Publications by authors named "Leonardo Liberman"

63 Publications

Conduction Properties and Ablation of Adenosine Sensitive Accessory Pathways in Children.

Pediatr Cardiol 2021 Apr 23. Epub 2021 Apr 23.

Division of Pediatric Cardiology, Department of Pediatrics, Morgan Stanley Children's Hospital, New York Presbyterian Hospital, 3959 Broadway, 2 North, New York, NY, 10032, USA.

Block in accessory pathway (AP) conduction with adenosine has been previously described. However, conduction characteristics of these APs has not been well defined to date. All patients with APs 
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http://dx.doi.org/10.1007/s00246-021-02618-wDOI Listing
April 2021

Cardiac monitoring: is longer better?

Kardiol Pol 2021 02 25;79(2):110-111. Epub 2021 Feb 25.

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http://dx.doi.org/10.33963/KP.15825DOI Listing
February 2021

MIS-C and Cardiac Conduction Abnormalities.

Pediatrics 2020 12 12;146(6). Epub 2020 Nov 12.

Division of Pediatric Cardiology, NewYork-Presbyterian Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, New York

Objectives: Multisystem inflammatory syndrome in children (MIS-C) has spread through the pediatric population during the coronavirus disease 2019 pandemic. Our objective for the study was to report the prevalence of conduction anomalies in MIS-C and identify predictive factors for the conduction abnormalities.

Methods: We performed a single-center retrospective cohort study of pediatric patients <21 years of age presenting with MIS-C over a 1-month period. We collected clinical outcomes, laboratory findings, and diagnostic studies, including serial electrocardiograms, in all patients with MIS-C to identify those with first-degree atrioventricular block (AVB) during the acute phase and assess for predictive factors.

Results: Thirty-two patients met inclusion criteria. Median age at admission was 9 years. Six of 32 patients (19%) were found to have first-degree AVB, with a median longest PR interval of 225 milliseconds (interquartile range 200-302), compared with 140 milliseconds (interquartile range 80-178) in patients without first-degree AVB. The onset of AVB occurred at a median of 8 days after the initial symptoms and returned to normal 3 days thereafter. No patients developed advanced AVB, although 1 patient developed a PR interval >300 milliseconds. Another patient developed new-onset right bundle branch block, which resolved during hospitalization. Cardiac enzymes, inflammatory markers, and cardiac function were not associated with AVB development.

Conclusions: In our population, there is a 19% prevalence of first-degree AVB in patients with MIS-C. All patients with a prolonged PR interval recovered without progression to high-degree AVB. Patients admitted with MIS-C require close electrocardiogram monitoring during the acute phase.
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http://dx.doi.org/10.1542/peds.2020-009738DOI Listing
December 2020

Long-Term Patient Experience Following Acutely Successful Ablation of Supraventricular Tachycardia Substrate in Children.

Pediatr Cardiol 2021 Jan 26;42(1):109-115. Epub 2020 Sep 26.

Division of Pediatric Cardiology, Columbia University Medical Center, 3959 Broadway, North Building, 2nd Floor, New York, NY, 10032, USA.

Definitive treatment of supraventricular tachycardia (SVT) substrate involves catheter ablation. While objective success rates have been well established, long-term subjective patient experiences have not been well described. We quantify a subjective cure rate and characterize long-term patient experience after acutely successful ablation. A cross-sectional survey of pediatric patients with accessory pathways or atrioventricular nodal reentrant tachycardia who underwent acutely successful ablation from 2008 to 2012 was performed. Data were obtained from medical records and patient surveys. Patients with congenital heart disease other than patent ductus arteriosus, patent foramen ovale, or coronary artery abnormalities were excluded. Statistical analyses included Student's t-test and χ analysis for continuous and categorical variables, respectively. Surveys were sent to 153 patients of which 147 responded with median follow-up of 7.2 (IQR 6.1-8.5) years. Of the 147 responders, 124 (84%) patients reported cure with a male predominance. Symptoms were present in 130/147 (88%) patients pre-ablation and in 53/147 (36%) post-ablation. Among those with post-ablation symptoms, 50/53 (94%) reported symptomatic improvement. Recurrence occurred in 23/147 (16%) patients and was more prevalent following cryoablation. Ablation of SVT substrate can be curative with excellent long-term results and patient satisfaction. Long-term subjective cure rate is high and there is a substantial decrease in symptoms post-ablation. Many patients continue to have symptoms following ablation; however, the majority of these patients consider themselves cured and symptoms can be attributed to other etiologies. Recurrence is uncommon and occurs more frequently following cryoablation.
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http://dx.doi.org/10.1007/s00246-020-02459-zDOI Listing
January 2021

COVID-19 reveals Brugada pattern in an adolescent patient.

Cardiol Young 2020 Nov 3;30(11):1735-1737. Epub 2020 Aug 3.

Division of Paediatric Cardiology, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, New York, USA.

A diagnosis of Brugada pattern in paediatric or adolescent patients is rare. COVID-19 is characterised by fevers and a pro-inflammatory state, which may serve as inciting factors for Brugada pattern. Recently described in two adult patients, we report the first case of Brugada pattern in an adolescent with COVID-19.
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http://dx.doi.org/10.1017/S1047951120002619DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7450227PMC
November 2020

Cardiac workup and monitoring in hospitalised children with COVID- 19.

