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Biomedical subjects

Barbara J Deal

Publications and source records attributed to Barbara J Deal.

12 recordsLinked to original sources
PubMed2005

Atrioventricular valve procedures with repeat fontan operations: influence of valve pathology, ventricular function, and arrhythmias on outcome.

BACKGROUND: The purpose of this study is to analyze atrioventricular valve procedures when performed in association with repeat Fontan operations and to determine the influence of atrioventricular valvar pathology, ventricular function, and arrhythmias on outcome. METHODS: Between December 1994 and August 2004, 80 patients had repeat Fontan operations that included arrhythmia surgery (78 of 80), venous pathway revision (78 of 80), atrioventricular valve repair-replacement (15 of 80), and other associated procedures. Mean ages were the following: at operation, 20.3 +/- 8.4 years; at prior Fontan, 7.1 +/- 5.8 years. Atrioventricular valve procedures were performed on 8 functionally mitral and 7 functionally tricuspid valves. The average cross-clamp and cardiopulmonary bypass times were 61.9 +/- 42.8 minutes and 218 +/- 82 minutes, respectively. RESULTS: Ventricular dysfunction (8% vs 54%, p < 0.0001), valvar dysfunction (13% vs 25%, p < 0.05), and atrial arrhythmias (18% vs 86%, p < 0.0001) increased during the preceding 12.0 +/- 4.7 years before the most recent Fontan operation. Multivariate analysis for death, orthotopic cardiac transplantation (OCT), or renal dialysis showed severe ventricular dysfunction, age greater than 25 years, right or ambiguous functional ventricle, and ischemic time greater than 100 minutes to be highly significant. Notably, cardiac index, elevated end diastolic pressure, and atrial fibrillation were not predictors of outcome. Mitral valve repairs were inconsistent due to probable technical misjudgments; most tricuspid valves could not be repaired. Operative and late mortality were 1.2% and 5.0%, respectively. Emergent and late OCT were 1.2% and 3.7%, respectively. CONCLUSIONS: Risk factors for poor outcome are severe ventricular dysfunction, right or ambiguous single ventricle, age greater than 25 years, and ischemic time greater than 100 minutes. Mitral valves are potentially more amenable to repair than are tricuspid valves. Prosthetic valve replacement should be considered when valve repair is questionable.

Adolescent↗
PubMed2004

Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases.

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.

Adolescent↗
PubMed2004

Amiodarone therapy for drug-refractory fetal tachycardia.

BACKGROUND: Fetal tachycardia complicated by ventricular dysfunction and hydrops fetalis carries a significant risk of morbidity and mortality. Transplacental digoxin is effective therapy in a small percentage, but there is no consensus with regard to antiarrhythmic treatment if digoxin fails. This study evaluates the safety, efficacy, and outcome of amiodarone therapy for digoxin-refractory fetal tachycardia with heart failure. METHODS AND RESULTS: Fetuses with incessant tachycardia and either hydrops fetalis (n=24) or ventricular dysfunction (n=2) for whom digoxin monotherapy and secondary antiarrhythmic agents (n=13) were not effective were treated transplacentally with a loading dose of oral amiodarone for 2 to 7 days, followed by daily maintenance therapy for <1 to 15 weeks. Digoxin therapy was continued throughout gestation. Newborns were studied by transesophageal pacing or ECG monitoring to determine the mechanism of tachycardia. Three fetuses were delivered urgently in tachycardia during amiodarone loading, and 3 required additional antiarrhythmic agents for sustained cardioversion. Amiodarone or amiodarone combinations converted 14 of 15 (93%) with reentrant supraventricular tachycardia, 2 of 2 with ventricular or junctional ectopic tachycardia, and 3 of 9 (33%) with atrial flutter. Amiodarone-related adverse effects were transient in 5 infants and 8 mothers. Mean gestational age at delivery was 37 weeks, with 100% survival. CONCLUSIONS: Orally administered amiodarone is safe and effective treatment for drug-refractory fetal tachycardia, specifically reentrant supraventricular tachycardia, junctional ectopic, or ventricular tachycardia, even when accompanied by hydrops fetalis or ventricular dysfunction.

Amiodarone↗
PubMed2004

Surgery for arrhythmias in children.

