Rare Cause of Infranodal Block.

The patient exhibits multiple features suggestive of Timothy syndrome, which is a multisystem autosomal-dominant condition with findings that include prolonged QT interval, hand and foot abnormalities, dysmorphic facial features, and mental retardation. A 2:1 infranodal atrioventricular block may occasionally be seen in the setting of severely prolonged QT interval. Functional nature of atrioventricular block is demonstrated by resumption of 1:1 conduction with changes in heart rate.

Cardiac resynchronization therapy in patients with challenging anatomy due to venous anomalies or adult congenital heart disease.

BACKGROUND: Cardiac resynchronization therapy (CRT) has proven salutary effects in patients with congestive heart failure, systolic dysfunction, and electromechanical dyssynchrony in the setting of ischemic, nonischemic, and congenital cardiomyopathy. While CRT device implants have become routine in the adult ischemic or nonischemic cardiomyopathy populations, patients with congenital heart disease offer special challenges due to unusual anatomic variations. METHODS: A comprehensive assessment of anatomic abnormalities is essential prior to implant. In addition, implant techniques and equipment must be tailored to the expected anatomy. A flexible approach is necessary-implant may require equipment and techniques adapted from vascular intervention. CONCLUSION: This article describes our approach to CRT implant in patients with congenital heart disease, and is illustrated by reports of several cases.

Inappropriate shocks in patients with Fidelis® lead fractures: impact of remote monitoring and the lead integrity algorithm.

INTRODUCTION: The role of remote monitoring combined with lead integrity algorithm (LIA) in patients with Fidelis (Medtronic Inc., Minneapolis, MN, USA) lead fractures is not well defined. METHODS: We retrospectively identified patients with Fidelis lead fractures at our institution, documenting all pertinent data (remote monitoring use, clinical presentation, lead fracture diagnosis criteria). Patients were classified into subgroups based on the type of home monitoring and whether LIA was uploaded before lead fracture. Subgroups were compared based on delivery of inappropriate shocks (IS). RESULTS: A total of 131 patients (mean age 62 ± 16 years, 70% male, 69% primary prevention implants) were followed until lead fracture (average 32 ± 12 months). IS were delivered in 21% of patients (n = 11/52) with LIA versus 52% (n = 41/79) without LIA, P < 0.001. LIA significantly decreased the number of IS (2.1 ± 1.0 IS vs 7.9 ± 12 IS, P < 0.001) and significantly increased the number of patients diagnosed through audible alert (P < 0.001). Wireless monitoring significantly decreased the time interval to reprogram defibrillators OFF (mean 1.5 ± 1 days vs 15.6 ± 18 days with nonwireless CareLink [Medtronic Inc.] and 12.4 ± 20 days without CareLink, P < 0.001); 14% of patients with LIA and wireless monitoring combined received IS. Without LIA, 63% of patients with wireless monitoring received IS. CONCLUSION: This study confirms that LIA significantly decreases IS therapy in patients with Fidelis lead fractures. Wireless technology enhances LIA benefits by significantly shortening time to reprogram defibrillators. However, despite the "best scenario" of combining LIA and wireless monitoring, 14% of patients with lead fractures still get IS. Further refinements of detection algorithms are required to eliminate this significant clinical problem.

Cardiac Image Registration: Rotational Error Correction and Gated Stabilization for Cardiac Motion.