Cardiol Young 2020 Jul 2;30(7):907-910. Epub 2020 Jul 2.

Division of Pediatric Cardiology, Columbia University Irving Medical Center, New York, NY, USA.

Approximately, 1.7 million individuals in the United States have been infected with SARS-CoV-2, the virus responsible for the novel coronavirus disease-2019 (COVID-19). This has disproportionately impacted adults, but many children have been infected and hospitalised as well. To date, there is not much information published addressing the cardiac workup and monitoring of children with COVID-19. Here, we share the approach to the cardiac workup and monitoring utilised at a large congenital heart centre in New York City, the epicentre of the COVID-19 pandemic in the United States.
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http://dx.doi.org/10.1017/S1047951120001778DOI Listing
July 2020

Limited Utility of Repeated Vital Sign Monitoring During Initiation of Oral Propranolol for Complicated Infantile Hemangioma.

J Am Acad Dermatol 2020 Apr 11. Epub 2020 Apr 11.

Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Background: Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged.

Methods: A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of ≥0.3 mg/kg/dose, age <2 years, and heart rate (HR) monitoring for ≥1 hour. Data collected included demographics, dose, vital signs and adverse events.

Results: 783 subjects met inclusion criteria; median age at initiation was 112 days. None of the 1148 episodes of prolonged monitoring warranted immediate intervention or drug discontinuation. No symptomatic bradycardia or hypotension occurred during monitoring. Mean HR change from baseline to 1 hour was -8.19 +/- 15.54 and baseline to 2 hours was -9.24 +/- 15.84 bpm. Three preterm subjects had dose adjustments due to prescriber concerns about asymptomatic vital sign changes. No significant difference existed in pre-treatment HR or in HR change between those with later adverse events during treatment and those without.

Conclusion: Prolonged monitoring for initiation and escalation of oral propranolol rarely changed management and did not predict future adverse events. Few serious adverse events occurred during therapy; none were cardiovascular.
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http://dx.doi.org/10.1016/j.jaad.2020.04.013DOI Listing
April 2020

Impact of dipyridamole on adenosine dosing in pediatric and young adult patients after heart transplantation.

Pediatr Transplant 2020 05 10;24(3):e13689. Epub 2020 Mar 10.

Division of Pediatric Cardiology, Columbia University Irving Medical Center, New York, NY, USA.

Background: Relative contraindications to adenosine use have included heart transplant and dipyridamole. We previously demonstrated the safety and efficacy of adenosine-induced atrioventricular (AV) block in healthy young heart transplant recipients while suspending dipyridamole therapy (dual antiplatelet agent). This prospective follow-up study evaluated the safety and efficacy of adenosine use in the same cohort of heart transplant recipients while on dipyridamole.

Methods: Adenosine was incrementally dosed until AV block occurred (maximum 200 mcg/kg up to 12 mg). The primary outcome was clinically significant asystole (≥12 seconds). Secondary outcomes included maximal adenosine dose, AV block duration, dysrhythmias, and clinical symptoms. Outcomes were compared to the parent study.

Results: Thirty of 39 eligible patients (5-24 years) were tested. No patient (0%, CI 0%-8%) experienced clinically significant asystole. AV block occurred in 29/30 patients (97%, CI 86%-100%). The median dose causing AV block was 50mcg/kg (vs 100 mcg/kg off dipyridamole; P = .011). Seventeen patients (57%, CI 39%-72%) required less adenosine to achieve AV block on dipyridamole; six (20%) required more. AV block occurred at doses ≥25 mcg/kg in all patients. In pairwise comparison to prior testing off dipyridamole, no significant change occurred in AV block duration, frequency of cardiac ectopy, or incidence of reported symptoms. No atrial fibrillation/flutter occurred.

Conclusions: AV block often occurs at twofold lower adenosine doses in healthy young heart transplant recipients taking oral dipyridamole, compared with previous testing of this cohort off dipyridamole. Results suggest that initial dosing of 25 mcg/kg (maximum 0.8 mg) with stepwise escalation poses low risk of prolonged asystole on dipyridamole.
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http://dx.doi.org/10.1111/petr.13689DOI Listing
May 2020

Comparison of Holter With Zio Patch Electrocardiography Monitoring in Children.

Am J Cardiol 2020 03 11;125(5):767-771. Epub 2019 Dec 11.

Department of Pediatrics, Division of Pediatric Cardiology, Columbia University, College of Physicians and Surgeons, New York, New York. Electronic address:

The standard for ambulatory arrhythmia detection in children is the Holter monitor. The Zio XT (Zio) patch has been FDA-approved for use in adults. However, its utility in children has not been directly compared with the Holter. We studied the ability to detect arrhythmias and patient comfort of the Zio versus the Holter in children. Patients <22 years old were prospectively enrolled to wear the Holter and Zio simultaneously for 48 hours at our institution. Detection of clinically significant arrhythmias was compared using McNemar's test. Wear-time and artifact time was compared using Wilcoxon sign test. Patient satisfaction ratings were analyzed with paired t tests. Two hundred patients (57% male) were included for analysis. The median age was 13.5 years (range 23 days to 21.7 years), and 40% had heart disease. The Zio and Holter had comparable median wear-times, 48.2 hours (interquartile range [IQR] 45.8 to 50.2]) versus 48.0 (48.0 to 48.0), respectively, p = 0.14, but the Zio had less artifact than the Holter, 2.8% (IQR 1.1 to 8.6) versus 5.6% (2.4 to 15.7), respectively, p <0.001. There was no difference in detection of clinically significant arrhythmias for the Zio versus the Holter (p = 0.23), however 75% of patients preferred the Zio over the Holter (p <0.001) due to lack of wires and the ability to shower. In conclusion, the Zio patch is as good as the Holter monitor in detection of clinically significant arrhythmias in children with less artifact. Patients/parents more often preferred the Zio over the Holter.
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http://dx.doi.org/10.1016/j.amjcard.2019.11.028DOI Listing
March 2020