BACKGROUND: Transcatheter radiofrequency ablation to treat supraventricular and ventricular arrhythmias has supplanted routine surgical ablative therapy and redefined its role. A small population of arrhythmia patients now requires surgical ablation: those who have failed catheter ablation, patients with concomitant congenital heart disease in association with arrhythmias, those with atrial fibrillation and very young patients for whom transcatheter techniques are prohibitive because of small size, cyanosis or distorted anatomy. METHODS: From July 1992 through August 2003, 133 patients underwent arrhythmia surgery at Children's Memorial Hospital, 50% (67/133) in association with Fontan conversion (FC), 22% (28/133) with concomitant initial Fontan (IF) procedure and 28% (38/133) for various arrhythmias (MISC) in patients with (36/38, 95%) or without (2/38, 5%) associated structural heart disease. Mean age at surgery in the FC group was 20+/-7.6 years (median 19 years), and in the IF group and the MISC group, mean ages were 8.1+/-8.9 (median 4.2) years and 16.4+/-10.9 (median 11.3) years, respectively. RESULTS: There were three operative (3/133, 2.6%; 1 FC, 2 MISC) and three late deaths (2 FC, 1 MISC). Four patients in the FC group had progressive ventricular failure and underwent successful cardiac transplantation. Follow-up data are available for non-transplant, surviving patients and reveal 11 incidences of persistent arrhythmia recurrence and 2 new-onset arrhythmias. Five of the 11 recurrences occurred early in our series of FC patients, when isthmus block interruption of arrhythmia foci was performed. Four additional recurrences occurred later in the FC series, two post-maze and two post-Cox-maze III. In the MISC group, there were two recurrences. Atrial reentry tachycardia (ART) recurred in a patient with no structural heart disease and accessory connection-mediated tachycardia recurred in a child who underwent concomitant initial Fontan. Two patients had ventricular tachycardia inducible at postoperative studies (2/7, 29%), but no clinical recurrence. Two new-onset tachycardias occurred, one child developed ART post-surgical ablation of accessory connections and one patient with inducible ventricular tachycardia developed ART 5 years postoperatively. CONCLUSION: Variations in atrial and ventricular anatomy that may limit the catheter approach can be addressed surgically. Patient size or anatomic complexity should not be limiting factors in the combined surgical arrhythmia approach. Incorporation of arrhythmia therapy into planned surgical revision should be considered.

Adolescent↗
PubMed2003

Beyond Fontan conversion: Surgical therapy of arrhythmias including patients with associated complex congenital heart disease.

BACKGROUND: Arrhythmia operations may be extended to patients with failed ablation procedures or associated structural defects requiring surgical intervention. The purpose of this study is to review our experience with arrhythmia operations in 29 patients who did not have Fontan conversions after the introduction of catheter ablation. METHODS: Between July 1992 and January 2002, 29 patients had operations for refractory atrial (n = 24) or ventricular (n = 5) arrhythmias. Mechanisms of arrhythmia included atrial reentry (n = 11), atrial fibrillation (n = 5), automatic atrial (n = 3), accessory connections (n = 6), atrioventricular nodal reentry (n = 2), and ventricular tachycardia (n = 5). Median age at operation was 12.3 years (range, 6 days to 45 years). Two patients had structurally normal hearts; the remaining 27 patients underwent concomitant repair of structural heart disease, including atrioventricular valve replacement or repair (n = 8), anatomy-specific repair of Ebstein's anomaly (n = 4), tetralogy of Fallot repair or revision (n = 4), atrial septal defect closure (n = 3), ventricular septal defect repair (n = 2), Mustard takedown with arterial switch (n = 2), initial Fontan (n = 2), right ventricle-to-pulmonary artery conduit revision (n = 2), Norwood procedure (n = 1), 1 ventricular repair for Uhl's anomaly (n = 1), Mustard baffle revision (n = 1), pulmonary valve replacement with aneurysm resection (n = 1), and aortic valve replacement with complex repair (n = 1). RESULTS: No patient developed heart block, and the surgical mortality rate was 7%. One patient died after Mustard takedown and arterial switch operation, and 1 neonate died after repair of severe Ebstein's anomaly. There was one late death after arterial switch conversion at another institution. Recurrent clinical supraventricular tachycardia was present in 2 patients (2 of 27, 7.4%) and 2 patients had new-onset tachycardias with different underlying mechanisms of arrhythmia at late follow-up (median follow-up 47 months). CONCLUSIONS: Successful surgical therapy of arrhythmias can be performed safely at the time of repair of complex congenital heart disease or in patients with failed catheter ablation procedures. Early consideration for single-stage therapy of arrhythmia and structural heart disease is indicated.

Adolescent↗
PubMed2003

Arrhythmia surgery in association with complex congenital heart repairs excluding patients with fontan conversion.

Surgical arrhythmia therapy may be performed for patients failing the catheter ablation approach or incorporated into repair of complex congenital heart disease. Variations in atrial and ventricular anatomy that may limit the catheter approach can be directly addressed surgically assuring lesion depth and continuity of anatomic lines of block. Between July 1992 and August 2002, we performed arrhythmia surgery on 34 patients for refractory atrial (n = 29) or ventricular (n = 5) arrhythmias. Not included in this series are patients who had arrhythmia surgery during Fontan conversion. The majority of patients had various forms of complex congenital heart disease; two had structurally normal hearts. Median age at surgery was 13.0 years (range, 7 days to 45 years). Five patients were infants (mean age, 25 days). Twenty-two patients (65%) had an average of 2.8 previous cardiac procedures; all required resternotomy. Operative mortality was 5.9% (2 of 34 patients) because of low cardiac output in one patient following Mustard takedown and arterial switch operation and in one neonate with Ebstein's anomaly and pulmonary atresia. Ablative surgery for supraventricular tachycardia (atrial re-entry, automatic atrial, atrioventricular nodal re-entry, and atrial fibrillation) had a 93% success rate (25 of 27 patients). Clinical tachycardia recurred in two of 27 surviving patients (7%) with atrial arrhythmia, one after an arrhythmia-free interval of several years. Ventricular tachycardia was inducible postoperatively in two of three patients with ventricular arrhythmias and congenital heart disease. Patient size or anatomic complexity should not be limiting factors in the combined surgical arrhythmia approach. Because older patients undergoing surgical revision of prior surgical repairs of congenital heart disease are at increased risk for the later development of atrial arrhythmias, incorporation of arrhythmia therapy into any planned surgical revision should be routinely considered.

