Cardiac QTc interval characteristics before and after hematopoietic stem cell transplantation: an analysis of 995 consecutive patients at a single center.

Hematopoietic stem cell transplantation (HSCT) treats disorders affecting patients of all ages. We studied the rate-corrected cardiac QT interval (QTc) in 995 consecutive children and adults undergoing HSCT at the University of Minnesota. We sought to (1) describe QTc before and after HSCT; (2) describe the change in QTc after HSCT; (3) identify factors affecting QTc and its change; and (4) scrutinize an 'at risk' sub-cohort with a long QTc before HSCT. Pre HSCT: 952 (96%) patients had an evaluable electrocardiography (ECG); median QTc was 426 ms and depended upon disease necessitating transplant. Post HSCT: 506 (51%) patients had an evaluable ECG; median QTc was 441 ms. Intrapatient QTc change: 490 (49%) evaluable patients showed median QTc change (pre to post HSCT) of +16 ms (P<0.0001). At risk group: 68 patients were 'at risk' (long pre-HSCT QTc). In some, 'at-risk' status trended toward predictive of post-transplant nonrelapse mortality. QTc interval prolongation is evident in a large, diverse cohort undergoing HSCT at our institution. Prospective studies of this patient population may be warranted, particularly for 'at-risk' patients who demonstrate significant QTc prolongation both pre and post HSCT.

Characterization of the critical cycle length of a left atrial driver which causes right atrial fibrillatory conduction.

A stable rhythm of very short cycle length (CL) in the left atrium (LA) can cause fibrillatory conduction, particularly in the right atrium (RA). Fast Fourier transform (FFT) analysis reliably identifies LA to RA conduction path(s) during atrial fibrillation (AF). We tested the hypotheses that FFT analysis of atrial electrograms (AEGs) during AF simulation will reliably identify the critical LA driver CL that causes RA fibrillatory conduction (i.e., the critical conduction breakdown CL) and that a longer critical conduction breakdown CL is found in atria of abnormal (sterile pericarditis) compared to normal dogs. We paced from Bachmann's bundle and the posterior-inferior LA at rapid rates to mimic an LA driver. During pacing, 4 sec of FFT analysis of 203 bipolar AEGs was performed and showed: 1) a single dominant frequency peak at the pacing CL in both atria when the atria followed the pacing in a 1:1 manner; 2) multiple and broad frequency peaks on the RA and parts of the LA at the conduction breakdown CL; and 3) the conduction breakdown CL is longer in pericarditis than normal dogs. FFT analysis allowed reliable detection of the critical CL of an LA driver that induces RA fibrillatory conduction.

Electrophysiological effects of ibutilide in patients with accessory pathways.

BACKGROUND: Atrial fibrillation (AF) may cause life-threatening ventricular arrhythmias in patients with Wolff-Parkinson-White syndrome. We prospectively evaluated the effects of ibutilide on the conduction system in patients with accessory pathways (AP). METHODS AND RESULTS: In part I, we gave ibutilide to 22 patients (18 men, 31+/-13 years of age) who had AF during electrophysiology study, including 6 pediatric patients

Quantitative effects of functional bundle branch block in patients with atrioventricular reentrant tachycardia.

