Faculty Bibliography

BACKGROUND: Transvenous pacemaker leads in children are often placed with redundant lead length to allow for anticipated patient growth. This excess lead may rarely prolapse into the pulmonary artery and potentially interfere with valve function. We sought to determine the response to lead repositioning on pulmonary valve insufficiency. METHODS: Retrospective reviews of demographics, lead type, implant duration, and radiography and echocardiography. RESULTS: A total of 11 pediatric patients were identified with lead prolapse through the pulmonary valve, of which nine patients underwent procedures to retract and reposition the lead (age at implant 9 +/- 4 years, age at revision 13 +/- 4 years). The implant duration prior to revision was 4 +/- 3 years. Two leads required radiofrequency extraction sheaths for removal, two pulled back using a snare, while five leads were simply retracted and repositioned. Tricuspid regurgitation was none/trivial (three), mild (four), or moderate (two) and only two improved with repositioning or replacement. Pulmonary regurgitation preoperatively was mild (three), mild-moderate (two), or moderate (four) compared with trivial (three), mild (four), and moderate (two) after revision. Patients with longer-term implanted leads had less improvement in pulmonary insufficiency. Two patients had mild pulmonary stenosis from lead-related obstruction. CONCLUSIONS: Prolapse of transvenous pacing leads into the pulmonary artery can occur when excess slack is left for growth. Leads can often be repositioned, but may require extraction and replacement, particularly if chronically implanted and adherent to valve apparatus. Lead revision does not always resolve pulmonary insufficiency, potentially leaving permanent valve damage.

BACKGROUND: Early results for anatomic repair of congenitally corrected transposition of the great arteries are excellent with respect to right ventricular and tricuspid valve function. However, development of left ventricular (systemic ventricle) dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late impairment in left ventricular performance. METHODS: From August 1992 to July 2005, 44 patients (median age at surgery, 1.6 years; range, 0.6 to 39.6 years) with congenitally corrected transposition of the great arteries had anatomic repair. Left ventricular function and mitral regurgitation were evaluated by echocardiography at follow-up. Twenty-three patients had a Rastelli procedure, and 21 underwent an arterial switch. Twelve patients (27%) were pacemaker dependent at latest follow-up. RESULTS: Early mortality was 4.5% (n = 2) with 1 late death as a result of leukemia. Median follow-up was 3.0 years (range, 7 days to 12.4 years). Left ventricular function remained unchanged (normal) in 35 patients, improved in 1 patient, and deteriorated in 8 patients (18%). Mitral regurgitation was unchanged in 30 patients, improved in 6 patients, and worsened in 8 patients (18%). Development of left ventricular dysfunction was significantly associated with pacemaker implantation (p = 0.005) and a widened QRS (>20% > 98% percentile of normal; p = 0.03). CONCLUSIONS: Anatomic repair of congenitally corrected transposition can be performed with low operative mortality. However, late left ventricular dysfunction is not uncommon, with higher incidence in those requiring pacing and with a prolonged QRS. Resynchronization may be of value in patients requiring a pacemaker.

BACKGROUND: Venous occlusion is a recognized complication of transvenous pacing, and lead cross-sectional area indexed to body surface area (BSA) has been used to predict venous obstruction in children. OBJECTIVE: The aim of this study was to identify the risk factors and incidence of angiographic venous obstruction after transvenous lead implantation in both children and young adults. METHODS: Contrast venography was obtained in 85 of 90 consecutive patients undergoing repeat pacemaker or ICD procedures from 2002 to 2004 at a single cardiac center. Venograms were graded as complete venous obstruction, significant partial obstruction (>70% with collaterals), or patent. RESULTS: The cohort had a median age of 15.0 years at implant and was divided into two age groups: 3-12 years (n = 35) and 13 years and over (n = 50). After a median interval of 6.5 years, complete obstruction was seen in 11 of 85 patients (13%) and partial obstruction in another 10 patients (12%). No significant differences were seen in the incidence of obstruction between the two age groups although younger patients had a larger lead indexed to BSA ratio (6.82 vs 5.05, P = 0.005). There were no significant differences between obstructed and nonobstructed patients in relation to age, size, growth, or lead factors. CONCLUSION: Transvenous lead systems implanted in young children have a similar incidence of venous occlusion compared to older patients. Furthermore, patient age, body size, and lead characteristics at implant do not clearly predict venous occlusion.

INTRODUCTION: ECG algorithms used to localize accessory pathways (AP) in patients with Wolff-Parkinson-White (WPW) syndrome have been validated in adults, but less is known of their use in children, especially in patients with congenital heart disease (CHD). We hypothesize that these algorithms have low diagnostic accuracy in children and even lower in those with CHD. METHODS: Pre-excited ECGs in 43 patients with WPW and CHD (median age 5.4 years [0.9-32 years]) were evaluated and compared to 43 consecutive WPW control patients without CHD (median age 14.5 years [1.8-18 years]). Two blinded observers predicted AP location using 2 adult and 1 pediatric WPW algorithms, and a third blinded observer served as a tiebreaker. Predicted locations were compared with ablation-verified AP location to identify (a) exact match for AP location and (b) match for laterality (left-sided vs right-sided AP). RESULTS: In control children, adult algorithms were accurate in only 56% and 60%, while the pediatric algorithm was correct in 77%. In 19 patients with Ebstein's anomaly, diagnostic accuracy was similar to controls with at times an even better ability to predict laterality. In non-Ebstein's CHD, however, the algorithms were markedly worse (29% for the adult algorithms and 42% for the pediatric algorithms). A relatively large degree of interobserver variability was seen (kappa values from 0.30 to 0.58). CONCLUSIONS: Adult localization algorithms have poor diagnostic accuracy in young patients with and without CHD. Both adult and pediatric algorithms are particularly misleading in non-Ebstein's CHD patients and should be interpreted with caution.

