Search

BackgroundRight ventricular ejection fraction (RVEF) and end-diastolic volume (RVEDV) are not readily assessed through traditional modalities. Deep-learning enabled 12-lead electrocardiogram analysis (DL-ECG) for estimation of RV size or function is unexplored.MethodsWe trained a DL-ECG model to predict RV dilation (RVEDV>120 mL/m2), RV dysfunction (RVEF<40%), and numerical RVEDV/RVEF from 12-lead ECG paired with reference-standard cardiac MRI (cMRI) volumetric measurements in UK biobank (UKBB; n=42,938). We fine-tuned in a multi-center health system (MSHoriginal; n=3,019) with prospective validation over 4 months (MSHvalidation; n=115). We evaluated performance using area under the receiver operating curve (AUROC) for categorical and mean absolute error (MAE) for continuous measures overall and in key subgroups. We assessed association of RVEF prediction with transplant-free survival with Cox proportional hazards models.ResultsPrevalence of RV dysfunction for UKBB/MSHoriginal/MSHvalidationcohorts was 1.0%/18.0%/15.7%, respectively. RV dysfunction model AUROC for UKBB/MSHoriginal/MSHvalidationcohorts was 0.86/0.81/0.77, respectively. Prevalence of RV dilation for UKBB/MSHoriginal/MSHvalidationcohorts was 1.6%/10.6%/4.3%. RV dilation model AUROC for UKBB/MSHoriginal/MSHvalidationcohorts 0.91/0.81/0.92, respectively. MSHoriginalMAE was RVEF=7.8% and RVEDV=17.6 ml/m2. Performance was similar in key subgroups including with and without left ventricular dysfunction. Over median follow-up of 2.3 years, predicted RVEF was independently associated with composite outcome (HR 1.37 for each 10% decrease, p=0.046).ConclusionsDL-ECG analysis can accurately identify significant RV dysfunction and dilation both overall and in key subgroups. Predicted RVEF is independently associated with clinical outcome.

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed. Optimal care for ACHDs therefore requires a heightened awareness of the relevant interactions as well as the pharmacologic and interventional resources that are increasingly available to the treating cardiologist. This review explores and highlights the overlap between these 2 fields to recommend a parallel, yet interactive, multidisciplinary approach to clinical management. Congenital heart disease categories are broken down into their archetypal subtypes to highlight subtleties of the pathophysiology, evaluation, and therapeutic approach.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns. Methods: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands. Results: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia. Conclusions: ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

We describe a rare case of spontaneous coronary artery thrombosis in a newborn leading to rapid severe ventricular dysfunction. Early diagnosis is critical and management strategies are varied including hemodynamic support with extracorporeal membrane oxygenation, systemic/local thrombolytic therapy with tissue plasminogen activator, or surgical thrombectomy. (Level of Difficulty: Advanced.), Graphical abstract

Success rates for catheter ablation of supraventricular tachycardia (SVT) in the young exceed 90%. While studies have described reasons for initial ablation failure, less is known about outcomes of repeat ablation attempts. The purpose of this study was to report acute and mid-term success rates for second ablation attempts in young patients, as well as to analyze factors that may affect these outcomes. Retrospective single-center study of all patients undergoing a second ablation attempt for WPW (Wolff-Parkinson-White) or SVT from 2008 to 2017. Inclusion criteria are all patients

Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown aetiology. We aimed to better understand familial recurrence pattern. Methods An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, leading to investigate 1043 unrelated ccTGA probands. Results Atrioventricular block at diagnosis and laterality defects were observed in 35.4% and 29.9%, respectively. ccTGA associated with primary ciliary dyskinesia in 10 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (d-TGA: 28.4%), laterality defects (13.6%) and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and d-TGA and 9 familial co-segregation of ccTGA and laterality defects. In one family there was co-segregation of ccTGA, d-TGA and heterotaxy syndrome in 3 distinct relatives. Conclusion ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, d-TGA, laterality defects and in some cases primary ciliary dyskinesia strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Introduction: Paramitral leak (PML) has been reported in 5-17% of patients after surgical prosthetic mitral valve replacements. Transcatheter closure of PML has emerged as a Class IIa alternative to surgical repair by the AHA/ACC. Reaching all areas around the mitral annulus for device delivery from a transseptal approach can be difficult. We describe our center’s experience utilizing a transseptal approach with use of a steerable sheath and real-time 3D echo to facilitate closure. Methods: Retrospective review of cases of transcatheter PML repair from 9/09 to 2/19 at Mount Sinai Medical Center. Results: 32 patients underwent 43 catheterizations for PML closure (see Table 1). Indications for PML repair were CHF in (78%) and hemolysis (22%). 25/32 were bioprosthetic valves and the rest mechanical (7/32; table). PML’s could be crossed in 41/43 attempts (95%). Location of leaks/devices demonstrated in Figure 1. The median number of devices placed at one cath was 1 (range 1-3) and the most commonly used device was the Amplatzer muscular VSD device (64/71devices; 90%). 9/32 (28%) required an additional cath to complete PML closure. At last cath, clinical PML closure was achieved in 26/32 (81%) pts attempted. 3/32 (9%) had hemolysis from residual PML after closure and required surgical mitral valve replacement. Aside from failed attempts or those requiring repeat cath, there were no serious complications. Conclusions: Use of the Agilis™ NxT Steerable Sheath allowed closure of the majority of PML encountered. Success rates were high though a large percentage required additional caths to achieve success. Serious complications were uncommon.

Introduction: The transition from residency to subspecialty fellowship in pediatric cardiology is challenging, with the daunting requirement to rapidly acquire a broad scope of knowledge and skill. In 2015, a pediatric cardiology boot camp was designed as an educational tool to help prepare trainees for this transition. Hypothesis: A national pediatric cardiology boot camp consistently improves knowledge and decreases anxiety for prospective fellows. Methods: In late spring each year (2015-2019), a 2.5-day intensive program was provided for trainees prior to beginning fellowship in July. Hands-on, simulation-based experiences were provided on topics including anatomy, auscultation, echocardiography, catheterization, cardiovascular intensive care, electrophysiology, heart failure, pulmonary hypertension, and cardiac surgery. Knowledge based exams and surveys were completed by each participant pre- and post-training. Pre- and post-training exam results were compared via paired t-tests and survey results were compared via Wilcoxon rank sum. Results: Over 5 years 144 participants (72 female, 50%) completed the course, representing 40 fellowship programs in the United States and Canada. In aggregate, significant improvement was seen in participants’ knowledge assessment (pre 45 ± 11% vs. post 71 ± 9%; p Conclusions: The Pediatric Cardiology Boot Camp provides a significant and reproducible educational benefit to participants nationwide. This intensive program simultaneously improves learners’ knowledge and alleviates anxiety as they transition to subspecialty training.

Objective To assess clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2-associated multisystem inflammatory syndrome in children (MIS-C). Study design Children with MIS-C admitted to pediatric intensive care units in New York City between April 23 and May 23, 2020, were included. Demographic and clinical data were collected. Results Of 33 children with MIS-C, the median age was 10 years; 61% were male; 45% were Hispanic/Latino; and 39% were black. Comorbidities were present in 45%. Fever (93%) and vomiting (69%) were the most common presenting symptoms. Depressed left ventricular ejection fraction was found in 63% of patients with median ejection fraction of 46.6% (IQR, 39.5-52.8). C-reactive protein, procalcitonin, d-dimer, and pro-B-type natriuretic peptide levels were elevated in all patients. For treatment, intravenous immunoglobulin was used in 18 (54%), corticosteroids in 17 (51%), tocilizumab in 12 (36%), remdesivir in 7 (21%), vasopressors in 17 (51%), mechanical ventilation in 5 (15%), extracorporeal membrane oxygenation in 1 (3%), and intra-aortic balloon pump in 1 (3%). The left ventricular ejection fraction normalized in 95% of those with a depressed ejection fraction. All patients were discharged home with median duration of pediatric intensive care unit stay of 4.7 days (IQR, 4-8 days) and a hospital stay of 7.8 days (IQR, 6.0-10.1 days). One patient (3%) died after withdrawal of care secondary to stroke while on extracorporeal membrane oxygenation. Conclusions Critically ill children with coronavirus disease-2019-associated MIS-C have a spectrum of severity broader than described previously but still require careful supportive intensive care. Rapid, complete clinical and myocardial recovery was almost universal.

