology Unit, the Medical Oncology Clinic, and Radiation Oncology provided care for many of the same patients, there was no mechanism for sharing nursing information, and little colleague input from one area to another. In order to meet this need, a nurse from each of the clinic areas was added to the inpatient unit's Patient Care Evaluation Committee. Working through this committee, these nurses developed an Inpatient/Outpatient Data Flow Sheet, which could be initiated in any oncology area to implement information flow when a patient was to be seen in a different setting. It proved to be an effective tool. The flow sheet, along with our rationale, was then presented for consideration as a computerized program to be used between the three areas. After careful investigation, it was approved. This provided the oncology areas with the first data storage capability for nursing in the hospital. It offered oncology nurses in distinct and separate areas access to obtain and update information on shared patients. This manuscript will focus on the computer program and the data base designed for the oncology department and its impact on nurses and patients.

Sonographic assessment of fetal cardiac arrhythmias.

The fetus with an arrhythmia may be at high risk both from the arrhythmia itself and from the condition that provoked it. Incorrect diagnosis and inappropriate or delayed treatment may further compound the hazard. Although echocardiography can specifically identify the arrhythmia, this technique requires very skilled and careful examination by a physician who fully understands how to differentiate optimally between similar-appearing but mechanistically different arrhythmias. Congenital heart disease may also be present and must be evaluated specifically. This report describes ultrasound recording techniques used to diagnose arrhythmias and includes a discussion of M-mode echocardiography, two-dimensional imaging, pulsed Doppler, and color-flow Doppler. It also differentiates specific arrhythmias--premature atrial contractions, ventricular tachycardia, atrial flutter, complete heart block, and supraventricular tachycardia--and presents the optimal diagnostic tools for each.

Effect of feeding organic and inorganic sources of additional zinc on growth performance and zinc balance in nursery pigs.

Three experiments were conducted to evaluate the effect of feeding pharmacological concentrations of zinc (Zn), from organic and inorganic sources, on growth performance, plasma and tissue Zn accumulation, and Zn excretion of nursery pigs. Blood from all pigs was collected for plasma Zn determination on d 14 in Exp. 1, d 7 and 28 in Exp. 2, and d 15 in Exp. 3. In Exp. 1, 2, and 3, 90, 100, and 15 crossbred (GenetiPorc USA, LLC, Morris, MN) pigs were weaned at 24+/-0.5, 18, and 17 d of age (6.45, 5.47, and 5.3 kg avg initial BW), respectively, and allotted to dietary treatment based on initial weight, sex, and litter. A Phase 1 nursery diet was fed as crumbles from d 0 to 14 in Exp. 1, 2, and 3, and a Phase 2 nursery diet was fed as pellets from d 15 to 28 in Exp. 1 and 2. The Phase 1 and Phase 2 basal diets were supplemented with 100 ppm Zn as ZnSO4. Both dietary phases contained the same five dietary treatments: 150 ppm additional Zn as zinc oxide (ZnO), 500 ppm added Zn as ZnO, 500 ppm added Zn as a Zn-amino acid complex (Availa-Zn 100), 500 ppm added Zn as a Zn-polysaccharide complex (SQM-Zn), and 3,000 ppm added Zn as ZnO. Overall in Exp. 1, pigs fed 500 ppm added Zn as SQM-Zn or 3,000 ppm added Zn as ZnO had greater ADG (P < 0.05) than pigs fed 150 ppm, 500 ppm added Zn as ZnO, or 500 ppm added Zn as Availa-Zn 100 (0.44 and 0.46 kg/d vs 0.35, 0.38, and 0.33 kg/d respectively). Overall in Exp. 2, pigs fed 3,000 ppm added Zn as ZnO had greater (P < 0.05) ADG and ADFI than pigs fed any other dietary treatment. On d 14 of Exp. 1 and d 28 of Exp. 2, pigs fed 3,000 ppm added Zn as ZnO had higher (P < 0.05) plasma Zn concentrations than pigs on any other treatment. In Exp. 3, fecal, urinary, and liver Zn concentrations were greatest (P < 0.05) in pigs fed 3,000 ppm added Zn as ZnO. On d 10 to 15 of Exp. 3, pigs fed 3,000 ppm added Zn as ZnO had the most negative Zn balance (P < 0.05) compared with pigs fed the other four dietary Zn treatments. In conclusion, feeding 3,000 ppm added Zn as ZnO improves nursery pig performance; however, under certain nursery conditions the use of 500 ppm added Zn as SQM-Zn may also enhance performance. The major factor affecting nutrient excretion appears to be dietary concentration, independent of source.

Evaluation and treatment of pediatric patients with congenital or acquired long QT interval syndromes.

The long QT syndrome should be considered when evaluating patients with syncope. Prolongation of the QT interval and abnormalities of T wave morphology due to abnormal ventricular repolarization characterize the syndrome. In the past decade, molecular genetics has revealed that abnormal repolarization is the result of gene mutations encoding integral ion channels that generate the cardiac action potential. Eight subgroups of long QT syndrome associated with five genes have been described to date. The explosion in research in this area has led to a greater understanding of the clinical expression of autosomal dominant (Romano-Ward syndrome), autosomal recessive (Jervell and Lange-Nielsen syndrome) and acquired forms of the disease. This has also led to investigation in the area of genotype-specific therapy. The purpose of this review is to outline the strides made in the field of molecular genetics and update the reader on the recent advances in diagnosis and treatment of the long QT syndrome.