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Biomedical subjects

B J Deal

Publications and source records attributed to B J Deal.

At least 19 recordsLinked to original sources

Magnetocardiographic rhythm patterns at initiation and termination of fetal supraventricular tachycardia.

BACKGROUND: Using fetal magnetocardiography (fMCG), we characterize for the first time the electrophysiological patterns of initiation and termination of reentrant fetal supraventricular tachycardia (SVT), the most common form of life-threatening fetal arrhythmia. METHODS AND RESULTS: In contrast to the expectation that reentrant SVT is initiated by spontaneous premature atrial contractions (PACs) and is terminated by spontaneous block, 5 distinct patterns of initiation and 4 patterns of termination were documented, with the most common patterns of initiation involving reentrant PACs. Waveform morphology and timing, including QRS and ventriculoatrial interval, were assessed. This enabled detection of such phenomena as Wolff-Parkinson-White syndrome, QRS aberrancy, and multiple reentrant pathways that were crucial for defining the rhythm patterns. In addition, fMCG actocardiography revealed an unexpectedly strong association between fetal trunk movement and the initiation and termination of SVT, suggesting that autonomic influences play a key role. CONCLUSIONS: This study demonstrates that the patterns of initiation and termination of fetal SVT are more diverse than is generally believed and that the most common patterns of initiation involve reentrant PACs. The ability to discern such patterns can help elucidate the underlying mechanisms and guide antiarrhythmic drug therapy. fMCG provides a noninvasive means of analyzing complex tachyarrhythmia in utero, with efficacy approaching that of postnatal electrocardiographic rhythm monitoring.

Cardiac Pacing, Artificial↗

Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation.

OBJECTIVES: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We review our experience with conversion to total cavopulmonary artery connections and arrhythmia surgery. METHODS: Between 1994 and 2001, 40 patients underwent Fontan conversion and arrhythmia surgery. Significant hemodynamic lesions such as aortic aneurysm (n = 1), atrioventricular valve insufficiency (n = 8), and pulmonary arterioplasty (n = 9) were repaired concomitantly. Thirty-four patients were in New York Heart Association class III or IV. Mean age at the original Fontan operation was 7.5 +/- 6.5 years and mean age at Fontan conversion was 18.7 +/- 9.0 years. Arrhythmia surgery has evolved from isthmus cryoablation in 10 patients to right-sided maze in 16 patients for atrial reentry tachycardia. The maze-Cox III operation was used for 14 patients with atrial fibrillation. Atrial (n = 33) and dual-chamber (n = 5) pacemakers were placed. RESULTS: There has been no early mortality. Chest tubes were removed on postoperative day 9.0 +/- 6.0. Hospital stay was 11.8 +/- 6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was 1 death 2 years postoperatively from acute myocardial infarction. For the entire series, arrhythmia recurrence is 12.5%, with only 10% of patients receiving long-term antiarrhythmic medications; these patients were among the first 8 patients in our series. Most patients are in New York Heart Association class I or II. Bruce protocol in 12 patients showed increased tolerance (P <.05). CONCLUSIONS: Fontan conversion to total cavopulmonary connection with concomitant arrhythmia surgery is excellent therapy for patients whose Fontan repair has failed. Fontan conversion is safe, improves New York Heart Association class, improves exercise tolerance, and has a low incidence of recurrent arrhythmias.

Adolescent↗

Simultaneous transcatheter closure of an atrial septal defect with an amplatzer septal occluder and radiofrequency ablation of an accessory connection.

This report describes the first case of simultaneous transcatheter closure of an atrial septal defect and radiofrequency ablation of an accessory connection. This was performed successfully on an 8-year-old boy and represents an attractive therapeutic alternative to surgical repair in this combination of relatively common cardiac conditions.

Catheter Ablation↗

Congenital Heart Surgery Nomenclature and Database Project: arrhythmias.

This discussion of arrhythmia terminology attempts to classify rhythm disorders for which surgical therapy may be necessary. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery, for the purpose of establishing a unified reporting system. Efforts were made to include all relevant nomenclature categories, using synonyms where appropriate. Extant surgical ablative procedures, detailed methods of pacemaker insertion, and AICD technology are discussed. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and will lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Arrhythmias, Cardiac↗

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.

Adolescent↗

Bidirectional Glenn shunt in association with congenital heart repairs: the 1(1/2) ventricular repair.