Background: Dynamic motion of the heart due to cardiac and respiratory cycles, and rotation from varying patient positions between imaging modalities, can cause errors during cardiac image registration. This study used phantom, patient and animal models to assess and correct these errors. Methods and Results: Rotational errors were identified and corrected using different phantom orientations. ECG-gated fluoro images were aligned with similarly gated CT images in 9 patients, and accuracy assessed during atrial fibrillation (AF) and sinus rhythm. A tracking algorithm corrected errors due to respiration; 4 independent observers compared 25 respiration sequences to an automated method. Following correction of these errors, target registration error was assessed. At 20 mm and 30 mm from the phantom model's center point with an in-plane rotation of 8 degrees, measured error was 2.94 mm and 5.60 mm, respectively, and the main error identified. A priori method accurately predicted ECG location in only 38% (p=0.0003) of 313 R-R intervals in AF. A posteriori method accurately gated the ECG during AF and sinus rhythm in 97% and 98% of 375 beats evaluated, respectively (p=NS). Tracking algorithm for ECG-gated motion compensation was identified as good or fair 96% of the time, with no difference between observers and automated method (chi-square=25; p=NS). Target registration error in phantom and animal models was 1.75±1.03 mm and 0 to 0.5 mm, respectively. Conclusions: Errors during cardiac image registration can be identified and corrected. Cardiac image stabilization can be achieved using ECG gating and respiration.

Periprocedural anticoagulation for atrial fibrillation ablation.

BACKGROUND: Catheter ablation for atrial fibrillation (AF) can increase risk of left atrial (LA) thrombi and stroke. Optimal periprocedural anticoagulation has not been determined. OBJECTIVE: We report the role of administering warfarin and aspirin without low molecular weight heparin in patients undergoing AF ablation. METHODS: A total of 207 patients underwent ablation for AF. Transesophageal echocardiography (TEE) guided transseptal puncture and ruled out clot in the LA. After first puncture, the sheath was flushed with heparin (5,000 Units/mL). After second puncture, a bolus of 80 units/kg of heparin was given, followed by an infusion to maintain activated clotting time (ACT) around 300-350 seconds. Warfarin was stopped and aspirin was started (325 mg/day) 3 days preprocedure. Warfarin was restarted on the day of the procedure. Both medications were continued for 6 weeks postablation. Warfarin was continued for 6 months in patients with prior history of persistent or recurrent AF. Thirty-seven patients who showed smoke in the LA on TEE were given low molecular weight heparin postprocedure until international normalized ratio (INR) was therapeutic. RESULTS: Thirty-two patients had persistent and 175 had paroxysmal AF; 87 were cardioverted during ablation. Two patients had transient ischemic attack (TIA) on the sixth and eighth days, respectively, following ablation, with complete recovery. Both had subtherapeutic INRs. CONCLUSION: In patients without demonstrable clot or smoke in the LA, starting aspirin 3 days prior and warfarin immediately post-radiofrequency ablation, without low molecular weight heparin, with meticulous anticoagulation during the procedure, appears to be a safe mode of anticoagulation.

Biventricular pacing and QT interval prolongation.

INTRODUCTION: The purpose of this study was to examine BiV pacing-dependent changes in QT interval and the related potential for proarrhythmia. Biventricular (BiV) pacing has emerged as a promising therapy for patients with advanced congestive heart failure (CHF) and bundle branch block (BBB). METHODS AND RESULTS: One hundred and seventy-six consecutive patients (123 men and 53 women; mean age 67 +/- 16 years) with ischemic (n = 128) or nonischemic (n = 48) cardiomyopathy in New York Heart Association Class II (8%) or III (92%) CHF (ejection fraction 24 +/- 9%) underwent atrial synchronous BiV pacing. The QRS, QT, and JT intervals were measured at 30 minutes after initiation of BiV pacing, at 24 hours, and at 1 month postimplant. QT interval was defined as the time interval between the initial deflection of the QRS complex and the point at which the T wave crossed the isoelectric line. At baseline, the average QRS duration was 178 +/- 10 ms, attributable to left BBB (n = 158) or intraventricular conduction delay (n = 18). BiV pacing resulted in a small but statistically significant reduction in QRS duration (148 +/- 9 ms during BiV pacing vs 178 +/- 10 ms at baseline [P < 0.0001]), yet the QT increased to 470 +/- 34 ms with BiV pacing versus 445 +/- 32 ms at baseline [P < 0.0001]). The JTc interval during BiV pacing was significantly shorter than during LV pacing (290 +/- 9 ms vs 320 +/- 20 ms, P < 0.0001). During a mean follow-up of 24 +/- 6 months, one patient developed recurrent torsade de pointes. That was eliminated once left ventricular pacing was discontinued. CONCLUSION: Biventricular pacing prolongs QT interval. However, the occurrence of torsade de pointes is uncommon.