3D-rendered Electromechanical Wave Imaging for Localization of Accessory Pathways in Wolff-Parkinson-White Minors

Annu Int Conf IEEE Eng Med Biol Soc 2019 Jul;2019:6192-6195

Arrhythmia localization prior to catheter ablation is critical for clinical decision making and treatment planning. The current standard lies in 12-lead electrocardiogram (ECG) interpretation, but this method is non-specific and anatomically limited. Accurate localization requires intracardiac catheter mapping prior to ablation. Electromechanical Wave Imaging (EWI) is a high frame-rate ultrasound modality capable of non-invasively mapping the electromechanical activation in all cardiac chambers in vivo. In this study, we evaluate 3D-rendered EWI as a technique for consistently localizing the accessory pathway (AP) in Wolff-Parkinson-White (WPW) pediatric patients. A 2000 Hz EWI diverging sequence was used to transthoracically image 13 patients with evidence of ECG pre-excitation, immediately prior to catheter ablation and after successful ablation whenever possible. 3D-rendered activation maps were generated by co-registering and interpolating the 4 resulting multi-2D isochrones. A blinded electrophysiologist predicted the AP location on 12-lead ECG prior to ablation. Double-blinded EWI isochrones and clinician assessments were compared to the successful ablation site as confirmed by intracardiac mapping using a segmented template of the heart with 19 ventricular regions. 3D-rendered EWI was shown capable of consistently localizing AP in all the WPW cases. Clinical ECG interpretation correctly predicted the origin with an accuracy of 53.8%, respectively 84.6% when considering predictions in immediately adjacent segments correct. Our method was also capable of assessing the difference in activation pattern from before to after successful ablation on the same patient. These findings indicate that EWI could inform current diagnosis and expedite treatment planning of WPW ablation procedures.
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http://dx.doi.org/10.1109/EMBC.2019.8857876DOI Listing
July 2019

Impact of Obesity on Left Ventricular Thickness in Children with Hypertrophic Cardiomyopathy.

Pediatr Cardiol 2019 Aug 1;40(6):1253-1257. Epub 2019 Jul 1.

Montefiore Hospital, New York, NY, USA.

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients' weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18-50, mean 29.1) was present in 140 children aged 2-19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (p = 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.
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http://dx.doi.org/10.1007/s00246-019-02145-9DOI Listing
August 2019

Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: An international multicenter study.

Heart Rhythm 2019 10 23;16(10):1462-1467. Epub 2019 Apr 23.

Montefiore Hospital, New York, New York.

Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM).

Objective: The purpose of this study was to determine predictors of LAE in children with HCM.

Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions.

Results: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE.

Conclusion: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.
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http://dx.doi.org/10.1016/j.hrthm.2019.04.040DOI Listing
October 2019

Localization of Accessory Pathways in Pediatric Patients With Wolff-Parkinson-White Syndrome Using 3D-Rendered Electromechanical Wave Imaging.

JACC Clin Electrophysiol 2019 04 30;5(4):427-437. Epub 2019 Jan 30.

Ultrasound Elasticity Imaging Laboratory, Department of Biomedical Engineering, Columbia University, New York, New York; Department of Radiology, Columbia University Medical Center, New York, New York. Electronic address:

Objectives: This study sought to demonstrate the feasibility of electromechanical wave imaging (EWI) for localization of accessory pathways (AP) prior to catheter ablation in a pediatric population.

Background: Prediction of AP locations in patients with Wolff-Parkinson-White syndrome is currently based on analysis of 12-lead electrocardiography (ECG). In the pediatric population, specific algorithms have been developed to aid in localization, but these can be unreliable. EWI is a noninvasive imaging modality relying on a high frame rate ultrasound sequence capable of visualizing cardiac electromechanical activation.

Methods: Pediatric patients with ventricular pre-excitation presenting for catheter ablation were imaged with EWI immediately prior to the start of the procedure. Two clinical pediatric electrophysiologists predicted the location of the AP based on ECG. Both EWI and ECG predictions were blinded to the results of catheter ablation. EWI and ECG localizations were subsequently compared with the site of successful ablation.

Results: Fifteen patients were imaged with EWI. One patient was excluded for poor echocardiographic windows and the inability to image the entire ventricular myocardium. EWI correctly predicted the location of the AP in all 14 patients. ECG analysis correctly predicted 11 of 14 (78.6%) of the AP locations.

Conclusions: EWI was shown to be capable of consistently localizing accessory pathways. EWI predicted the site of successful ablation more frequently than analysis of 12-lead ECG. EWI isochrones also provide anatomical visualization of ventricular pre-excitation. These findings suggest that EWI can predict AP locations, and EWI may have the potential to better inform clinical electrophysiologists prior to catheter ablation procedures.
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http://dx.doi.org/10.1016/j.jacep.2018.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6478397PMC
April 2019

Advanced Heart Block in Children with Lyme Disease.

