Mechanism and ablation of arrhythmia following total cavopulmonary connection.

BACKGROUND: The ability to identify and ablate different arrhythmia mechanisms after the total cavopulmonary connection has not been studied in detail. METHODS AND RESULTS: After obtaining Institutional Review Board approval according to institutional guidelines, consecutive patients after a total cavopulmonary connection undergoing electrophysiology study over a 6-year period were included (2006-2012). Arrhythmia mechanism was determined, and the procedural outcome was defined as complete, partial success, or failure. A 12-point arrhythmia severity score was calculated for each patient at baseline and on follow-up. Fifty-seven procedures were performed on 52 patients (18.4 ± 11.8 years; 53.0 ± 27.2 kg). Access to the pulmonary venous atrium was necessary in 33 procedures, via fenestration (16) or transbaffle puncture (17), and in 2 cases, an additional retrograde approach was used. In total, 80 arrhythmias were identified in 47 cases: macroreentrant (n = 25) or focal atrial tachycardia (n = 8), atrioventricular nodal reentry tachycardia (n = 13), reentry via an accessory pathway (n = 4) or via twin atrioventricular nodes (n = 4), ventricular tachycardia (n = 5), and undefined atrial tachycardia (n = 21). Procedural outcome in 32 patients who underwent ablation was complete success (n = 25), partial success (n = 3), failure (n = 3), or empirical ablation (n = 1). After successful ablation, there was a significant decrease in arrhythmia score over 18.2 (4-32) months follow-up, with a sustained trend even in the face of arrhythmia recurrence (50%). CONCLUSIONS: Arrhythmia mechanism post total cavopulmonary connection is highly varied, encompassing simple and more complex substrates, documentation of which facilitates a strategic approach to invasive arrhythmia management. Despite the anatomic limitations, successful and clinically meaningful ablation is possible.

The use of an integrated electroanatomic mapping system and intracardiac echocardiography to reduce radiation exposure in children and young adults undergoing ablation of supraventricular tachycardia.

AIMS: Non-fluoroscopic imaging (NFI) devices are increasingly used in ablations. The objective was to determine the utility of intracardiac echocardiography (ICE) in ablating paediatric supraventricular tachycardias (SVTs) and assess whether its integrated use with electroanatomic mapping (EAM) resulted in lower radiation exposure than use of EAM alone. METHODS AND RESULTS: Prospective, controlled, single-centre study of patients (pts) age ≥10 years, weight ≥35 kg, with SVT and normal cardiac anatomy. Patients were randomized to ICE + EAM (ICE) or EAM only (no ICE). Both had access to fluoroscopy as needed. Eighty-four pts were enroled (42 ICE, 42 no ICE). Median age was 15 years (range 10.4-23.7 years); 57% had accessory pathways, 42% atrioventricular nodal reentry tachycardia. There was no difference in radiation dose (9 mGy ICE vs. 23 mGy no ICE, P = 0.37) or fluoroscopy time (1.1 min ICE vs. 1.5 min no ICE, P = 0.38). Transseptal punctures were performed in 25 pts (16 ICE, 9 no ICE), with ICE reducing radiation (8 mGy ICE vs. 62 mGy no ICE, P = 0.002) and fluoroscopy time (1.1 min ICE vs. 4.5 min no ICE, P = 0.01). Zero fluoroscopy was achieved in 13 pts (15% of total, 5 ICE, 8 no ICE), and low-dose cases (<50 mGy) in 57 pts (68% of total, 33 ICE, 24 no ICE). Acute success was 95% for ICE, 88% for no ICE. CONCLUSION: Use of an integrated EAM/ICE system was no better than EAM alone in limiting radiation, but can be helpful for transseptal punctures. Given the low dose savings, use of ICE may be weighed against its financial cost. Low-fluoroscopy cases are performed in most NFI procedures.

Radiofrequency-assisted transseptal perforation for electrophysiology procedures in children and adults with repaired congenital heart disease.

BACKGROUND: Patients with congenital heart disease carry a high burden of arrhythmias and may pose special challenges when these arrhythmias are addressed invasively. We sought to describe our early experience with radiofrequency (RF) needle transseptal perforation to facilitate ablation procedures in this population. METHODS: Retrospective chart review to identify all cases of attempted transseptal access with a commercial RF needle at Children's Hospital Boston between February 2007 and January 2010. RESULTS: A total of 10 patients had attempted RF transseptal perforation. Median age was 27 years. Five patients had undergone atrial switch procedures (Mustard/Senning), four had undergone Fontan operations, and one had atrial septal defect repair. The indication for left atrial access was mapping/ablation of atrial flutter in nine cases, and left-sided accessory pathway in one case. The RF needle was chosen primarily in eight of 10 cases, whereas in the remaining two cases RF was used only after failed attempts with a conventional Brockenbrough needle. Septal material was atrial muscle in five cases, pericardium in three, and synthetic fabric in two. In nine of 10 patients, RF transseptal perforation was successful, including both patients in whom a conventional needle had failed. There were no clinically significant complications. CONCLUSIONS: RF transseptal perforation can be an effective method of obtaining left atrial access for electrophysiologic procedures in patients with complex congenital heart disease, including cases where a conventional Brockenbrough needle has failed.