Background Axillary venous access with ultrasound guidance for pediatric transvenous lead implantation may reduce risks for pneumothorax and hemothorax. The objective was to retrospectively evaluate ultrasound-guided axillary vein access as an alternative to the subclavian approach. Methods The technique consists of ultrasonographic identification of the axillary vein at the deltopectoral groove after initial contrast venography. A micropuncture kit is used for initial ultrasound-guided percutaneous access with fluoroscopic confirmation of wire position. Pocket creation is performed, and sheath insertion and lead implantation proceed as usual. Demographic, procedural, and radiation exposure data were collected and analyzed. Results Sixteen patients (median age = 13 years, 8-50 years; median weight = 56 kg, 29-77 kg) underwent lead implantation; two additional patients required fluoroscopy due to poor acoustic windows (89% success). Fifteen of 21 leads (71%) were ventricular; 50% of implants were pacemakers, and 31% were dual chamber. Median time to venous access was 13 min (interquartile range (IQR) = 9.25-20.25) and median implant procedure time was 156 min (IQR = 112-172). Median fluoroscopy time was 18.0 min (IQR = 11.9-29.6), median air kerma was 9.0 mGy (IQR = 3.0-28.5), and median dose-area product was 30.2 Gy-cm2 (IQR = 16.1-234.5). One patient required generator pocket revision 2 days postprocedure without lead dislodgement. There were no other complications encountered. Conclusions Transvenous pacemaker and implantable cardioverter-defibrillator lead implantation in the pediatric and adult congenital population through ultrasound-guided axillary venous access is safe and efficacious. This technique may provide a low-risk alternative for vascular access for pediatric implantation procedures.

BACKGROUND Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients. METHODS A retrospective, descriptive study of aortic cusp ablation in five pediatric electrophysiology centers from 2008 to 2014 was performed. All patients

The ability to differentiate right ventricular outflow tract (RVOT) from coronary cusp (CC) site of origin (SOO) by 12-lead ECG in pediatric patients may impact efficacy and procedural time. The objective of this study was to predict RVOT versus CC SOO by ECG in pediatric patients.Pediatric patients (21 years) without structural heart disease with RVOT or CC premature ventricular contraction (PVC) ablations performed (2014-2018) were evaluated through multi-institution retrospective review. Demographics, ECG PVC parameters, ablation site, recurrence, and repeat procedures were collected.Thirty-seven patients were evaluated (mean age 14.6 years, weight 60.6 kg): 11 CC and 26 RVOT PVC SOO. CC PVCs were less likely to exhibit left bundle branch block (64% vs 100%, P = .005), had larger R-wave amplitude in V1 (0.27 vs 0.11 mV, P = .03), larger R/S ratio in V1 (0.37 vs 0.09, P = .003), and had precordial transition in V3 or earlier (73% vs 15%, P = .002). A composite score was created with the following variables: isodiphasic or positive QRS in V1, R/S ratio in V1 0.05, S wave in V1 0.9 mV, and precordial transition at or before V3. Composite score ≥ 2 was associated with a CC SOO (OR 42.0, P = .001, and AUC 0.86).12-lead ECG of PVCs from the CC was associated with larger V1 R-wave amplitude, larger R/S ratio in V1, and precordial transition at or before V3. A composite score may help predict PVC/VT arising from the CC.

Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation.Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Nonpersistent preexcitation was defined as absence/sudden loss of preexcitation on electrocardiogram, Holter monitoring, or exercise stress test. RC-AF was defined as clinical preexcited atrial fibrillation with shortest preexcited R-R interval (SPERRI) ≤ 250 ms. AP effective refractory period (APERP), SPERRI at EPS , and shortest preexcited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤ 250 ms.Of 1589 patients, 244 (15%) had nonpersistent preexcitation and 1345 (85%) had persistent preexcitation. There were no differences in sex (58% vs 60% male; P=.49) or age (13.3±3.6 years vs 13.1±3.9 years; P=.43) between groups. Although APERP (344±76 ms vs 312±61 ms; P.001) and SPPCL (394±123 ms vs 317±82 ms; P.001) were longer in nonpersistent vs persistent preexcitation, there was no difference in SPERRI at EPS (331±71 ms vs 316±73 ms; P=.15). Nonpersistent preexcitation was associated with fewer high-risk APs (13% vs 23%; P.001) than persistent preexcitation. Of 61 patients with SCA or RC-AF, 6 (10%) had nonpersistent preexcitation (3 SCA, 3 RC-AF).Nonpersistent preexcitation was associated with fewer high-risk APs, though it did not exclude the risk of SCA or RC-AF in children with WPW.