BACKGROUND: Microvolt t-wave alternans (TWA) in early exercise is a noninvasive marker of life-threatening ventricular arrhythmia in some adult cardiac populations. The incidence and potential significance of sustained TWA in pediatric and congenital heart disease (CHD) populations has not been well defined. METHODS: TWA treadmill exercise studies in pediatric patients with CHD, myopathy, potential myocardial ischemia, syncope, or history of cardiac arrest were analyzed. Tests were categorized as abnormal for sustained TWA with onset heart rate < 130 beats/min with specific analyses for lower onset heart rates. Patient characteristics were analyzed as possible correlates of TWA. RESULTS: Over 2 years, 318 consecutive TWA exercise studies were performed in 304 patients (60% male, median 14 years, 6-41) for indications of syncope, cardiac arrest, possible ventricular arrhythmia, or evaluation of functional myocardial perfusion. Underlying conditions included apparently normal hearts (45%), CHD (16%), cardiomyopathy (11%), coronary anomalies (11%), electrical myopathy (9%), and transplant (8%). Abnormal TWA was seen in 24 (7%, onset HR 106 +/- 18) and included 19 at high clinical risk for serious events including 3 with cardiac arrest. By multivariate analysis sustained TWA was associated with cardiac arrest, ventricular arrhythmias, and a clinical classification of high risk. CONCLUSIONS: TWA is associated with pediatric and CHD diagnoses at high risk of serious events and may contribute, with other diagnostic tools, to management choices. While the absence of TWA has relatively high negative predictive value, it does not completely exclude the potential for serious sustained ventricular arrhythmias. A more robust noninvasive marker for risk stratification in these populations is required.

Lyme disease, caused by the spirochete Borrelia burgdorferi, has known cardiovascular effects typically manifesting in varying degrees of atrioventricular block. Three patients presented with QT interval prolongation associated with Lyme disease, a previously unreported manifestation of Lyme carditis. Implications and a proposed clinical management approach are discussed.

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.

BACKGROUND: Cryoablation may offer advantages over radiofrequency (RF) ablation for certain arrhythmia substrates, such as septal accessory pathways (APs). Data for young patients, especially regarding recurrence risk, require expansion. OBJECTIVES: The purpose of this study was to study institutional outcomes for cryoablation of APs located in potentially difficult septal regions for children and young adults. METHODS: Cryoablation was attempted in 35 young patients (mean age 15.6 years) with 37 APs that were either close to normal conduction tissues or inside the coronary venous system. Outcomes were compared with previously published institutional data for RF ablation at these same locations. RESULTS: Acute cryoablation success was achieved for 29 (78%) of 37 APs. Apart from permanent PR prolongation in one case and right bundle branch block in one other, there were no detrimental effects on normal conduction. At median follow-up of 207 days (range 2-695 days), AP conduction recurred for 13 (45%) of 29 ablated APs. Younger patient age and midseptal AP location correlated with higher likelihood of recurrence. Acute success rates for cryoablation were similar to RF ablation in our laboratory, but recurrence rates were significantly higher (P <.001). CONCLUSION: Cryoablation yields acute success rates comparable with RF ablation for difficult septal APs in young patients. The risk of AP recurrence appears higher after cryoablation, although safety benefits may provide suitable compensation for this deficiency. Methods for creating more effective cryoablation lesions need to be explored.

INTRODUCTION: In pediatric and congenital heart disease patients, transvenous ICD implantation may be limited secondary to patient size, venous, or cardiac anatomy. Epicardial patches require a thoracotomy, and may lead to a restrictive pericardial process. Because of these issues, we have explored novel ICD configurations. METHODS: Retrospective review at 10 centers implanting ICDs without a transvenous shocking coil or epicardial patches. RESULTS: Twenty-two patients underwent implant at a mean age of 8.9 years (range: 0.3-43.5), with a mean weight of 25.5 kg (range: 5.2-70). Diagnoses included complex CHD, intracardiac tumors, cardiomyopathy, idiopathic VT, LV noncompaction, and long QT syndrome. Three configurations were used: subcutaneous array, a transvenous design ICD lead placed on the epicardium, or a transvenous design ICD lead placed subcutaneously. Difficulties were found at implant in 8 patients: 4 had difficulty inducing VT/VF, and 4 had high DFTs. Over a mean follow-up of 2.2 years (range: 0.2-10.5), 7 patients had appropriate shocks. Inappropriate shocks occurred in 4 patients. System revisions were required in 7 patients: 2 generator changes (in 1 patient), 3 pace-sense lead replacement, 1 additional subcutaneous coil placement due to increased DFT, 1 upgrade to a transvenous system, and 1 revision to epicardial patch system. CONCLUSIONS: ICD implantation can be performed without epicardial patches or transvenous high-energy leads in this population, using individualized techniques. This will allow ICD use in patients who have intracardiac shunting or are deemed too small for transvenous ICD leads. The long-term outcome and possible complications are as yet unknown in this population, and they should be monitored closely with follow-up DFTs.