Ablation of nonautomatic focal atrial tachycardia in children and adults with congenital heart disease.

INTRODUCTION: Nonautomatic focal atrial tachycardia (NAFAT) has been characterized in adults with structurally normal hearts. This article characterizes NAFAT in a population of patients with complex congenital heart disease. METHODS AND RESULTS: Electrophysiologic and electroanatomic mapping data and acute outcomes were reviewed in patients undergoing mapping and ablative procedures for atrial tachycardia at Children's Hospital, Boston, between January 1999 and December 2003. Twenty-two NAFAT foci were identified in 17 patients out of 216 patients studied. Fourteen of these 17 patients had congenital heart disease. The average age of the patients with a NAFAT mechanism was 27 years and there was no gender predilection. The presumptive diagnosis based on clinical grounds and surface ECG assessment in 11 of 17 patients with NAFAT was atrial flutter. None of the 17 patients were suspected of having a NAFAT mechanism by noninvasive assessment. Four of the 10 patients had both NAFAT and macroreentrant atrial tachycardias. NAFAT cycle lengths varied widely (200-680 ms) between patients. Sixteen of the 22 NAFAT foci were mapped to the anatomic right atrium (RA). Acute ablative success was achieved in 17 out of 22 foci (77%). CONCLUSION: NAFAT is relatively uncommon in a pediatric tertiary care setting, and in that setting occurs most often in adults with congenital heart disease. NAFAT is indistinguishable from other forms of atrial tachycardia by noninvasive means and can mimic other forms of atrial tachycardia on electrocardiogram. The foci were predominantly found in the RA and were, in most cases, acutely amenable to catheter ablation therapy.

Inability to cannulate the coronary sinus in patients with supraventricular arrhythmias: congenital and acquired coronary sinus atresia.

We report 4 cases of congenital and acquired coronary sinus ostial atresia incidentally found during electrophysiologic assessment for supraventricular arrhythmias. Congenital variants consisted of coronary sinus drainage predominantly via persistent left superior vena cavae and partial coronary sinus unroofing into the left atrium. The acquired variant was inadvertently produced during surgery for cor triatriatum. A variety of electrophysiologic substrates including right and left-sided accessory pathways and both typical and atypical AV nodal reciprocating tachycardia were identified. Approaches to imaging and accessing the coronary sinus when the os cannot be cannulated are discussed, including a search for right atrial accessory venous collaterals, venography to rule-out a persistent left superior vena cava, and coronary angiography.

Improved localization of right-sided accessory pathways with microcatheter-assisted right coronary artery mapping in children.

INTRODUCTION: Radiofrequency ablation of some right-sided accessory pathways continues to be challenging. The purpose of this study was to evaluate the efficacy of introducing a multielectrode microcatheter in the right coronary artery to localize accessory pathways when conventional mapping was unsuccessful. METHODS AND RESULTS: A retrospective study was conducted of all right-sided accessory pathway radiofrequency ablation procedures in which a multielectrode microcatheter as a reference in the right coronary artery was used to assist mapping. Between January 1998 and January 2002, 10 patients (5 males) underwent mapping of 11 accessory pathways with this technique at mean age of 12.3 +/- 3.8 years. Five patients had Ebstein's anomaly of the tricuspid valve. Accessory pathways were identified in the following locations: right anterior 3, right anterolateral 2, right lateral 1, right posterolateral 2, and right posterior 3. Mean procedure time was 285 +/- 70.3 minutes, and fluoroscopy time was 68.7 +/- 21 minutes. Average mapping duration prior to microcatheter insertion in the right coronary artery was 136 +/- 40 minutes. After microcatheter placement in the right coronary artery, the time to successful ablation of accessory pathways was 38 +/- 11 minutes. All 11 (100%) accessory pathways were successfully ablated. CONCLUSION: Mapping in the right coronary artery with a microcatheter is an effective method to improve localization and successful ablation of difficult right-sided accessory pathways.

Ablation of atrioventricular nodal reentrant tachycardia in tricuspid atresia.

A 31-year-old woman with tricuspid atresia and a palliative Waterston shunt presented with intractable poorly tolerated supraventricular tachycardia. Electrophysiologic evaluation was consistent with AV nodal reentrant tachycardia. The fast pathway region was mapped electroanatomically during tachycardia and with constant rate ventricular pacing. Successful radiofrequency ablation was achieved by placement of lesions in an area adjacent and inferior to the His-bundle electrogram on the left side of the septum.