Background: Smaller femoral arterial sheaths may be associated with fewer vascular complications. The 3.3 Fr Mongoose® Pediavascular pigtail catheter is a catheter that allows higher flow rates, potentially resulting in improved angiographic quality. We reviewed our experience with this small catheter during patent ductus arteriosus (PDA) closure. Materials and Methods: Review of patients ≤20 kg in whom the Mongoose® catheter was used during PDA closure from 12/13 to 4/15. Angiographic efficacy and procedural details were compared to ten 4 Fr catheter cases. Comparisons were performed using Mann–Whitney U-test; P < 0.05 was statistically significant. Results: Twelve (9 female) patients were catheterized with a 3.3 Fr Mongoose®. Median weight 10.5 kg (range 6.4–18.2), height 81 cm (range 37–111), and body surface area (BSA) 0.47 m2 (range 0.33–0.75) were similar to ten patients (3 females) in the 4 Fr control group (P = NS); median weight 9.9 kg (range 6–16.8), height 80 cm (range 64–102), and BSA 0.46 m2 (range 0.31–0.74). Angiographic quality was subjectively adequate with both with no difference in the median pixel density between the two techniques (3.3 Fr: 76.7 [range 33.5–90] and 4 Fr: [70; 38–102]; P = NS). Contrast used was similar between the groups (3.3 Fr: median 4.2 ml/kg and 4 Fr: 4.9 ml/kg; P = NS). Median radiation dose was similar in the two groups (3.3 Fr: 28.1 mGy [range 17.2–38] and 4 Fr: 38 mGy [range 20.4–58.5]; P = NS). All ducts were closed at latest follow-up ( P = NS). No complications were encountered. Conclusions: The 3.3 Fr Mongoose® allowed similar angiography to the 4 Fr pigtail catheter, allowing safe and effective transcatheter PDA closure in small children.

Introduction Patients with lung cancer who harbor multiple pulmonary sites of disease have been challenging to classify; a subcommittee of the International Association for the Study of Lung Cancer Staging and Prognostic Factors Committee was charged with developing proposals for the eighth edition of the tumor, node, and metastasis (TNM) classification to address this issue. Methods A systematic literature review and analysis of the International Association for the Study of Lung Cancer database was performed to develop proposals for revision in an iterative process involving multispecialty international input and review. Results Details of the evidence base are summarized in other articles. Four patterns of disease are recognized; the clinical presentation, pathologic correlates, and biologic behavior of these suggest specific applications of the TNM classification rules. First, it is proposed that second primary lung cancers be designated with a T, N, and M category for each tumor. Second, tumors with a separate tumor nodule of the same histologic type (either suspected or proved) should be classified according to the location of the separate nodule relative to the index tumor—T3 for a same-lobe, T4 for a same-side (different lobe), and M1a for an other-side location—with a single N and M category. Third, multiple tumors with prominent ground glass (imaging) or lepidic (histologic) features should be designated by the T category of the highest T lesion, the number or m in parentheses (#/m) to indicate the multiplicity, and a collective N and M category for all. Finally, it is proposed that diffuse pneumonic-type lung cancers be designated by size (or T3) if in one lobe, T4 if involving multiple same-side lobes, and M1a if involving both lungs with a single N and M category for all areas of involvement. Conclusion We propose to tailor TNM classification of multiple pulmonary sites of lung cancer to reflect the unique aspects of four different patterns of presentation. We hope that this will lead to more consistent classification and clarity in communication and facilitate further research in the nature and optimal treatment of these entities.

