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Papers by Barbara Deal

World journal for pediatric & congenital heart surgery, 2015

Long-term growth outcomes and the prevalence of obesity among older single ventricle (SV) patient... more Long-term growth outcomes and the prevalence of obesity among older single ventricle (SV) patients have not been well characterized. We investigated these parameters, as well as the impact of obesity on survival, in an older cohort of SV patients presenting for Fontan conversion. We analyzed preoperative height, weight, and body mass index (BMI) of patients who underwent Fontan conversion. Overweight and obese were defined as BMI ≥85 percentile and ≥95 percentile for patients <20 years and BMI 25 to 30 kg/m(2) and ≥30 kg/m(2) for patients ≥20 years, respectively. Postoperative transplant-free survival was assessed among obese, overweight, and normal weight patients. We evaluated 139 patients presenting for Fontan conversion at a median age of 23.2 years. Patients had shorter stature compared to the normal population (mean Z score -0.6, P < .001). Younger patients had lower BMI compared to the normal population (<20 years: mean Z score -0.5, P = .02), while older patients ha...

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, Jan 5, 2015

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 1999

Patients who have undergone atriopulmonary Fontan procedures are at risk for significant atrial a... more Patients who have undergone atriopulmonary Fontan procedures are at risk for significant atrial arrhythmias and obstructive connections, which have been related to intra-atrial suture lines, atrial enlargement, and somatic growth. Twenty patients (mean age, 17.3 +/- 6.8 years) had conversion to total cavopulmonary artery connection 8.9 +/- 2.1 years after the previous Fontan procedure (for atrial arrhythmias in 19 patients and for obstructive lesions in one). Arrhythmia ablative surgery evolved over the study period from "arrhythmia circuit cryoablation" (cryoablation lesions completing lines of block) to the more standard approaches of modified right-sided Maze and Maze-Cox III procedures. Preoperative functional New York Heart Association class was IV in nine patients, III in nine, and II in two. All patients survived. Two patients had prolonged chest drainage (there was pericardial effusion in one). The average length of hospital stay was 11.3 +/- 5.4 days; chest tubes ...

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 2011

This article was prepared to summarize the points made in a debate that the first author (C.L.B.)... more This article was prepared to summarize the points made in a debate that the first author (C.L.B.) had with Dr. Richard Jonas at the American Association for Thoracic Surgery 90th Annual Meeting. The topic of the debate was the optimal surgical approach for functional single-ventricle patients: extracardiac versus intra-atrial lateral tunnel Fontan. My role was to take the viewpoint that the extracardiac Fontan is better. This review summarizes our results at Children's Memorial Hospital (Chicago, IL) with 180 patients undergoing a primary Fontan procedure and 126 patients undergoing an extracardiac Fontan as part of a Fontan conversion. The world literature was reviewed on outcomes following the Fontan procedure, focusing on six main areas supporting the superiority of the extracardiac Fontan: hemodynamics, arrhythmias, applicability to complex anatomy, use of cardiopulmonary bypass, complications of fenestration and thromboembolism, and operative mortality. Based on this review...

The Annals of Thoracic Surgery, 2015

Specific congenital heart anomalies significantly increase the risk for late atrial arrhythmias, ... more Specific congenital heart anomalies significantly increase the risk for late atrial arrhythmias, raising the question whether prophylactic arrhythmia operations should be incorporated into reparative open heart procedures. Currently no consensus exists regarding standard prophylactic arrhythmia procedures. Questions remain concerning the arrhythmia-specific lesions to perform, energy sources to use, need for atrial appendectomy, and choosing a right, left, or biatrial Maze procedure. These considerations are important because prophylactic arrhythmia procedures are performed without knowing if the patient will actually experience an arrhythmia. This review identifies congenital defects with a risk for the development of atrial arrhythmias and proposes standardizing lesion sets for prophylactic arrhythmia operations.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 2007

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by... more The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them. The cases reviewed herein include: takedown of a Bjork-Fontan modification, right ventricular hypertension and tricuspid regurgitation after ...

Pacing and Clinical Electrophysiology, 2002

Pacing and Clinical Electrophysiology, 1993

Automatic junctional tachycardia is frequently refractory to medical management and difficult to ... more 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.

Pacing and Clinical Electrophysiology, 1997

and Children's Mercy Hospital, Kansas City, Missouri DEAL, B.J., ET AL.: The Medical Therapy of C... more and Children's Mercy Hospital, Kansas City, Missouri DEAL, B.J., ET AL.: 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 s 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

New England Journal of Medicine, 1994

Although radiofrequency catheter ablation has been used extensively to treat refractory supravent... more 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. 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. 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. 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.

The Journal of Thoracic and Cardiovascular Surgery, 2009

Objectives: We assessed our pacemaker strategy, use of antitachycardia therapies, generator longe... more Objectives: We assessed our pacemaker strategy, use of antitachycardia therapies, generator longevity, and need for programming changes in patients having Fontan conversion with arrhythmia surgery.

The Journal of Thoracic and Cardiovascular Surgery, 1998

Objectives: We review our surgical experience patients with atriopulmonary Fontan operations who ... more 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).