Arrhythmias, Cardiac↗
PubMed2003

Location of accessory connection in infants presenting with supraventricular tachycardia in utero: clinical correlations.

The most common mechanism of fetal tachycardia is orthodromic reciprocating tachycardia utilizing an accessory atrioventricular connection, however, data regarding accessory connection location in patients with fetal tachycardia is limited. To investigate the location of accessory connections in fetal tachycardia, postnatal transesophageal electrophysiology studies were performed at one institution over a 10-year period in 24 infants with documented fetal tachycardia. The 18 infants with inducible orthodromic reciprocating tachycardia were grouped according to accessory connection location, and groups were compared regarding prenatal presentation and clinical course. Left-sided connections were found in 13 (72%) patients, while accessory connection location could not be determined in the remaining 5 (28%) patients. The presence of a left-sided accessory connection was associated with sustained tachycardia, depressed ventricular function, and the need for antiarrhythmic therapy in utero. No other difference in clinical or electrophysiologic data was found between groups. Our findings indicate that a high proportion of patients with fetal tachycardia have left-sided accessory connections, and a left-sided connection may adversely affect fetal hemodynamics and cardiac output.

Bundle-Branch Block↗
PubMed2002

Comparison of anatomic isthmus block with the modified right atrial maze procedure for late atrial tachycardia in Fontan patients.

BACKGROUND: Late atrial reentry tachycardia (AT) after Fontan repair is common, with limited efficacy of medical therapy in preventing AT recurrence. In this study, two approaches to surgical arrhythmia ablation in patients with refractory AT undergoing Fontan revision are compared: cryoablation of the inferomedial right atrium (RA), and a more extensive modified RA maze procedure designed to eliminate all potential RA reentrant circuits. METHODS AND RESULTS: Fontan revision was performed in 23 patients with AT, using inferomedial RA cryoablation (Group 1, n=8) and modified RA maze procedure (Group 2, n=15). There was no difference in age at initial Fontan, age at Fontan revision, age at onset of AT, or number of failed antiarrhythmic medications. Patients underwent preoperative, intraoperative, and postoperative electrophysiological studies. Thirty-eight different tachycardia circuits were induced in preoperative studies with 3 major areas of RA involvement: the lower lateral RA, the atrial septum, and the inferomedial RA. At postoperative electrophysiological study, AT was inducible in 62% of Group 1 patients but only 7% of Group 2 patients (P<0.02). With mean follow-up of 43 months, 5 of 8 patients in Group 1 experienced AT recurrence compared with none in Group 2 (P<0.001). There was no significant difference in length of hospital stay or complication rate comparing the two groups. CONCLUSION: Modified RA maze procedure is superior to anatomic isthmus block in treating reentrant AT in postoperative Fontan patients. The modified RA maze has eliminated AT recurrence at mid-term follow-up with low morbidity and mortality.

Adolescent↗
PubMed2002

The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan.

Postoperative Fontan patients can develop hemodynamic abnormalities and refractory atrial arrhythmias resulting in significant morbidity and mortality. We present our experience with total cavopulmonary artery conversion and arrhythmia surgery. Between 1994 and 2001, 41 patients underwent total cavopulmonary artery conversion and arrhythmia surgery. Significant hemodynamic lesions were repaired concomitantly: aortic aneurysm (n=1), atrioventricular valve insufficiency (n=8), and pulmonary artery stenosis (n=9). Thirty-five patients were in New York Heart Association class III or IV. Mean age at original Fontan was 7.5+/-6.5 years, at Fontan conversion, 18.7+/-9.0 years. Arrhythmia surgery for atrial re-entry tachycardia evolved from isthmus cryoablation (n=10) to right-sided maze (n=17). Maze-Cox III was used for 14 patients with atrial fibrillation. Atrial (n=34) and dual chamber (n=5) pacemakers were placed. Mortality and reoperation for bleeding rates are 0%. Chest tubes were removed on postoperative day 9.0+/-6.0. Mean hospital stay was 11.8+/-6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was one long-term death from acute myocardial infarction 2 years postoperatively. For the entire series, arrhythmia recurrence is 12.2% (5/41). Only 9.8% of patients (4/41) receive chronic antiarrhythmic medications; these patients were among the first eight in the series. Most patients are in New York Heart Association I or II. Bruce protocol in 12 patients showed increased tolerance (P<.05) Total cavopulmonary artery conversion with concomitant arrhythmia surgery is excellent therapy for patients with failed Fontan. It is safe, improves New York Heart Association class, improves exercise tolerance, and the incidence of recurrent arrhythmias is low.

Adolescent↗