Device implantation requires fluoroscopic guidance, which carries inherent risks of ionizing radiation. We evaluated the impact of a low-dose fluoroscopic protocol on radiation exposure during device implantation. All patients who underwent pacemaker or ICD implantation with new transvenous leads from July 2011 to January 2018 were included. A novel ALARA protocol consisting of ultra-low frame rates (2–3 frames/s), low dose/frame (6–18 mGy/frame), and use of the “air-gap” technique in patients

Background: Interpretation of pediatric ECGs is limited by lack of accurate sex- and race-specific normal reference values obtained with modern technology for all ages. We sought to obtain contemporary digital ECG measurements in healthy children from North America, to evaluate the effects of sex and race, and to compare our results to commonly used published datasets. Methods: Digital ECGs (12-lead) were retrospectively collected for children ≤18 years old with normal echocardiograms at 19 centers in the Pediatric Heart Network. Patients were classified into 36 groups: 6 age, 2 sex, and 3 race (white, black, and other/mixed) categories. Standard intervals and amplitudes were measured; mean±SD and 2nd/98th percentiles were determined by age group, sex, and race. For each parameter, multivariable analysis, stratified by age, was conducted using sex and race as predictors. Parameters were compared with 2 large pediatric ECG data sets. Results: Among ECGs from 2400 children, significant differences were found by sex and race categories. The corrected QT interval in lead II was greater for girls compared with boys for age groups ≥3 years ( P ≤0.03) and for whites compared with blacks for age groups ≥12 years ( P P P ≤0.006), and greater compared with a commonly used public data set for age groups ≥12 years ( P Conclusions: In this large, diverse cohort of healthy children, most ECG intervals and amplitudes varied by sex and race. These differences have important implications for interpreting pediatric ECGs in the modern era when used for diagnosis or screening, including thresholds for left ventricular hypertrophy.

Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis.The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes.Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed.Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type.TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR.

There are few data on the incidence of echocardiographic (echo) abnormalities following catheter ablation in children in the era of 3D mapping. Wide practice variation exists regarding routine post-ablation echo. We hypothesized a low incidence of clinically significant echo abnormalities following SVT ablation in otherwise healthy children. Single center data from 2009 to 2015 were reviewed; routine post-ablation echo was standard practice. Cases were categorized as utilizing fluoroscopy alone (FLUORO) or 3D mapping with a low fluoroscopic protocol (CARTO3). Congenital heart disease was excluded. Outcomes of interest included new valvular abnormalities, pericardial effusions, and wall motion abnormalities. Findings were compared to baseline studies when available and classified as normal/unchanged, clinically insignificant, or clinically significant. Outcomes were compared between FLUORO and CARTO3 groups. Of 347 ablations, 319 (92%) underwent post-procedural echo: 57% male; 55% FLUORO; mean age 13.4 ± 3.6 years. The most common ablation target was an accessory pathway (AP) in 66% (n = 144 WPW, 66 concealed), followed by AVNRT in 32% (n = 102). Radiofrequency (RF) energy was utilized in 82% (n = 262). Post-ablation echos were normal in 81% (n = 259). Clinically insignificant findings were seen in 18% (n = 58), most commonly trivial-small pericardial effusions in 11% (n = 34). Two significant findings required additional follow-up or treatment. There were no cases of wall motion abnormalities or clinically significant effusions. There were no differences in frequency of echo abnormalities between the FLUORO and CARTO3 groups. Clinically significant echocardiographic abnormalities are rare following SVT ablation in children with structurally normal hearts, independent of the use of 3D mapping.

Catheter stability, an important factor in ablation success, is affected by ventilation. Optimal ventilation strategies for pediatric catheter ablation are not known. We hypothesized that small tidal volume and positive end-expiratory pressure are associated with reduced ablation catheter movement at annular positions. Subjects aged 5–25 years undergoing ablation for supraventricular tachycardia (SVT) or WPW at two centers from March 2015 to September 2016 were prospectively enrolled and randomized to receive mechanical ventilation with either positive end-expiratory pressure of 5 cm H2O (PEEP) or 0 cm H2O (ZEEP). Movement of the ablation catheter tip at standard annular positions was measured using 3D electroanatomic mapping systems under two conditions: small tidal volume (STV) (3–5 mL/kg) or large TV (LTV) (6–8 mL/kg). 58 subjects (mean age 13.8 years) were enrolled for a total of 266 separate observations of catheter movement. STV ventilation was associated with significantly reduced catheter movement, compared to LTV at all positions (right posteroseptal: 2.5 ± 1.4 vs. 5.2 ± 3.1 mm, p