Computed tomography-fluoroscopy image integration-guided catheter ablation of atrial fibrillation.

INTRODUCTION: This study examines the feasibility of atrial fibrillation (AF) ablation using registered three-dimensional computed tomography (CT) images of the left atrium with fluoroscopy. METHODS AND RESULTS: A total of 50 consecutive patients with symptomatic AF refractory to medical therapy (32 paroxysmal, 18 persistent, age 55 +/- 10 years) were randomized to undergo a catheter-based AF ablation procedure with or without the CT-fluoroscopy guidance system. All patients underwent preprocedural contrast-enhanced CT imaging and segmentation of the left atrium. For the CT-fluoroscopy group, circumferential lesions encompassing the pulmonary vein (PV) antrum and linear lesions along the roof of the left atrium between the superior PVs and the mitral isthmus were created on the CT image, which was registered with real-time fluoroscopy. The registered images were then used to navigate the ablation catheters to the sites of planned ablation. After the ablation sites were completed, any remaining PV potentials were isolated with electrophysiological guidance. In the control patients, the same technique was performed without using the CT-fluoro guidance system. CT scans were accurately registered to fluoroscopic images with minimal manual correction. Operators could navigate catheters on the registered images to preplanned, extraostial sites for ablation. CT-fluoroscopy guidance decreased procedure duration and fluoro times (P < 0.05). At a mean follow-up of 9 +/- 2 months, 21 patients (84%) in the CT-fluoro guidance group and 16 patients (64%) in the control group have had no recurrence of AF. CONCLUSION: CT-fluoroscopic-guided left atrial ablation is feasible and allows appropriate catheter manipulation in the left atrium.

Posterior left atrial-esophageal relationship throughout the cardiac cycle.

BACKGROUND: Radiofrequency energy delivered throughout the cardiac cycle has the potential to cause thermal injury to the esophagus if the anatomical relationship between the posterior left atrium and the esophagus changes during cardiac motion. OBJECTIVE: To assess the posterior left atrial-esophageal relationship throughout the cardiac cycle. METHODS: In this study, the anatomical relationship between the posterior left atrium and the esophagus was assessed throughout the cardiac cycle in 10 consecutive patients. All patients underwent contrast-enhanced, ECG-gated CT scanning. Left atrial volumes and the esophageal structure were generated from the reconstructed data at 10 phases of the cardiac cycle from 5% to 95% of the R-R interval. The posterior left atrial-esophageal anatomical relationship was measured at four levels, the superior pulmonary vein ostial site, and the upper, mid and lower left atrium. RESULTS: There were significant variations in the left atrial-esophageal relationship in the 10 patients. The relative movement between the esophagus and the posterior left atrium throughout the cardiac cycle in the anteroposterior and right-to-left orientations was 0.55 +/- 0.99 mm and 0.60 +/- 1.02 mm (95% confidence interval, 2.03 and 1.98 respectively). CONCLUSIONS: Under normal conditions, there is little change in the anatomical relationship between the posterior left atrium and the esophagus during the entire cardiac cycle. However, due to the interpatient variability at the esophageal location, identification of esophageal location may help prevent complications during catheter ablation procedures involving the left atrium.

Pulmonary vein isolation and linear lesions in atrial fibrillation ablation.