Changes in the retrograde conduction time (ventriculoatrial [VA]) interval during functional bundle branch block (BBB) have been used to separate septal from free wall accessory pathways (APs), but different values of the VA interval prolongation (deltaVA) have been described in different reports. A total of 95 patients with single nondecremental APs who developed BBB during atrioventricular reentrant tachycardia were studied. Free wall APs were found in 60 patients, and 35 had septal APs. For patients with free wall APs, complete and incomplete BBB ipsilateral to the atrial insertion site of APs were observed in 39 of 60 patients (65%) and 31 of 60 patients (52%), respectively. For patients who had both complete (QRS > or = 120 ms) and incomplete (QRS <120 ms) BBB during atrioventricular reentrant tachycardia, deltaVA for patients with complete BBB was significantly greater than in those with incomplete BBB, 59 +/- 19 ms versus 30 +/- 11 ms, p <0.001. For patients with septal APs and complete and incomplete BBB during tachycardia, the mean deltaVA for those with complete BBB was 31 +/- 20 ms and was significantly longer than in patients with incomplete BBB (14 +/- 6 ms), p <0.001. There was no significant difference in deltaVA between those with free wall APs and incomplete BBB compared with those with septal APs and complete BBB. The criteria of QRS > or = 120 ms associated with deltaVA > or =40 ms served to best separate free wall from septal APs with a sensitivity of 88% and a specificity of 89%. Left anterior fascicular block was associated with marked lengthening of deltaVA for those with left free wall APs, whereas a left posterior fascicular block pattern resulted in a marked increase in the deltaVA for patients with posteroseptal APs. In the absence of fascicular block patterns, a deltaVA > or =40 ms provides strong evidence of a free wall AP, with a sensitivity of 95% and a specificity of 100%. The left posterior fascicle appears to provide predominant innervation of the posterior septum.

Long-term follow-up of patients with long-QT syndrome treated with beta-blockers and continuous pacing.

BACKGROUND: The long-QT syndrome is associated with sudden cardiac death. Combination of beta-blocker and pacing therapy has been proposed for treatment of drug-resistant patients. The purpose of this study was to summarize our long-term experience with combined therapy in patients with long-QT syndrome. METHODS AND RESULTS: A total of 37 patients with idiopathic long-QT syndrome were treated with combined therapy consisting of continuous cardiac pacing and maximally tolerated beta-blocker therapy and followed up for 6.3+/-4. 6 years (mean+/-SD). The group consisted of 32 female and 5 male patients with a mean age of 31.6 years. The mean paced rate was 82+/-7 bpm (range, 60 to 100 bpm). On follow-up, recurrent symptoms caused by pacemaker malfunction were documented in 3 patients. Four patients died during the follow-up period: 2 adolescents stopped beta-blocker therapy, 1 patient died suddenly while treated with combined therapy, and 1 patient died of unrelated causes. In addition, 3 patients had resuscitated cardiac arrest while on combined therapy, and 1 patient had repeated, appropriate implantable cardioverter-defibrillator discharges on follow-up. CONCLUSIONS: Because 28 of 37 patients remain without symptoms with beta-blocker therapy and continuous pacing, combined therapy appears to provide reasonable, long-term control for this high-risk group. However, the incidence of sudden death and aborted sudden death (24% in all patients and 17% in compliant patients) strongly suggests the use of a "back-up" defibrillator, particularly in noncompliant adolescent patients. Implantable cardioverter-defibrillator therapy, however, may be associated with recurrent shocks in susceptible patients.

Electrophysiologic effects of adenosine in patients with supraventricular tachycardia.

BACKGROUND: We correlated the electrophysiologic (EP) effects of adenosine with tachycardia mechanisms in patients with supraventricular tachycardias (SVT). METHODS AND RESULTS: Adenosine was administered to 229 patients with SVTs during EP study: atrioventricular (AV) reentry (AVRT; n=59), typical atrioventricular node reentry (AVNRT; n=82), atypical AVNRT (n=13), permanent junctional reciprocating tachycardia (PJRT; n=12), atrial tachycardia (AT; n=53), and inappropriate sinus tachycardia (IST; n=10). There was no difference in incidence of tachycardia termination at the AV node in AVRT (85%) versus AVNRT (86%) after adenosine, but patients with AVRT showed increases in the ventriculoatrial (VA) intervals (13%) compared with typical AVNRT (0%), P<0.005. Changes in atrial, AV, or VA intervals after adenosine did not predict the mode of termination of long R-P tachycardias. For patients with AT, there was no correlation with location of the atrial focus and adenosine response. AV block after adenosine was only observed in AT patients (27%) or IST (30%). Patients with IST showed atrial cycle length increases after adenosine (P<0.05) with little change in activation sequence. The incidence of atrial fibrillation after adenosine was higher for those with AVRT (15%) compared with typical AVNRT (0%) P<0.001, or atypical AVNRT (0%) but similar to those with AT (11%) and PJRT (17%). CONCLUSIONS: The EP response to adenosine proved of limited value to identify the location of AT or SVT mechanisms. Features favoring AT were the presence of AV block or marked shortening of atrial cycle length before tachycardia suppression. Atrial fibrillation was more common after adenosine in patients with AVRT, PJRT, or AT. Patients with IST showed increases in cycle length with little change in atrial activation sequence after adenosine.