BACKGROUND: The bidirectional Glenn shunt has been used to incorporate a smaller tripartite ventricle into the circulation and create pulsatile pulmonary artery flow. We reviewed our operative experience and assessed hemodynamics of the bidirectional Glenn shunt in 1(1/2) ventricular repair or in conjunction with other repairs of congenital heart defects. METHODS: Between 1992 and 1998, 15 patients (mean age, 8.1+/-7.9 years) had bidirectional Glenn shunt in association with repair of congenital heart defects. Eighty-seven percent had at least one previous operation. All patients had simultaneous or previous intracardiac repair and had bidirectional Glenn shunt to volume unload the small right ventricle (group A, n = 7), to unload the poorly functioning right ventricle (group B, n = 2), to redirect superior vena cava-pulmonary venous atrial connection to treat cyanosis (group C, n = 2), or to unload the pulmonary left ventricle for residual intracavitary hypertension in patients with L-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (group D, n = 4). Intraoperative hemodynamic assessment was done in 2 patients in group A by selective use of inflow occlusion and flow probes. RESULTS: All patients survived. Four patients had successful, concurrent arrhythmia circuit cryoablation for Wolf-Parkinson-White syndrome (n = 1) or atrial reentry tachycardia (n = 3). Superior and inferior vena caval flow averaged 36% and 64% of cardiac output, respectively. Postoperative superior vena caval pressure (n = 13) was 13.7+/-4.0 mm Hg with pulmonary arterial flow pattern contributed by the ventricle in systole (pulsatile) and the superior vena cava in diastole (laminar). CONCLUSIONS: The bidirectional Glenn shunt is an effective adjunct to congenital heart repair to treat pulmonary ventricular pressure-volume problems and anomalous superior vena caval to left atrial connections.

Adolescent↗

Transmural atrial pacing in patients with postoperative congenital heart disease.

INTRODUCTION: Some patients with postoperative congenital heart disease require permanent cardiac pacing, but the use of transvenous or epicardial pacing leads may be limited by type of cardiac malformation, venous connections, body size, or fibrosis. Transmural atrial pacing may provide an alternative in difficult patients, but to date has been described in only a few articles with small patient numbers, and data from lead performance are lacking. METHODS AND RESULTS: Records were reviewed in 18 consecutive patients (4 months to 21 years old) with postoperative congenital heart disease receiving transmural atrial pacing leads from July 1994 to December 1996. Implantation materials and techniques were described. Lead sensing and capture thresholds obtained acutely and during short-term follow-up (mean: 11.0 months) were evaluated, and comparisons were made between patients with postoperative Fontan anatomy and non-Fontan patients, and between patients receiving steroid-eluting and nonsteroid leads. Overall, the median acute sensing and capture thresholds of transmural leads were 4.1 m V and 0.7 V at 0.5 msec, respectively. Median follow-up thresholds were 2.8 m V and 0.8 V, respectively. Performance of leads in Fontan patients was similar to those in non-Fontan patients. Steroid-eluting leads had a chronic capture threshold of 0.6 V versus 0.9 V for nonsteroid leads (P = 0.038). CONCLUSION: Transmural atrial pacing leads were successfully implanted in patients with diverse ages and types of postoperative congenital heart disease. Lead performance was acceptable both acutely and during the first year of follow-up.

Adolescent↗

Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation.

OBJECTIVES: We review our surgical experience patients with atriopulmonary Fontan operations who had obstructive or arrhythmia indications for conversion to total cavopulmonary artery connections, arrhythmia circuit cryoablation, and placement of atrial antitachycardia pacemaker. METHODS: Fourteen patients (mean age 14 +/- 4 years) had conversion to total cavopulmonary artery connection 8 +/- 3 years after the original atriopulmonary Fontan operation primarily for atrial arrhythmias in 11, obstructive lesions in 2, and bradycardia with cyanosis in 1. Arrhythmia circuit cryoablation was performed on 11 patients and 10 had atrial antitachycardia pacemakers. Preoperative functional New York Heart Association class was IV in 9, III in 4, and II in 1. RESULTS: One patient had brain death (7%) presumably caused by resternotomy complications despite an excellent hemodynamic result. Another required reoperation for a maldeployed clamshell device after attempted fenestration closure. Average length of stay was 10 +/- 3 days; chest tubes were removed on day 7 +/- 3. There were no long-term deaths (mean follow-up 1.7 years, range 5 months to 5 years). Postoperative arrhythmias occurred in five patients, three of whom had successful termination by antitachycardia pacemaker and two who had pharmacologic control of their respective junctional ectopic and slow atrial tachycardia. All patients have improved to New York Heart Association class I or II. CONCLUSIONS: Total cavopulmonary artery conversion in association with arrhythmia circuit cryoablation and atrial antitachycardia pacemaker placement can be accomplished with low morbidity and mortality, oftentimes resulting in dramatic increases in functional class and control of life-threatening arrhythmias.