Pediatr Cardiol 2019 Mar 31;40(3):513-517. Epub 2018 Oct 31.

Department of Pediatrics, Division of Pediatric Cardiology, Columbia University, College of Physicians and Surgeons, 3959 Broadway, 2-North, New York, NY, USA.

Background: The clinical course of children with advanced heart block secondary to Lyme disease has not been well characterized.

Objective: To review the presentation, management, and time to resolution of heart block due to Lyme disease in previously healthy children.

Methods: An IRB approved single-center retrospective study was conducted of all patients < 21 years old with confirmed Lyme disease and advanced second or third degree heart block between 2007 and 2017.

Results: Twelve patients (100% male) with a mean age of 15.9 years (range 13.2-18.1) were identified. Six patients (50%) had mild to moderate atrioventricular valve regurgitation and all had normal biventricular function. Five patients had advanced second degree heart block and 7 had complete heart block with an escape rate of 20-57 bpm. Isoproterenol was used in 4 patients for 3-4 days and one patient required transvenous pacing for 2 days. Patients were treated with 21 days (n = 6, 50%) or 28 days (n = 6, 50%) of antibiotics. Three patients received steroids for 3-4 days. Advanced heart block resolved in all patients within 2-5 days, and all had a normal PR interval within 3 days to 16 months from hospital discharge.

Conclusion: Symptomatic children who present with new high-grade heart block from an endemic area should be tested for Lyme disease. Antibiotic therapy provides quick and complete resolution of advanced heart block within 5 days, while steroids did not appear to shorten the time course in this case series. Importantly, no patients required a permanent pacemaker.
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http://dx.doi.org/10.1007/s00246-018-2003-8DOI Listing
March 2019

SCN5A mutations in 442 neonates and children: genotype-phenotype correlation and identification of higher-risk subgroups.

Eur Heart J 2018 08;39(31):2879-2887

L'institut du thorax, INSERM, CNRS, UNIV Nantes, CHU Nantes, Nantes, France.

Aims: To clarify the clinical characteristics and outcomes of children with SCN5A-mediated disease and to improve their risk stratification.

Methods And Results: A multicentre, international, retrospective cohort study was conducted in 25 tertiary hospitals in 13 countries between 1990 and 2015. All patients ≤16 years of age diagnosed with a genetically confirmed SCN5A mutation were included in the analysis. There was no restriction made based on their clinical diagnosis. A total of 442 children {55.7% boys, 40.3% probands, median age: 8.0 [interquartile range (IQR) 9.5] years} from 350 families were included; 67.9% were asymptomatic at diagnosis. Four main phenotypes were identified: isolated progressive cardiac conduction disorders (25.6%), overlap phenotype (15.6%), isolated long QT syndrome type 3 (10.6%), and isolated Brugada syndrome type 1 (1.8%); 44.3% had a negative electrocardiogram phenotype. During a median follow-up of 5.9 (IQR 5.9) years, 272 cardiac events (CEs) occurred in 139 (31.5%) patients. Patients whose mutation localized in the C-terminus had a lower risk. Compound genotype, both gain- and loss-of-function SCN5A mutation, age ≤1 year at diagnosis in probands and age ≤1 year at diagnosis in non-probands were independent predictors of CE.

Conclusion: In this large paediatric cohort of SCN5A mutation-positive subjects, cardiac conduction disorders were the most prevalent phenotype; CEs occurred in about one-third of genotype-positive children, and several independent risk factors were identified, including age ≤1 year at diagnosis, compound mutation, and mutation with both gain- and loss-of-function.
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http://dx.doi.org/10.1093/eurheartj/ehy412DOI Listing
August 2018

Usefulness of High-Dose Oral Flecainide for Termination of Recent-Onset Atrial Fibrillation in Children.

Am J Cardiol 2018 06 21;121(12):1530-1533. Epub 2018 Mar 21.

Department of Pediatrics, Division of Pediatric Cardiology, Columbia University Medical Center, New York, New York.

A high dose of oral flecainide has been used for acute termination of atrial fibrillation (AF) and atrial flutter or intra-atrial re-entry tachycardia (AFL-IART) in adults. The use of flecainide for these conditions in children has not been well described. We describe our institutional experience on acute termination of AF or AFL-IART in children with a single high dose of oral flecainide in a hospital setting. All patients who received a single high dose of oral flecainide from 2009 to 2016 who were <21 years of age were included. Patients were treated only if AF or AFL-IART was less than 24 hours of duration. The dose was 300 mg for patients >70 kg, 200 mg for patients 40 to 70 kg, and 5 mg/kg for patients <40 kg. Charts were reviewed to determine demographic information, flecainide dose, termination of arrhythmia, and time to termination. There were 22 patients identified. The median age was 16 years (range 4.6 to 20.3) with a median weight of 75 kg (range 19 to 112). There were 13 patients with AF (11 with a normal heart, 85%) and 9 patients with AFL-IART (1 with a normal heart, 11%) (p <0.05). The median dose of flecainide given was 3.6 mg/kg (range 2.7 to 6.1) or 136 mg/m (range 90 to 171). AF in all patients (13/13, 100%) and AFL-IART in 5 of 9 patients (55%) terminated acutely (p <0.05). All patients with normal heart (12/12, 100%) and 6 of the 10 patients (60%) with heart disease have their arrhythmia terminated acutely (p <0.05). The only patients whose tachycardia did not terminate were 4 patients with IART and heart disease. The arrhythmia terminated in a median time of 60 minutes (range 30 to 120). There were no adverse events or proarrhythmia encountered. In conclusion, a single high dose of oral flecainide successfully terminated AF of less than 24 hours' duration in all pediatric patients without side effects. This approach is less effective for AFL-IART in patients with heart disease.
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http://dx.doi.org/10.1016/j.amjcard.2018.03.001DOI Listing
June 2018

The "hidden" concealed left-sided accessory pathway: An uncommon cause of SVT in young people.