Clinical course of persistent junctional reciprocating tachycardia.

OBJECTIVE: The purpose of this study is to review the clinical course of persistent junctional reciprocating tachycardia (PJRT) in 21 patients spanning a wide age range to examine the electrophysiologic characteristics of the conduction system in these patients with PJRT, particularly in regards to its incessant nature and to evaluate the long-term response to radiofrequency ablation. BACKGROUND: Persistent junctional reciprocating tachycardia is uncommon, occurring in 1% of patients with supraventricular tachycardia. Its presentation, course and treatment are incompletely characterized. METHODS: The clinical, electrocardiographic, electrophysiologic and echocardiographic data of 21 patients with PJRT were reviewed. RESULTS: In 9 of these 21 patients, the mean tachycardia cycle length increased significantly (p < 0.0001) as the patients grew, from a mean tachycardia cycle length of 308+/-64 ms in the patients less than 2 years, 414+/-57 ms in the patients between 2 years and 5 years, to 445+/-57 ms in the patients greater than 5 years, primarily due to slowing of retrograde conduction in the accessory pathway. Persistent junctional reciprocating tachycardia was associated with impaired ventricular function in 11, improving spontaneously in 4 and, after successful ablation of the accessory pathway, in 7. All patients except one were uncontrolled on one or more medications. Ablation of the accessory pathway was successful in 19 of 21 patients. CONCLUSIONS: We conclude that PJRT is characterized by an onset in early childhood and by an age-related prolongation of the tachycardia cycle length mediated primarily through conduction delay in the concealed, retrogradely conducting accessory pathway. Ablation of the accessory pathway provides definitive treatment for PJRT.

Electroanatomical mapping and ablation of the substrate supporting intraatrial reentrant tachycardia after palliation for complex congenital heart disease.

In patients with congenital heart disease who have undergone palliative surgical interventions postoperative arrhythmias frequently complicate the clinical course. Intraatrial reentrant tachycardias (IARTs) are one of the most common forms of postoperative arrhythmias in these patients and can lead to significant morbidity and even mortality. Drug therapy and/or antitachycardia pacing have been disappointing. Ablative therapy with radiofrequency energy offers a potential for cure for these patients but the conventional approach using multielectrode recordings and fluoroscopic guidance is technically difficult and provides limited success. Recent development of a novel nonfluoroscopic technology with electroanatomical mapping using the CARTO mapping/ablation system has shown promising results in defining the arrhythmia circuit, facilitating diagnosis, and guiding ablative therapy. Based on our preliminary experience, a systematic approach to postoperative IART using electroanatomical mapping is described. Further studies are needed to fully evaluate the impact of this new technology on the management and therapy of IART.

Narrow complex tachycardia with VA block: diagnostic and therapeutic implications.