Background: Cardiac catheterizations expose both the patient and staff to the risks of ionizing radiation. Studies using the “air gap” technique (AGT) in various radiological procedures indicate that its use leads to reduction in radiation exposure but there are no data on its use for pediatric cardiac catheterization. The aim of this study was to retrospectively review the radiation exposure data for children weighing

Background CARTO3 is frequently used during ablation but is not designed to allow visualization of non-CARTO3 ablation catheters. We describe how cryoablation catheters can be visualized and recorded using CARTO3 with minimal fluoroscopy (FLUORO) usage. Methods Retrospective review of patients ≤21 years undergoing cryoablation with CARTO3 from 2010 to 2013 for ablation of supraventricular tachycardia. After mapping with a Navistar catheter, the Navistar was removed and a cryocatheter was utilized. The cryocatheter was connected to the pin box via a jumper cable and the pin box was connected to the CARTO3 patient interface unit. Locations of ablation attempts with the cryocatheter were recorded with the “Create Snapshot” tool. Clinical characteristics and radiation doses were compared between patients undergoing cryoablation (cryoenergy [CRYO]) to an age- and diagnosis-matched control group (CONTROL) undergoing RF ablation. Results A total of 174 ablations were performed and 14 patients underwent cryoablation (CRYO, 13.3 ± 4.7 years, weight 42 ± 14 kg). Indications for cryoablation were: five atrioventricular nodal reentry tachycardia (36%), four ectopic atrial tachycardia (29%), three concealed accessory pathways (21%), and two Wolff-Parkinson-White syndromes (14%). Acute success was achieved in all patients (100%) with no complications and one recurrence (7%). The site of successful cryoablation was successfully recorded on the CARTO3 system in all cases. Radiation doses were low and not different from an age-, era-, and diagnosis-matched control group undergoing RF ablation (CRYO 3.2 ± 0.8 mGy vs CONTROL 1.6 ± 0.4 mGy, P = 0.07). Conclusions Though a “closed” system, CARTO3 can be “tricked” to allow for the use of cryoablation, allowing clear catheter visualization, mapping, and recording of ablation lesions with minimal FLUORO usage.

Purpose To compare the efficacy, safety, and cost-effectiveness of a three-catheter approach with a conventional five-catheter approach for the mapping and ablation of supraventricular tachycardia in pediatric patients with Wolff-Parkinson-White Syndrome (WPW) and concealed accessory pathways (APs). Methods A retrospective review from 2008 to 2012 of patients less than 21 years with WPW who underwent a three-catheter radiofrequency (RF) ablation of a left-sided AP (ablation, right ventricular [RV] apical, and coronary sinus [CS] decapolar catheters) was performed. The three-catheter group was compared to a control group who underwent a standard five-catheter (ablation, RV apical, CS decapolar, His catheter, and right atrial catheter) ablation for the treatment of left-sided WPW or concealed AP. Demographics, ablation outcomes, and costs were compared between groups. Results Twenty-eight patients met inclusion criteria with 28 control patients. The groups did not differ in gender, age, weight, or body surface area. Locations of the AP on the mitral annulus were similar between the groups. All patients were ablated via transseptal approach. Note that 28 of 28 in the three-catheter group (100%) and 27 of 28 (96%) controls were acutely successfully ablated (P = 0.31). No complications were encountered. There was no difference in procedural time, time to loss of AP conduction, or number of RF applications. Use of the three-catheter technique resulted in a total savings of $2,465/case, which includes the $680 savings from using fewer catheters as well as the savings from a shortened procedure time. Conclusions Ablation in patients with WPW and a left-sided AP can be performed using three catheters with similar efficacy and safety while offering significant cost savings compared to a conventional five-catheter approach.