Adolescent↗

The medical therapy of cardioinhibitory syncope in pediatric patients.

A small percentage of pediatric patients with neurally mediated syncope will have an asystolic response during upright tilt table testing. The purpose of this study is to evaluate the incidence of asystole during tilt table testing, and to assess the outcome of medical management of such patients. Of 398 patients undergoing evaluation for recurrent syncope between January 1989 and 1994, 18 (4.5%) experienced asystole lasting > or = 5 seconds during baseline tilt test. Patients had experienced a mean of four episodes of syncope, with a mean age at the time of tilt test of 11.1 +/- 4.0 years. The median duration of asystole was 10 seconds (range 5-40 s). Treatment was individualized to increased fluids and salt intake (3 patients), metoprolol (8 patients), pseudoephedrine (4 patients), disopyramide (1 patient), or combination therapy with fludrohydrocortisone (2 patients). During a median duration of follow-up of 31 months, no additional syncope was experienced by 78% of patients. Recurrent syncope in 4 patients was associated with either noncompliance or discontinuation of therapy in 3 patients; in 1 patient, increasing the dose of metoprolol was effective in preventing recurrences. We conclude that young patients with recurrent syncope and asystole during tilt test may be safely and effectively managed with pharmacological therapy, without resorting to pacemaker implantation.

Adrenergic Agonists↗

Fetal tachycardia: mechanisms and predictors of hydrops fetalis.

OBJECTIVES: This study had three objectives: 1) to determine the electrophysiologic mechanisms of fetal supraventricular tachycardia at presentation and postnatally; 2) to identify the clinical and electrophysiologic predictors of hydrops fetalis; and 3) to describe the medium-term follow-up (1 to 7 years) of patients with fetal supraventricular tachycardia. BACKGROUND: Fetal supraventricular tachycardia causes significant fetal and neonatal morbidity and mortality. Prenatal analysis and postnatal confirmation of fetal supraventricular tachycardia mechanisms have been limited. METHODS: Supraventricular tachycardia mechanisms were evaluated by prenatal Doppler/M-mode echocardiography, immediate neonatal surface electrocardiography and postnatal transesophageal electrophysiologic procedures in 30 consecutive patients presenting with fetal supraventricular tachycardia (17 managed prenatally, 13 first managed postnatally). RESULTS: The fetal supraventricular tachycardia mechanism was 1:1 atrioventricular conduction in 22 patients and supraventricular tachycardia with atrioventricular block (atrial flutter) in 8. At the postnatal transesophageal electrophysiologic procedure, tachycardia was induced in 27 of 30 patients; atrioventricular reentrant tachycardia in 25 (93%) of 27 and intraatrial reentrant tachycardia in only 2 (7%) of 27. Hydrops was present in 12 of 30 fetuses. Sustained supraventricular tachycardia (> 12 h) and lower gestation at presentation correlated with hydrops (p < 0.02, p < 0.05), but mechanism of tachycardia and heart rate did not. Gestational age at delivery was significantly greater in those who received intrauterine management (39 +/- 1.3 vs. 37 +/- 2.9 weeks, p = 0.04) despite earlier presentation (32.6 vs. 37.1 weeks). Cesarean section deliveries were reduced in the same group (3 of 17 vs. 11 of 13, p = 0.0006). CONCLUSIONS: Atrioventricular reentrant tachycardia was the predominant mechanism of supraventricular tachycardia in the fetus. There was a high association of supraventricular tachycardia with atrioventricular block in utero and accessory atrioventricular connections. Outcome at 1 to 7 years was excellent regardless of severity of illness at clinical presentation.

Echocardiography, Doppler↗

Comparison of transesophageal and intracardiac electrophysiologic studies in characterization of supraventricular tachycardia in pediatric patients.

OBJECTIVES: This study sought to determine the accuracy of transesophageal electrophysiologic studies in diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients. BACKGROUND: Transesophageal electrophysiologic studies are a relatively noninvasive means of characterizing supraventricular tachycardia. Although widely used, to our knowledge no data exist that directly compare information obtained from transesophageal electrophysiologic studies with that from intracardiac electrophysiologic studies. METHODS: We reviewed the records of 57 pediatric patients undergoing both transesophageal and intracardiac electrophysiologic studies at our institution. The results of these studies were compared with respect to mechanism of tachycardia, localization of accessory atrioventricular (AV) connections (if present) and characterization of anterograde accessory connection conduction properties. RESULTS: Tachycardia mechanisms were concordant in 56 of 57 patients: orthodromic reciprocating tachycardia in 43, antidromic reciprocating tachycardia in 1, both orthodromic and antidromic tachycardia in 2, AV node reentrant tachycardia in 5, atrial reentrant tachycardia in 4 and ectopic atrial tachycardia in 2. Of 29 patients with orthodromic reciprocating tachycardia using a concealed accessory connection, transesophageal study predicted the accessory connection site through changes induced by transient bundle branch block in 12. By the Bland-Altman method in 14 patients with pre-excitation, the anterograde accessory connection effective refractory period determined by transesophageal study compared favorably with that determined by intracardiac study (mean difference 5.0 ms, limits of agreement -55 and 65 ms). CONCLUSIONS: Transesophageal electrophysiologic studies are a highly accurate means of diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients.