To review our experience with cases of narrow complex tachycardia with VA block, highlighting the difficulties in the differential diagnosis, and the therapeutic implications. Prior reports of patients with narrow complex tachycardia with VA block consist of isolated case reports. The differential diagnosis of this disorder includes: automatic junctional tachycardia, AV nodal reentry with final upper common pathway block, concealed nodofascicular (ventricular) pathway, and intra-Hissian reentry. Between June 1994 and January 1996, six patients with narrow complex tachycardia with episodes of ventriculoatrial block were referred for evaluation. All six patients underwent attempted radiofrequency ablation of the putative arrhythmic site. Three of six patients had evidence suggestive of a nodofascicular tract. Intermittent antegrade conduction over a left-sided nodofascicular tract was present in two patients and the diagnosis of a concealed nodofascicular was made in the third patient after ruling out other tachycardia mechanisms. Two patients had automatic junctional tachycardia, and one patient had atrioventricular nodal reentry with proximal common pathway block. Attempted ablation in the posterior and mid-septum was unsuccessful in patients with nodofascicular tachycardia. In contrast, those with atrioventricular nodal reentry and automatic junctional tachycardia readily responded to ablation. The presence of a nodofascicular tachycardia should be suspected if: (1) intermittent antegrade preexcitation is recorded, (2) the tachycardia can be initiated with a single atrial premature producing two ventricular complexes, and (3) a single ventricular extrastimulus initiates SVT without a retrograde His deflection. The presence of a nodofascicular pathway is common in patients with narrow complex tachycardia and VA block. Unlike AV nodal reentry and automatic junctional tachycardia, the response to ablation is poor.

Electrophysiology of "incisional" reentrant atrial tachycardia complicating surgery for congenital heart disease.

Intraatrial reentrant tachycardia, which occurs frequently in patients who have undergone corrective surgery for congenital heart disease, presents a challenge to successful management. Because the surgical repair sites are invariably critical to the development and maintenance of reentrant atrial tachycardia, we use the term "incisional reentry" to describe these arrhythmias. An understanding of the electrophysiology of such "incisional reentry," and techniques to identify a critical isthmus, are essential for successful ablation of these circuits. A critical isthmus may be identified by the presence of entrainment with concealed fusion. Confirmation that the site is critical to the tachycardia circuit is obtained by an analysis of the relationship between the postpacing interval and the tachycardia cycle length. Advances in mapping from multiple simultaneous sites, along with the ability to create larger, deeper lesions will be needed in order to cure a larger number of these patients. Ultimately, in some cases one must consider each procedure palliative rather than curative, as the disease progresses and substrate evolves and more tachycardia circuits become active.

Selective catheter ablation of the tachycardia focus in patients with nonreentrant junctional tachycardia.

We describe a technique for ablation of junctional tachycardia focus by means of examining the earliest atrial activation sequence during tachycardia. The procedure was successful in 7 of 9 patients, and 1 developed complete atrioventricular block.

Stent redilation in canine models of congenital heart disease: pulmonary artery stenosis and coarctation of the aorta.

In a canine puppy model, pulmonary artery stenosis was created by banding the left pulmonary artery to 30-40% of its original diameter. Animals underwent right heart catheterization and angiography 1-2 mo later, and Palmaz P308 stents were implanted. Stent redilation was performed 3-5 mo later. One mo postredilation, the animals were restudied and sacrificed. Coarctations of the aorta were created by transverse aortic incision and longitudinal repair. P308 stent implantation was performed 2-3 mo later. Stent redilation was performed after 6-10 mo, and the animals were restudied and sacrificed 1-2 mo later. Stent implantation was performed in 6 puppies with pulmonary artery stenosis, as 2 animals developed postoperative pulmonary arterial hypoplasia, precluding stenting. The stenosis diameter increased from 4.8 +/- 0.5 mm to 7.4 +/- 0.6 mm (mean +/- SE) following stenting (P = 0.005), and increased further to 9.2 +/- 0.7 mm following redilation (P < 0.001). There were no significant vessel tears or ruptures. Coarctation stenting was performed in 8 animals. The coarctation was dilated from 5.8 +/- 0.9 mm to 9.8 +/- 0.6 mm (P < 0.001), and to 13.5 +/- 0.5 mm at redilation (P = 0.002). Redilation could not be performed in 1 animal. Aortic rupture and death occurred in 2 of 7 animals at redilation. Stent implantation and redilation in experimental pulmonary artery stenosis appears safe and effective. Though stent implantation for coarctation of the aorta appears safe, there was a 28% aortic rupture rate at stent redilation in this model.