Pacing Clin Electrophysiol 2018 04 23;41(4):368-371. Epub 2018 Feb 23.

Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA, USA.

Background: Concealed left-sided accessory pathways (CLAP) are a cause of supraventricular tachycardia (SVT) in the young. Most are mapped with right ventricular (RV) apical/outflow pacing. Rarely, alternative means of mapping are required. We review our experience from three pediatric electrophysiology (EP) centers with a rare form of "hidden" CLAP.

Methods: All patients <21 years undergoing EP study from 2008 to 2014 with a "hidden" CLAP (defined as an accessory pathway [AP] for which RV pacing at cycle lengths [CL] stable for mapping did not demonstrate eccentric retrograde conduction) were included.

Exclusion Criteria: preexcitation. Demographic, procedural, and follow-up data were collected.

Results: A total of 23 patients met the criteria (median age, 14.3 years [range 7-21], weight, 51 kg [31-99]). 21 (96%) had SVT and one AFIB (4%). APs were adenosine sensitive in 7/20 patients (35%) and VA conduction was decremental in six (26%). CLAP conduction was demonstrable with orthodromic reentrant tachycardia in all patients, with RV extrastimulus testing in seven (30%) and with rapid RV pacing (
Conclusions: Some CLAPs are only demonstrable with LV pacing, entrained ORT, or rapid RV pacing. LV pacing facilitated preferential AP conduction, allowing for mapping while maintaining stable hemodynamics.
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http://dx.doi.org/10.1111/pace.13279DOI Listing
April 2018

Prospective Study of Adenosine on Atrioventricular Nodal Conduction in Pediatric and Young Adult Patients After Heart Transplantation.

Circulation 2017 Jun 27;135(25):2485-2493. Epub 2017 Apr 27.

From Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY.

Background: Supraventricular tachycardia is common after heart transplantation. Adenosine, the standard therapy for treating supraventricular tachycardia in children and adults without transplantation, is relatively contraindicated after transplantation because of a presumed risk of prolonged atrioventricular block in denervated hearts. This study tested whether adenosine caused prolonged asystole after transplantation and if it was effective in blocking atrioventricular nodal conduction in these patients.

Methods: This was a single-center prospective clinical study including healthy heart transplant recipients 6 months to 25 years of age presenting for routine cardiac catheterization during 2015 to 2016. After catheterization, a transvenous pacing catheter was placed and adenosine was given following a dose-escalation protocol until atrioventricular block was achieved. The incidence of clinically significant asystole (≥12 seconds after adenosine) was quantified. The effects of patient characteristics on adenosine dose required to produce atrioventricular block and duration of effect were also measured.

Results: Eighty patients completed adenosine testing. No patient (0%; 95% confidence interval, 0-3) required rescue ventricular pacing. Atrioventricular block was observed in 77 patients (96%; 95% confidence interval, 89-99). The median longest atrioventricular block was 1.9 seconds (interquartile range, 1.4-3.2 seconds), with a mean duration of adenosine effect of 4.3±2.0 seconds. No patient characteristic significantly predicted the adenosine dose to produce atrioventricular block or duration of effect. Results were similar across patient weight categories.

Conclusions: Adenosine induces atrioventricular block in healthy pediatric and young adult heart transplant recipients with minimal risk when low initial doses are used (25 μg/kg; 1.5 mg if ≥60 kg) and therapy is gradually escalated.

Clinical Trial Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT02462941.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.028087DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5539538PMC
June 2017

Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.

Cardiol Young 2017 May 21;27(4):804-807. Epub 2016 Nov 21.

Department of Pediatrics,Division of Pediatric Cardiology,New York-Presbyterian Morgan Stanley Children's Hospital,Columbia University Medical Center, New York,New York,United States of America.

Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.
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http://dx.doi.org/10.1017/S1047951116002055DOI Listing
May 2017

Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.

Eur J Pediatr 2016 Sep 28;175(9):1235-1248. Epub 2016 Jun 28.

Mayo Clinic, Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, MN, USA.

Unlabelled: Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities.

Conclusion: Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block.

What Is Known: • Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death. • Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases. What is New: • Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders. • Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy.
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http://dx.doi.org/10.1007/s00431-016-2748-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5005411PMC
September 2016

Incidence and characteristics of heart block after heart surgery in pediatric patients: A multicenter study.

J Thorac Cardiovasc Surg 2016 Jul 7;152(1):197-202. Epub 2016 May 7.

Division of Pediatric Cardiology, Department of Pediatrics, New York-Presbyterian/Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, NY.