3-D Mapping Improves Success Rates for WPW AblationIntroduction Three-dimensional mapping (3-D) systems are frequently used for ablation of supraventricular tachycardia. Prior studies have demonstrated radiation dosage reduction with 3-D, but there are no data on whether 3-D improves the efficacy of ablation of Wolff-Parkinson-White syndrome (WPW). We sought to determine if 3-D improves the success rate for ablation of WPW in children. Methods Multicenter retrospective study including patients ≤21 years of age with WPW undergoing ablation from 2008 to 2012. Success rates using the 2 techniques (3-D vs. fluoroscopy alone [FLUORO]) were compared. Results Six hundred and fifty-one cases were included (58% male, mean age 13 ± 4 years, 366 [56%] 3-D). Baseline characteristics including gender, weight, accessory pathway (AP) location, number of APs, and repeat ablation attempts were similar between the 2 groups (3-D and FLUORO) The 3-D group was slightly younger (12.7 ± 4.0 vs. 13.3 ± 4.0 years; P = 0.04) and less likely to undergo ablation utilizing cryoenergy (38 [10%] vs. 56 [20%]; P < 0.01). The 3-D group had a higher acute success rate of ablation (355 [97%] vs. 260 [91%]; P < 0.01). No differences were seen in recurrence (16 [5%] vs. 26 [9%]; P = 0.09) or complication rates (1 [0.3%] vs. 1 [0.4%]; P = 0.86) between the groups. On multivariable analysis, 3-D was shown to significantly improve success at ablation with an odds ratio of 3.1 (95% CI 1.44–6.72; P < 0.01). Conclusions Use of 3-D significantly improved success rates for ablation of WPW in children. The increase in acute success associated with 3-D suggests it is an important adjunct for catheter ablation of WPW in children.

Background: Smaller femoral arterial sheaths may be associated with fewer vascular complications. The 3.3 Fr Mongoose® Pediavascular pigtail catheter is a catheter that allows higher flow rates, potentially resulting in improved angiographic quality. We reviewed our experience with this small catheter during patent ductus arteriosus (PDA) closure. Materials and Methods: Review of patients ≤20 kg in whom the Mongoose® catheter was used during PDA closure from 12/13 to 4/15. Angiographic efficacy and procedural details were compared to ten 4 Fr catheter cases. Comparisons were performed using Mann–Whitney U-test; P < 0.05 was statistically significant. Results: Twelve (9 female) patients were catheterized with a 3.3 Fr Mongoose®. Median weight 10.5 kg (range 6.4–18.2), height 81 cm (range 37–111), and body surface area (BSA) 0.47 m2 (range 0.33–0.75) were similar to ten patients (3 females) in the 4 Fr control group (P = NS); median weight 9.9 kg (range 6–16.8), height 80 cm (range 64–102), and BSA 0.46 m2 (range 0.31–0.74). Angiographic quality was subjectively adequate with both with no difference in the median pixel density between the two techniques (3.3 Fr: 76.7 [range 33.5–90] and 4 Fr: [70; 38–102]; P = NS). Contrast used was similar between the groups (3.3 Fr: median 4.2 ml/kg and 4 Fr: 4.9 ml/kg; P = NS). Median radiation dose was similar in the two groups (3.3 Fr: 28.1 mGy [range 17.2–38] and 4 Fr: 38 mGy [range 20.4–58.5]; P = NS). All ducts were closed at latest follow-up (P = NS). No complications were encountered. Conclusions: The 3.3 Fr Mongoose® allowed similar angiography to the 4 Fr pigtail catheter, allowing safe and effective transcatheter PDA closure in small children.

Patients often use the internet as a source of information about their condition and treatments. However, this information is unregulated and varies in quality. To evaluate the readability and quality of online information for pediatric and adult patients and caregivers regarding sclerotherapy for venous malformations. “Venous malformation sclerotherapy” was entered into Google, and results were reviewed until 20 sites that satisfied predefined inclusion criteria were identified. Scientific and non-patient-focused web pages were excluded. Readability was assessed using the Flesch Reading Ease Score and American Medical Association reading difficulty recommendations and quality was assessed using Journal of the American Medical Association standards and assessing if the site displayed HONcode (Health on the Net Code) certification. Assessment of the breadth of relevant information was made using a predefined checklist. Forty-nine search engine results were reviewed before 20 sites were identified for analysis. Average Flesch Reading Ease Score was 44 (range: 24.2–70.1), representing a “fairly difficult” reading level. None of the sites had a Flesch Reading Ease Score meeting the American Medical Association recommendation of 80-90. Only one site met all four Journal of the American Medical Association quality criteria (average: 2.1). None of the sites displayed a HONcode seal. The information most frequently found was: sclerotherapy is performed by radiologists, multiple treatments may be needed and surgery is an alternative treatment. Online information regarding sclerotherapy for venous malformations is heterogeneous in quality and breadth of information, and does not meet readability recommendations for patient information. Radiologists should be aware of and account for this when meeting patients.