Electrogram patterns associated with successful radiofrequency ablation of accessory pathways in children.

Electrograms observed prior to successful and unsuccessful ablation trials in 33 patients (362 attempts) with manifest pathways and 18 patients (194 attempts) with concealed pathways were compared to identify the electrogram patterns that are associated with successful radiofrequency ablation of accessory atrioventricular connections in young patients (mean age 12.7 years; range 4-22 years). Success was defined as permanent or transient interruption of conduction in the accessory connection. Predictors of success in patients with manifest pathways were local ventricular preexcitation (p &equals 0.0001), left-sidedness (43 or 174) of the accessory connection compared (p &equals 0.04) to right-sidedness (27 of 172), a probable Kent bundle potential (29 of 84 versus 39 of 256; p &equals 0.0001), and short antegrade atrioventricular conduction intervals (53.1 +/- 31.9 ms versus 64.6 +/- 32.0 ms; p &equals 0.02). Predictors of success in patients with concealed pathways were short ventriculoatrial conduction times (103.3 +/- 35.8 ms versus 117.9 +/- 34.8 ms; p &equals 0.01), and left-sided (42 of 125) pathways (p &equals 0.03; versus right-sided, 11 of 60). The presence of a Kent bundle potential was not significant. We conclude that specific electrogram patterns can predict successful ablation of either manifest or concealed accessory pathways. Use of these criteria may reduce the delivery of unnecessary energy to young myocardium.

Mapping and radiofrequency ablation of intraatrial reentrant tachycardia after the Senning or Mustard procedure for transposition ofthe great arteries.

The Senning and Mustard procedures are often associated with the development of atrial tachyarrhythmias, which may be a cause of sudden death. We hypothesized that atrial surgery creates barriers to impulse propagation, establishing potential routes for atrial reentry, and that mapping combined with knowledge of the surgical anatomy could identify zones that are critical to the tachycardia to be targeted for radiofrequency catheter ablation. Patients underwent mapping to identify early sites of atrial activation that were related to anatomic or surgically created obstacles, with confirmation by pacing to demonstrate concealed entrainment. Radiofrequency lesions were placed to connect these obstacles, while observing for tachycardia termination. Thirteen tachycardias were attempted in 10 patients, 10 successfully. Three patients had 2 distinct tachycardias. Successful sites were in right atrial tissue, although in many, a retrograde approach to the pulmonary venous atrium was necessary. Ablation of the clinically documented tachycardia was successful in 9 of 10 patients. The most common successful site was the region of the coronary sinus mouth, approached antegrade or retrograde. Ablation of intraatrial reentrant tachycardias after the Senning or Mustard procedure is feasible using concealed entrainment mapping techniques, but requires a detailed knowledge of the individual surgical anatomy and the ability to approach the pulmonary venous atrium. Radiofrequency ablation offers significant advantages over other management modalities in this patient group.

Use of the His/RVA electrode catheter in children.

