Value of provocative electrophysiology testing in the management of pediatric patients after congenital heart surgery.

OBJECTIVE: To determine the impact of provocative electrophysiology testing in postoperative congenital heart disease (CHD) patients on the management of supraventricular tachycardia (SVT) and clinical outcomes. METHODS: This is a retrospective study including patients <18 years of age with surgery for CHD who had postoperative SVT between 2006 and 2017. Postoperative outcomes were compared between patients with and without postoperative electrophysiology testing using the Wilcoxon rank sum test, Fisher's exact test, Kaplan-Meier method with the log-rank test, and Cox proportional hazard model. RESULTS: From 341 patients who had SVT after surgery for CHD, 65 (19%) had postoperative electrophysiology testing. There was no significant difference in baseline patient characteristics or surgical complexity between patients with and without electrophysiology testing. Patients with inducible SVT on electrophysiology testing were more likely to have recurrence of SVT prior to hospital discharge with an odds ratio 4.0 (95% confidence interval 1.3, 12.0). Patients who underwent postoperative electrophysiology testing had shorter intensive care unit (12 [6, 20] vs 16 [9, 32] days, HR 2.1 [95% CI 1.6, 2.8], P < .001) and hospital (25 [13, 38] vs 31 [18, 54] days, HR 1.8 [95% CI 1.4, 2.4], P < .001) length of stay. CONCLUSION: Postoperative electrophysiology testing was associated with improved postoperative outcomes, likely related to the ability to predict recurrence of arrhythmia and tailored antiarrhythmic management.

Catheter ablation for atrioventricular nodal reentrant tachycardia in patients with congenital heart disease.

BACKGROUND: Variability in atrioventricular (AV) node location in congenital heart disease (CHD) can make catheter ablation for atrioventricular nodal reentrant tachycardia (AVNRT) challenging. OBJECTIVE: The purpose of this study was to describe institutional technique and outcomes for slow pathway modification in a cohort with CHD. METHODS: The study consisted of a retrospective review of CHD patients who underwent study from 2001 to 2013 with a diagnosis of AVNRT. Outcomes for slow pathway modification were recorded. In cases in which ablation was deferred, the reasons for this choice were examined. RESULTS: Forty-nine patients (median age 19 years) were included. CHD anatomy involved d-transposition of the great arteries (n = 6), "congenitally corrected" transposition of the great arteries (n = 4), Ebstein anomaly (n = 4), tetralogy of Fallot (n = 5), venous anomalies (n = 8), single ventricle (n = 16), and miscellaneous (n = 6). Ablation was attempted in 39 patients, using radiofrequency energy in 24, cryoablation in 8, and both in 7. Acute success rate was 92% (36/39). One patient had first-degree block in response to cryoablation, but no other complications occurred. At median follow-up 32 months, 1 patient had AVNRT recurrence. Most of the 10 patients in whom ablation was deferred had single-ventricle anatomy with uncertain AV node location. CONCLUSION: Ablation for AVNRT in CHD can be accomplished successfully with attention to underlying anatomy and prior surgery. Patients with single ventricle are a difficult subgroup, and a pharmacologic approach may be indicated in some cases if node localization is ambiguous.

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.

Radiofrequency catheter ablation of septal accessory pathways in the pediatric age group.

Radiofrequency catheter ablation (RCA) of septal accessory pathways may be technically challenging in children due to the risk of inadvertent atrioventricular (AV) block in the setting of small cardiac dimensions. Outcomes were reviewed for all patients aged < or =19 years with manifest and concealed septal accessory pathways undergoing RCA since 1990 at a single institution. One hundred forty-five procedures were performed in 127 patients (mean age 11.6 years). The number of studies according to accessory pathway location were: anteroseptal (n = 36), midseptal (n = 20), mouth of coronary sinus (n = 40), middle cardiac vein (n = 6), right posteroseptal (n = 21), and left posteroseptal (n = 22). Ablation was deferred for 9 patients (6 anteroseptal and 3 midseptal) in favor of additional pharmacologic trials. Acute success rates for targeted accessory pathways were: anteroseptal (96%), midseptal (94%), mouth of coronary sinus (88%), middle cardiac vein (100%), right posteroseptal (100%), and left posteroseptal (96%). Recurrence rates during follow-up were: anteroseptal (14%), midseptal (12%), mouth of coronary sinus (3%), right posteroseptal (4%), and left posteroseptal (4%). Permanent second or third degree AV block occurred in 4 of 136 RCA attempts (3%), involving 2 anteroseptal and 2 midseptal pathways. In 3 of these 4 cases, a high probability of block was anticipated from prior ablation efforts, prompting pacemaker insertion before or in conjunction with RCA. Thus, in the pediatric age group, acute RCA success rates for septal accessory pathways can exceed 90%. The risks of AV block and accessory pathway recurrence are most relevant to anteroseptal and midseptal pathways. These data may be factored into patient selection and the decision whether to ablate.