Background: Advanced second- or third-degree heart block has been reported with variable incidence after surgery for congenital heart disease in children. We report the incidence of heart block requiring a pacemaker and describe the risk factors for this complication in a large multicenter study.

Methods: We performed a retrospective cohort study, using the Pediatric Health Information System database from 45 hospitals in the United States, for all children aged 18 years, discharged between January 1, 2004, and December 31, 2013, who underwent open surgery for congenital heart disease. Patients who had heart block and placement of a pacemaker during the same hospitalization were identified. Demographic characteristics, procedure and diagnostic codes, length of stay, and mortality were analyzed. Univariable and multivariable analyses were performed.

Results: There were 101,006 surgeries performed. The median age of patients was 0.5 years (interquartile range, 26 days to 3.2 years), and 1% of patients (n = 990) had heart block and placement of a pacemaker. Surgeries associated with the highest incidences of heart block and placement of a pacemaker included the double switch operation (15.6%), tricuspid valve (7.8%) and mitral valve (7.4%) replacement, atrial switch with ventricular septal defect repair (6.4%), and Rastelli operation (4.8%). On multivariable analysis, after controlling for surgical complexity, other comorbidities, age at surgery, admission year, and clustering by institution, patients with heart block and placement of a pacemaker had higher odds of mortality (odds ratio, 1.67; 95% confidence interval, 1.24-2.26; P < .001).

Conclusions: The incidence of postoperative heart block requiring permanent pacemaker placement immediately after congenital heart surgery is low (1%). However, these patients have higher mortality even after adjusting for heart surgery complexity.
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http://dx.doi.org/10.1016/j.jtcvs.2016.03.081DOI Listing
July 2016

Revisiting Wolff-Parkinson-White risk stratification: a malignant arrhythmia in a patient with intermittent pre-excitation.

Cardiol Young 2016 Apr 21;26(4):781-3. Epub 2015 Sep 21.

1Department of Pediatrics,Division of Pediatric Cardiology,New York-Presbyterian Morgan Stanley Children's Hospital,Columbia University Medical Center,New York,New York,United States of America.

It has been reported that the presence of intermittent pre-excitation indicates low risk of rapid conduction via the accessory pathway in atrial fibrillation. We report a case of a 10-year-old boy with a history of intermittent pre-excitation who presented with atrial fibrillation with very rapid conduction.
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http://dx.doi.org/10.1017/S1047951115001754DOI Listing
April 2016

A Smartphone Application to Diagnose the Mechanism of Pediatric Supraventricular Tachycardia.

Pediatr Cardiol 2015 Oct 10;36(7):1452-7. Epub 2015 May 10.

Division of Pediatric Cardiology, Department of Pediatrics, College of Physicians and Surgeons, Columbia University Medical Center, 3959 Broadway, 2-North, New York, NY, 10032, USA.

Smartphone applications that record a single-lead ECG are increasingly available. We sought to determine the utility of a smartphone application (AliveCor) to record supraventricular tachycardia (SVT) and to distinguish atrioventricular reentrant tachycardia (AVRT) from atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients. A prior study demonstrated that interpretation of standard event and Holter monitors accurately identifies the tachycardia mechanism in only 45 % of recordings. We performed an IRB-approved prospective study in pediatric patients undergoing an ablation for SVT. Tracings were obtained by placing the smartphone in three different positions on the chest (PI-horizontal, PII-rotated 60° clockwise, and PIII-rotated 120° clockwise). Two blinded pediatric electrophysiologists jointly analyzed a pair of sinus and tachycardia tracings in each position. Tracings with visible retrograde P waves were classified as AVRT. The three positions were compared by Chi-square test. Thirty-seven patients (age 13.7 ± 2.8 years) were enrolled in the study. Twenty-four had AVRT, and 13 had AVNRT. One hundred and eight pairs of tracings were obtained. The correct diagnosis was made in 27/37 (73 %) with position PI, 28/37 (76 %) with PII, and 20/34 (59 %) with PIII (p = 0.04 for PII vs. PIII and p = NS for other comparisons). A single-lead ECG obtained with a smartphone monitor can successfully record SVT in pediatric patients and can predict the SVT mechanism at least as well as previously published reports of Holter monitors, along with the added convenience of not requiring patients to carry a dedicated monitor.
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http://dx.doi.org/10.1007/s00246-015-1185-6DOI Listing
October 2015

Implantation of permanent pacemaker and ICD leads in children using a three-dimensional electroanatomic mapping system as an aid to fluoroscopy.

Pacing Clin Electrophysiol 2015 Apr 5;38(4):448-54. Epub 2015 Feb 5.

Division of Pediatric Cardiology, Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York.

Background: Three-dimensional electroanatomic mapping systems (3D mapping) allow the creation of an anatomic representation of the cardiac anatomy and real-time monitoring of transvenous pacing catheters. Their use to facilitate pacemaker (PM) and implantable cardioverter defibrillator (ICD) lead placement in the pediatric population has not been well defined. We sought to review our experience using 3D mapping as an adjunct to fluoroscopy for positioning of permanent PM and ICD leads in pediatric patients.

Methods: We performed a retrospective review of all patients ≤21 years of age who underwent PM or ICD implantation with the aid of the Ensite Velocity system (Ensite) from May 2012 to September 2014. Fluoroscopy was utilized in addition to 3D mapping to aid obtaining vascular access, to visualize fixation of the lead to the myocardium, and to evaluate lead length within the vasculature.