Adolescent↗

Radiofrequency catheter ablation for tachyarrhythmias in children and adolescents. The Pediatric Electrophysiology Society.

BACKGROUND: Although radiofrequency catheter ablation has been used extensively to treat refractory supraventricular tachycardia in adults, few data are available on its safety and efficacy in children and adolescents. We reviewed registry data obtained from 24 centers to evaluate the indications, early results, complications, and short-term follow-up data in young patients who underwent this procedure. METHODS: Standardized data were submitted for 652 patients who underwent 725 procedures between January 1, 1991, and September 1, 1992. The mean length of follow-up was 13.5 months. RESULTS: The median age of the patients was 13.5 years, and 84 percent of them had structurally normal hearts. The initial success rates for ablation of atrioventricular accessory pathways (508 of 615 procedures) and atrioventricular-node reentry (63 of 76 procedures) were both 83 percent. Greater institutional experience in performing ablation in children and location of the accessory pathway in the left free wall correlated with greater likelihood of sustained success. Conversely, a right free-wall pathway, the presence of other heart disease, and higher body weight were all associated with a lesser chance of sustained success. Recurrences of arrhythmia accounted for 45 percent of the failures overall in the series. Atrial ectopic-focus tachycardia had the highest recurrence rate. The total complication rate was 4.8 percent (35 of 725 procedures), and the only variables that independently correlated with a higher complication rate were very low weight and less institutional experience. CONCLUSIONS: These early results suggest that radio-frequency catheter ablation has a good success rate and a low complication rate in pediatric patients, especially when it is carried out in experienced pediatric cardiology centers.

Adolescent↗

Usefulness of metoprolol for unexplained syncope and positive response to tilt testing in young persons.

The efficacy of intravenous metoprolol in preventing symptoms during a repeat tilt test was compared with the outcome of chronic oral treatment in 21 patients (14 female, 7 male), age 8 to 20 years (mean 13 +/- 3) with unexplained syncope (> or = 1 episode) and a positive response to tilt testing. A positive response was defined as the development of either syncope or presyncope. During the initial tilt test, a positive response occurred during baseline (14 patients) or isoproterenol (0.03 to 0.1 microgram/kg/min) infusion (7 patients) with a cardioinhibitory (1 patient), vasodepressor (5 patients) or mixed (15 patients) pattern. Metoprolol (0.1 to 0.2 mg/kg) was administered intravenously. During the repeat tilt test, response was negative in 18 patients, including 11 of 14 patients with a positive response in the baseline and 7 of 7 patients with a positive response during isoproterenol infusion. Metoprolol (0.8 to 2.8 mg/kg/day) was administered orally to 15 patients for an average of 10 months. Symptoms were absent (7 patients) or improved (2 patients); metoprolol was discontinued because of adverse effects (3 patients) or recurrence of symptoms (3 patients). In 7 of 12 patients with a negative response and 2 of 3 patients with a positive response after intravenous metoprolol, oral administration of metoprolol prevented or improved symptoms without adverse effects. Many young patients (60%) with recurrent syncope obtained symptomatic improvement from chronic oral metoprolol treatment without adverse effects; repeat tilt testing after intravenous metoprolol did not appear to offer any additional information than would have been obtained from a trial of chronic oral treatment.

Administration, Oral↗

Successful radiofrequency energy ablation of automatic junctional tachycardia preserving normal atrioventricular nodal conduction.

Automatic junctional tachycardia is frequently refractory to medical management and difficult to treat with nonpharmacological methods. A 12-year-old female with symptomatic, refractory automatic junctional tachycardia is reported. Earliest atrial activation during supraventricular tachycardia was in the posterior portion of the intraatrial septum. The patient underwent electrophysiological study and successful radiofrequency current ablation of the ectopic automatic focus within the atrioventricular junction. Normal atrioventricular junctional conduction was maintained, and at 7-month follow-up the patient has been free of tachycardia.

Atrioventricular Node↗