BACKGROUND: Various strategies have been used for atrial fibrillation (AF) ablation. It is unclear whether adding linear lesions to pulmonary vein (PV) isolation has significant advantages. OBJECTIVES: We assessed the clinical benefit of adding linear lesions in patients undergoing PV isolation for AF. METHODS: One hundred patients (63 male and 37 female; mean age of 59 +/- 11 years) with documented paroxysmal AF were included in the study. Patients were randomized into two groups. The first group underwent PV isolation alone. The second group underwent PV isolation and had two linear lesions created; one line between the superior PVs, and a second line from the left inferior PV to the mitral valve annulus. Patients' clinical progress after the ablation was evaluated and compared at 1, 3, and 9 months after their respective ablation procedures. RESULTS: The linear lesions group maintained sinus rhythm and had fewer symptoms than the lone PV isolation group (86 vs. 58%, respectively) (p < 0.05) at 1 month. At 9 months, when patients who reverted to AF underwent additional management to regain sinus rhythm (90 vs. 82%, respectively) (p = NS), there was no statistical difference between the groups regarding the use of antiarrhythmics, the need for electrical cardioversion, and subjective improvement. CONCLUSION: The addition of linear lesions to PV isolation more effectively achieved sinus rhythm initially and fewer patients required additional management to maintain their rhythm when compared to patients who underwent lone PV isolation. However, at 9 months, the overall results were similar in both groups.

Catheter location, tracking, cardiac chamber geometry creation, and ablation using cutaneous patches.

OBJECTIVE: The ability to construct a three-dimensional (3-D) surface model of the endocardium and track the location of catheters within a cardiac chamber, using only cutaneous patches, would be a useful advancement in treating arrhythmias. We tested the feasibility of such a system, Ensite NavX (Endocardial Solutions, Inc., St. Paul, MN, USA), in patients undergoing catheter ablation for SVTs. METHODS: Sixteen patients with 20 arrhythmias undergoing ablation were selected. Skin electrode patches were placed on the chest to create a 3-D coordinate system. A low-amplitude, 5.7 kHz signal emitted from the patches was received by conventional catheters positioned in the heart. Catheter location was determined by measuring the field strength received by the catheters. Location points were successively acquired while catheters were moved throughout the chamber. This information was collected and processed by a workstation to create a detailed 3-D model of the endocardial surface. Anatomic landmarks were labeled on the model as the mapping catheter was navigated. 3-D cardiac chamber geometry reconstruction, landmark labeling, and real time catheter tracking were performed successfully in all patients. Up to six catheters, with a total of up to 26 intracardiac electrodes, were tracked simultaneously. RESULTS: Constructed geometries, including major vessels and valves, correlated closely with traditional anatomic models as well as intracardiac recordings and fluoroscopic images. CONCLUSIONS: Real-time catheter tracking and 3-D cardiac chamber model construction is feasible using cutaneous patches and conventional catheters. This approach may be useful in the treatment of patients with cardiac arrhythmias where ablation therapy is primarily anatomically based.

Role of invasive and noninvasive testing in risk stratification of sudden cardiac death in children and young adults: an electrophysiologic perspective.

Sudden cardiac death is a rare, but devastating, event in the young population. Arrhythmia is the mechanism of death in many cases. In addition to clinical history, noninvasive and invasive tests can be used to identify patients who are at risk. Although these tools are not perfect, they can prove valuable if used in proper clinical circumstances. An overview of these tests is presented.

The implantable cardioverter defibrillator: technology, indications, and impact on cardiovascular survival.

Since the introduction of the implantable cardioverter defibrillator (ICD) for the management of patients with high risk of arrhythmic SCD, there has been increasing use of this device. Its basic promise to effectively terminate ventricular tachycardia (VT)-ventricular fibrillation (VF) has been repeatedly met. In several randomized trials, the ICD has been shown to be superior to conventional anti-arrhythmic therapy, both in patients with documented VT-VF (secondary prevention) and those with high risk such as left ventricular ejection fraction and no prior sustained VT-VF (primary prevention). In both groups, the ICD showed overall and cardiac mortality reduction. The device now can more accurately detect VT-VF and differentiate these from other arrhythmias through a series of algorithms and direct-chamber sensing. Therapy options include painless antitachycardia pacing, low-energy cardioversion, and high-energy defibrillation. The technique implant is now simple as a pacemaker with one lead attached to an active (hot) can functioning as the other electrode. Among other improvements is its weight, volume, multiprogrammability, and storage of information,dual-chamber pacing and sensing, dual-chamber defibrillation, and addition of biventricular pacing for cardiac synchronization. It is anticipated that further improvement in ICD technology will take place and the list of indications will grow.