Results: Nineteen patients (mean age 14.6 ± 4.4 years) underwent placement of active fixation PM leads (N = 17) and/or ICD leads (N = 10) with the aid of Ensite. All leads were successfully placed (eight atrial, 19 ventricular). Fluoroscopic exposure was a mean of 3.2 minutes (± 2.8, 0.1-10.5). There was one lead dislodgement.

Conclusions: Ensite was used successfully as an adjunct to fluoroscopy in all patients in whom it was attempted to aid the placement of permanent transvenous PM and ICD leads. Future evaluation could focus on the use of 3D mapping systems to further minimize or eliminate fluoroscopy from PM and ICD implantations.
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http://dx.doi.org/10.1111/pace.12579DOI Listing
April 2015

Cryoablation of anteroseptal accessory pathways with a his bundle electrogram on the ablation catheter.

Indian Pacing Electrophysiol J 2014 Nov-Dec;14(6):284-90. Epub 2014 Dec 5.

Pediatric Arrhythmia Service, Department of Pediatrics, Morgan Stanley Children Hospital, Columbia University, New York, New York.

Background: Radiofrequency catheter ablations of anteroseptal (AS) accessory pathways (AP) in pediatric patients have higher incidence of atrioventricular (AV) block than other AP locations. We report our experience using cryoablation in pediatric patients where a His bundle electrogram was noted on the ablation catheter at the site of the successful ablation.

Methods And Results: We retrospectively reviewed all patients ≤21 years that underwent cryoablation for an AS AP from 2005 to 2012 at our institution (n=70). Patients with a His bundle electrogram noted on the cryoablation catheter at the location of the successful lesion were identified (n=6, 8.5%). All six patients had ventricular preexcitation. Median age of 15.9 years (7.2 - 18.2). AV nodal function was monitored during the cryoablation with intermittent rapid atrial pacing conducted through the AV node (n=2), with atrial extra-stimulus testing (n=2), or during orthodromic reentrant tachycardia (n=2). Acute success occurred in all patients. Two patients had early recurrence of AP conduction. Both patients underwent a second successful cryoablation, again with a His bundle electrogram on the cryoablation catheter. At a median follow-up of 13 months (3 to 37 months) there was no recurrence of accessory pathway conduction and AVN function was normal.

Conclusion: In a small number of pediatric patients with AS AP with a His bundle electrogram seen on the ablation catheter, the use of cryotherapy was safe and effective for elimination of AP conduction without impairment of AV nodal conduction.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4286952PMC
http://dx.doi.org/10.1016/s0972-6292(16)30816-6DOI Listing
January 2015

Defining the electrocardiogram in the neonate with hypoplastic left heart syndrome.

Pediatr Cardiol 2015 Jun 21;36(5):1014-8. Epub 2015 Jan 21.

Division of Cardiology, Department of Pediatrics, Morgan Stanley Children's Hospital, Columbia University College of Physicians and Surgeons, 3959 Broadway, CH-2 North, New York, NY, 10032-3784, USA.

Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease characterized by underdevelopment of the left heart. There has been no previously defined "classic" ECG pattern in a large homogenous population of patients with HLHS. We performed a retrospective review of ECGs from neonates with HLHS from 2001 to 2011 with electrocardiograms available prior to surgical intervention. Eighty-nine neonates met the inclusion criteria and were compared to a control population. HLHS patients had a longer PR interval 108 ± 18 versus 98 ± 11 ms (p < 0.05), a wider QRS complex 84 ± 17 versus 54 ± 5 ms (p < 0.05), lower voltage S waves in V1 2.0 ± 3.3 versus 5.8 ± 4.6 mm (p < 0.001) or absent S waves in V1 52 versus 4 % (p < 0.001) and lower voltage R waves in V6 7.8 ± 4.8 versus 9.3 ± 4.2 mm (p < 0.05). Patients with HLHS were more likely to have absent Q waves in the lateral precordial leads 78 versus 0 % (p < 0.001) and inferior leads 20 versus 1 % (p < 0.001) and an abnormal frontal plane QRS axis 26 versus 11 % (p < 0.05). HLHS patients were more likely to have a preexcited appearance 11 versus 0 % (p = 0.001). Despite these findings, 20 % of patients with HLHS had a normal ECG. Compared to age-matched controls, patients with HLHS were more likely to exhibit a longer PR interval, a wider QRS complex, decreased left-sided forces, an absence of septal Q waves in the inferior and lateral leads, an abnormal frontal plane QRS axis and a preexcited appearance.
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http://dx.doi.org/10.1007/s00246-015-1112-xDOI Listing
June 2015

QT prolongation and torsades de pointes in a patient with heart block and a pacemaker.

Cardiol Young 2016 Jan 20;26(1):161-3. Epub 2015 Jan 20.

Pediatric Electrophysiology,Department of Pediatrics,Morgan Stanley Children's Hospital of New York Presbyterian,New York,New York,United States of America.

Prolongation of the QT interval and development of torsades de pointes are known in patients with complete heart block and profound bradycardia. We report the case of a patient with complete heart block and torsades, with long QT seen during a period of junctional tachycardia at a rate faster than the minimum pacemaker rate.
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http://dx.doi.org/10.1017/S1047951114002674DOI Listing
January 2016

Post-pericardiotomy syndrome in pediatric patients following surgical closure of secundum atrial septal defects: incidence and risk factors.