Comprehensive electrophysiologic study with radiofrequency ablation requires a number of intracardiac catheters. To reduce the number of catheters placed in children, the authors evaluated a series of customized catheters that combined the functions of two catheters. The customized 6F catheter contains eight electrodes placed in pairs at 4, 5, 6, 7, or 8 cm from the tip for recording the His electrogram and at the tip for right ventricular pacing. The amplitude of the bundle of His potential recorded through the His right ventricular apex (RVA) catheter (n = 63) and the ventricular pacing threshold (in mA) (n = 48) were measured and compared to the maximal bundle of His potential recorded with a 6F hexapolar catheter in 24 and 13 other patients, respectively. The relationship between the distance from the distal electrode pair at the tip and the third electrode from the tip (the His/RVA distance) and patient size was analyzed in 42 patients. Following the initial study in the 90 patients, the selection of the optimal His/RVA catheter for 19 patients was determined by examining the regression plots derived from the first group of 90 patients. The measured His/RVA distance was then determined by noting the His/RVA distance of the catheter used. Regression analysis was then used to evaluate the fit between the predicted His/RVA distance based on weight, height, or body surface area (BSA) and the observed His/RVA distance. The maximal bundle of His electrogram measured in the two groups using the His/RVA catheter was compared. To evaluate catheter stability during the study, the amplitude of the maximal His potential was measured in the 19 patients at the onset, midpoint, and end of the study. The maximal His potential recorded through the octapolar catheter (0.21 mV) was significantly (P < .04) greater than that recorded through the hexapolar catheter (0.10 mV). The mean ventricular threshold measured through the octapolar catheter (0.44 mA) was significantly (P < .001) less than that measured through the hexapolar catheter (1.13 mA). There was a significant (P < .0001) correlation between BSA, weight, and height and the His/RVA distance. There was no significant difference in the mean maximal amplitude of the His potential (0.21 +/- 0.31 mV vs 0.15 +/- 0.12 mV) recorded through the His/RVA catheter between the two groups. The His/RVA distance estimated by weight, when plotted against the measured distance, demonstrated a good correlation (r = .84) between the expected His/RVA distance based on the subject's weight and that actually observed. In 18 of 19 subjects, the first catheter based on the patient's weight (in kilograms) predicted the appropriate and only catheter used. There was no significant difference in the mean maximal bundle of His electrogram recorded at the beginning of the study (0.15 +/- 0.12 mV), midway into the study (0.15 +/- 0.11 mV), and at the end (0.13 +/- 0.13 mV); however, there was extensive variation within individuals and over time. These data support the recording of a stable, high-quality bundle of His electrogram and RVA pacing through a single catheter system and, hence, have important, practical implications for invasive electrophysiologic studies in children.

The use of radiofrequency energy in pediatric cardiology.

The use of radiofrequency energy for the treatment of supraventricular tachycardia in pediatric patients has gained widespread acceptance, especially for tachyarrhythmias associated with palpitations, dizziness, presyncope or syncope, cardiomyopathy, and cardiac arrest. Ablation of the substrate supporting atrioventricular reentry, atrioventricular node reentry, and automatic atrial tachycardia yields a 90%-98% success rate with low incidence (< 1%) of complications and adverse side-effects. Ablation of intra-atrial reentry, including atrial flutter and fibrillation, appears to be promising and would be a significant advance in the management of patients following extensive atrial surgery for congenital heart disease. Radiofrequency energy is also used to treat various forms of idiopathic ventricular tachycardia. Finally, radiofrequency energy has been extended to control the ventricular rate associated with malignant atrial tachycardia by either modification or ablation of the atrioventricular node, and subsequent pacemaker implant. Long-term outcome of radiofrequency ablation is unknown, but the short-to-intermediate (1-5 yrs) outcome is excellent, with low recurrence rate of the tachycardia, no proarrhythmic effect, and excellent clinical state.

Catheter interruption of atrioventricular conduction using radiofrequency energy in a patient with transposition of the great arteries.

Percutaneous catheter mapping and radiofrequency ablation of the AV node-His bundle system (with subsequent transvenous endocardial ventricular pacing) were performed on an 18-year-old woman with transposition of the great arteries and intact ventricular septum and chronic arrhythmias 15 years following a Mustard operation. Exclusion of the AV conduction tissue from the systemic venous circulation by the complex anatomy and the Mustard repair was circumvented by a retrograde approach across the aortic valve to the morphological right ventricle yielding access to the AV node-His bundle system.

Improving results with first-stage palliation for hypoplastic left heart syndrome.

Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.

Delayed response to radiofrequency ablation of accessory connections.

This article summarizes delayed interruption in anomalous conduction through accessory connections following radiofrequency ablation attempts in three patients. The time course of the delayed interruption in accessory connection conduction suggests that such an effect is unlikely to occur after the first week following unsuccessful radiofrequency ablation.