The Journal of Thoracic and Cardiovascular Surgery, 2001

TX ET CSP ACD CHD GTS EDITORIAL Mavroudis et al Surgery for Congenital Heart Disease

Journal of the American College of Cardiology, 1996

Journal of the American College of Cardiology, 1995

Objectives. This study sought to determine the accuracy of transesophageal electrophysiologic stu... more Objectives. This study sought to determine the accuracy of transesophageal electrophysiologic studies in diagnosing and characterizing various mechanisms of supraventricular tachycardia in pediatric patients.

Journal of the American College of Cardiology, 1985

The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricula... more The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricular tachycardia in the first 4 months of life were reviewed. Among these, 63% were male. Structural heart disease was present in 20%, most commonly Ebstein's anomaly. All patients presented with a regular narrow QRS tachycardia, and pre-excitation became evident only when normal sinus rhythm was established. Only one infant had atrial flutter and none had atrial fibrillation. Type A Wolff-Parkinson-White syndrome was most common (49%), with heart disease occurring in only 5% of these patients. In contrast, heart disease was identified in 45% of those with type B syndrome. Initially, normal sinus rhythm was achieved in 88% of the 66 infants treated with digoxin with no deaths. Normal sinus rhythm resumed after electrical countershock in 87% of the 15 infants so treated. Maintenance digoxin therapy was used in 85 patients. The Wolff-Parkinson-White pattern disappeared in 36% of the patients. Four infants died of cardiac causes during the mean follow-up period of 6.5 years. Two of these four infants had congenital heart disease; the third, with a normal heart initially, developed ventricular fibrillation and died from a cardiomyopathy considered related to resuscitation. The remaining infant, with a normal heart, died suddenly at 1 month of age. All were receiving digoxin. A wide QRS tachycardia later appeared in three patients, all with heart disease, one of whom died.(ABSTRACT TRUNCATED AT 250 WORDS)

Journal of Cardiovascular Electrophysiology, 1999

Some patients with postoperative congenital heart disease require permanent cardiac pacing, but t... more 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. 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). 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.

Circulation, 2004

A group of relatively uncommon but important genetic cardiovascular diseases (GCVDs) are associat... more A group of relatively uncommon but important genetic cardiovascular diseases (GCVDs) are associated with increased risk for sudden cardiac death during exercise, including hypertrophic cardiomyopathy, long-QT syndrome, Marfan syndrome, and arrhythmogenic right ventricular cardiomyopathy. These conditions, characterized by diverse phenotypic expression and genetic substrates, account for a substantial proportion of unexpected and usually arrhythmia-based fatal events during adolescence and young adulthood. Guidelines are in place governing eligibility and disqualification criteria for competitive athletes with these GCVDs (eg, Bethesda Conference No. 26 and its update as Bethesda Conference No. 36 in 2005). However, similar systematic recommendations for the much larger population of patients with GCVD who are not trained athletes, but nevertheless wish to participate in any of a variety of recreational physical activities and sports, have not been available. The practicing clinician is frequently confronted with the dilemma of designing noncompetitive exercise programs for athletes with GCVD after disqualification from competition, as well as for those patients with such conditions who do not aspire to organized sports. Indeed, many asymptomatic (or mildly symptomatic) patients with GCVD desire a physically active lifestyle with participation in recreational and leisure-time activities to take advantage of the many documented benefits of exercise. However, to date, no reference document has been available for ascertaining which types of physical activity could be regarded as either prudent or inadvisable in these subgroups of patients. Therefore, given this clear and present need, this American Heart Association consensus document was constituted, based largely on the experience and insights of the expert panel, to offer recommendations governing recreational exercise for patients with known GCVDs.

Circulation, 2003

Background-Using fetal magnetocardiography (fMCG), we characterize for the first time the electro... more 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. (Circulation. 2003;107:307-312.)

Cardiology in the Young, 2008

A detailed hierarchal nomenclature of arrhythmias is offered with definition of its applications ... more A detailed hierarchal nomenclature of arrhythmias is offered with definition of its applications to diagnosis and complications. The conceptual and organizational approach to discussion of arrhythmias employs the following sequence: location--mechanism--aetiology--duration. The classification of arrhythmias is heuristically divided into an anatomical hierarchy: atrial, junctional, ventricular, or atrioventricular. Mechanisms are most simplistically classified as either reentrant, such as macro-reentrant atrial tachycardia, previously described as atrial flutter, or focal, such as automatic or micro-reentrant tachycardia, for example, junctional ectopic tachycardia. The aetiology of arrhythmias can be either iatrogenic, such as postsurgical, or non-iatrogenic, such as genetic or congenital, and in many cases is multi-factorial. Assigning an aetiology to an arrhythmia is distinct from understanding the mechanism of the arrhythmia, yet assignment of a possible aetiology of an arrhythmia may have important therapeutic implications in certain clinical settings. For example, postoperative atrial arrhythmias in patients after cardiac transplantation may be harbingers of rejection or consequent to remediable imbalances of electrolytes. The duration, frequency of, and time to occurrence of arrhythmia are temporal measures that further refine arrhythmia definition, and may offer insight into ascription of aetiology. Finally, arrhythmias do not occur in a void, but interact with other organ systems. Arrhythmias not only can result from perturbations of other organ systems, such as renal failure, but can produce dysfunction in other organ systems due to haemodynamic compromise or embolic phenomena.