Pediatr Cardiol 2015 Mar 8;36(3):498-502. Epub 2014 Oct 8.

Department of Pediatric Cardiology, Morgan Stanley Children's Hospital, 3959 Broadway, 2 North, New York, NY, 10032, USA,

Surgical repair for atrial septal defects (ASD) generally occurs during childhood. Post-pericardiotomy syndrome (PPS) after cardiac surgery has a reported incidence of 1-40 %. We focused exclusively on secundum ASD repair to evaluate the incidence of PPS. The purpose of this study is to determine the incidence of PPS after surgical repair of secundum ASD and investigate what risk factors may be predictive of its development. A retrospective study was performed, and 97 patients who underwent surgical closure of a secundum ASD were identified. 27 (28 %) were diagnosed with PPS within the first postoperative year. Diagnosis was made if they had evidence of new or worsening pericardial effusion and the presence of ≥2 of the following criteria: fever >72 h postoperatively, irritability, pleuritic chest pain, or pericardial friction rub. Closure of secundum ASDs was performed at a median age of 3.8 years (Interquartile Range (IQR): 2.2-6.0 years) and a median weight of 14.3 kilograms (IQR: 10.9-19.3 kilograms). The median time for development of PPS was 8 days post-op (IQR: 5-14). Significantly, 19 (27 %) of 70 patients in the non-PPS group had a small pericardial effusion on their discharge echocardiogram, while of the 27 patients who developed PPS, 17 (63 %) had a small pericardial effusion on their discharge echocardiogram (p = 0.001). PPS is relatively common following surgical closure of secundum ASDs. A small pericardial effusion on discharge echocardiogram is predictive of development of PPS postoperatively. In patients who develop PPS, there is a good response to therapy with a benign course.
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http://dx.doi.org/10.1007/s00246-014-1039-7DOI Listing
March 2015

Usefulness of arrhythmias as predictors of death and resource utilization in children with myocarditis.

Am J Cardiol 2014 Nov 12;114(9):1400-5. Epub 2014 Aug 12.

Division of Pediatric Cardiology, NewYork-Presbyterian/Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, New York.

Myocarditis in children can result in significant morbidity and mortality, yet limited prognostic data exist. The aim of this study was to test the hypothesis that pediatric patients with arrhythmias during hospitalization for acute myocarditis have worse outcomes and increased resource utilization. A retrospective study using the Pediatric Health Information System database was performed to examine the effects of clinically significant arrhythmias on in-hospital mortality, length of stay, and costs per day. Data were obtained for children ≤18 years of age, discharged from January 1, 2004 to March 31, 2013, with a diagnosis of myocarditis. Clinically significant tachyarrhythmia was defined as supraventricular tachycardia, atrial fibrillation or flutter, or ventricular tachycardia or fibrillation in patients receiving antiarrhythmic medications or cardioversion. Clinically significant bradyarrhythmia was defined as second-degree, complete, or other heart block for which a pacemaker was placed. Multivariable analyses were performed. A total of 2,041 subjects with myocarditis were identified. Tachyarrhythmias were reported in 234 (11.5%) and bradyarrhythmias in 22 (1.1%). Overall mortality was 8.7%. In multivariable analyses, after considering the effects of gender, age at admission, geographic region, year and month of admission, presence of congenital heart disease or an identified virus, and use of steroids, nonsteroidal anti-inflammatories, or inotropes, and after controlling for clustering by institution, tachyarrhythmias were associated with a 2.3 times increase in the odds of mortality (95% confidence interval 1.6 to 3.3, p < 0.001), a 58% increase in length of stay (95% confidence interval 38% to 82%, p < 0.001), and a 28% increase in costs per day (95% confidence interval 15% to 43%, p < 0.001). Bradyarrhythmia was not associated with mortality, length of stay, or costs per day. In conclusion, tachyarrhythmias are associated with significant increases in mortality and resource utilization in children with myocarditis.
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http://dx.doi.org/10.1016/j.amjcard.2014.07.074DOI Listing
November 2014

Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly: a 21-year experience.

Circulation 2013 Sep;128(11 Suppl 1):S194-8

Departments of Pediatric Cardiology (D.A.H., M.E.R., J.S.G., L.L.) and Pediatric Cardiothoracic Surgery (S.J., J.M.Q., J.M.C., E.B.), Morgan Stanley Children's Hospital and Mailman School of Public Health (H.F.A.), Columbia University, New York, NY.

Background: Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly.

Methods And Results: A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (± 6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reintervention was 73% at 1 year, 64% at 5 years, and 60% at 10 years. Eleven patients underwent 13 catheter reinterventions on the pulmonary arteries (n=8) or aortic arch (n=5). Seven patients underwent 11 reoperations, including relief of right ventricular outflow tract obstruction (n=5), pulmonary arterioplasty (n=3), recoarctation repair (n=2), and tricuspid valve repair (n=1). By multivariate analysis, a preoperative aortic valve annulus z score of ≤-2.5 was associated with reintervention (hazard ratio, 7.66 [95% confidence interval, 1.29-45.6], P=0.03).

Conclusions: Although reintervention is common, primary correction of Taussig-Bing anomaly with arterial switch operation can be achieved in all patients with low mortality and good long-term outcomes.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.112.000061DOI Listing
September 2013