Noncontact and electroanatomic mapping of atrial flutter in surgically repaired sinus venosus atrial septal defect and rerouting of anomalous pulmonary venous drainage.

Atypical atrial flutter with two prior failed ablations, complicating surgically repaired sinus venosus atrial septal defect and partial anomalous pulmonary venous connection, mapped by noncontact and electroanatomic mapping, is described. Electroanatomic and noncontact mapping clearly identified a narrow zone of normal voltage and activation which was targeted, with successful termination of the arrhythmia.

Termination of paroxysmal supraventricular tachycardia by tecadenoson (CVT-510), a novel A1-adenosine receptor agonist.

OBJECTIVES: The aim of this study was to evaluate tecadenoson safety and efficacy during conversion of paroxysmal supraventricular tachycardia (PSVT) to sinus rhythm. BACKGROUND: Tecadenoson (CVT-510), a novel adenosine receptor (Ado R) agonist, selectively activates the A1 Ado R and prolongs atrioventricular (AV) nodal conduction at doses lower than those required to cause A2 Ado R-mediated coronary and peripheral vasodilation. Unlike adenosine, which non-selectively activates all four Ado R subtypes and produces unwanted effects, tecadenoson appears to terminate AV node-dependent supraventricular tachycardias without hypotension and bronchoconstriction. METHODS: In this open-label, multicenter, dose escalation study, tecadenoson was administered to 37 patients (AV node re-entrant tachycardia, n = 29; AV re-entrant tachycardia, n = 8) with inducible PSVT sustained for > or =1 min during an electrophysiology study. Seven regimens (0.3 to 15 microg/kg) of up to two identical tecadenoson intravenous bolus doses were administered. RESULTS: After the first or second bolus, PSVT converted to sustained sinus rhythm for > or =5 min in 86.5% (32/37) of the patients, with 91% (29/32) of the conversions occurring after the first bolus (most within 30 s), coincident with anterograde conduction block in the AV node. No effects on sinus cycle length (SCL) or systolic blood pressure were observed. The atrial-His (AH), but not the His-ventricular (HV) interval was prolonged up to 5 min after the final tecadenoson bolus, returning to baseline by 10 min. Tecadenoson was generally well tolerated. CONCLUSIONS: In this study, tecadenoson rapidly terminated sustained PSVT by depressing AV nodal conduction without causing hypotension. After sinus rhythm restoration, there was minimal AH interval prolongation without HV interval or SCL prolongation.

Signal-averaged P-wave analysis of normal controls and patients with paroxysmal atrial fibrillation: a study in gender differences, age dependence, and reproducibility.

BACKGROUND: Atrial fibrillation is often first recognized after a complication such as embolic stroke has occurred. Limited data are available for the prospective identification of patients at risk for developing atrial fibrillation. HYPOTHESIS: Demonstration of areas of slow conduction in the atrium by means of P-wave signal averaging may identify individuals at risk for atrial fibrillation. METHODS: P-wave signal averaging from the surface electrocardiogram was performed in 199 normal controls and 81 patients with paroxysmal atrial fibrillation using an automated, P-triggered, high-resolution signal for analysis. RESULTS: Of the variables measured, the filtered P-wave duration and P-wave integral were significantly different between controls and patients (filtered P-wave duration 120 +/- 9 vs. 145 +/- 21 and P-wave integral 666 +/- 208 vs. 868 +/- 352), whereas the terminal root-mean-square (RMS) voltages (RMS 20, RMS 30, RMS 40) showed no significant differences between the two groups. Regression analysis of the first and second measurement of the filtered P-wave duration obtained during consecutive tests showed excellent reproducibility (r and r2 of 0.96 and 0.92). The duration of the filtered P wave showed no age dependence but was shorter in women. CONCLUSION: Utilizing the 90th percentile value of the filtered P-wave duration of 133 ms in men and 130 ms in women, the sensitivity was 80 and 81%, the specificity 92 and 90%, the positive predictive value 84 and 73%, and the negative predictive value 90 and 93%, respectively.