INTRODUCTION: Revisions to the TNM stage classifications for lung cancer, informed by the international database (N = 94,708) of the International Association for the Study of Lung Cancer (IASLC) Staging and Prognostic Factors Committee, need external validation. The objective was to externally validate the revisions by using the National Cancer Data Base (NCDB) of the American College of Surgeons.METHODS: Cases presenting from 2000 through 2012 were drawn from the NCDB and reclassified according to the eighth edition stage classification. Clinically and pathologically staged subsets of NSCLC were analyzed separately. The T, N, and overall TNM classifications were evaluated according to clinical, pathologic, and "best" stage (N = 780,294). Multivariate analyses were carried out to adjust for various confounding factors. A combined analysis of the NSCLC cases from both databases was performed to explore differences in overall survival prognosis between the two databases.RESULTS: The databases differed in terms of key factors related to data source. Survival was greater in the IASLC database for all stage categories. However, the eighth edition TNM stage classification system demonstrated consistent ability to discriminate TNM categories and stage groups for clinical and pathologic stage.CONCLUSIONS: The IASLC revisions made for the eighth edition of lung cancer staging are validated by this analysis of the NCDB database by the ordering, statistical differences, and homogeneity within stage groups and by the consistency within analyses of specific cohorts.

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

Exercise testing is commonly performed in children for evaluation of cardiac disease. Few data exist, however, on the prevalence, types of arrhythmias, predictors for arrhythmias, and safety of exercise testing in children. A retrospective review of all patients ≤21 years undergoing exercise testing at our center from 2008 to 2012 was performed. Patients with clinically relevant arrhythmias were compared to those not experiencing a significant arrhythmia. 1,037 tests were performed in 916 patients. The mean age was 14 ± 4 years, 537 (55 %) were male, 281 (27 %) had congenital heart disease, 178 (17 %) had a history of a prior arrhythmia, and 17 (2 %) had a pacemaker or ICD. 291 (28 %) patients had a rhythm disturbance during the procedure. Clinically important arrhythmias were noted in 34 (3 %) patients and included: 19 (1.8 %) increasing ectopy with exercise, 5 (0.5 %) VT, 5 (0.5 %) second degree AV block, 3 (0.3 %) SVT, and 2 (0.2 %) AFIB. On multivariate logistic regression, variables associated with the development of clinically relevant arrhythmias included severe left ventricular (LV) dysfunction on echo (OR 1.99, CI 1.20-3.30) and prior history of a documented arrhythmia (OR 2.94, CI 1.25-6.88). There were no adverse events related to testing with no patient requiring cardioversion, defibrillation, or acute anti-arrhythmic therapy. A total of 28 % of children developed a rhythm disturbance during exercise testing and 3 % were clinically important. Severe LV dysfunction and a history of documented arrhythmia were associated with the development of a clinically important arrhythmia.

Background: “ALARA – As Low As Reasonably Achievable” protocols reduce patient radiation dose. Addition of electroanatomical mapping may further reduce dose. Methods: From 6/11 to 4/12, a novel ALARA protocol was utilised for all patients undergoing supraventricular tachycardia ablation, including low frame rates (2–3 frames/second), low fluoro dose/frame (6–18 nGy/frame), and other techniques to reduce fluoroscopy (ALARA). From 6/12 to 3/13, use of CARTO® 3 (C3) with “fast anatomical mapping” (ALARA+C3) was added to the ALARA protocol. Intravascular echo was not utilised. Demographics, procedural, and radiation data were analysed and compared between the two protocols. Results: A total of 75 patients were included: 42 ALARA patients, and 33 ALARA+C3 patients. Patient demographics were similar between the two groups. The acute success rate in ALARA was 95%, and 100% in ALARA+C3; no catheterisation-related complications were observed. Procedural time was 125.7 minutes in the ALARA group versus 131.4 in ALARA+C3 (p=0.36). Radiation doses were significantly lower in the ALARA+C3 group with a mean air Kerma in ALARA+C3 of 13.1±28.3 mGy (SD) compared with 93.8±112 mGy in ALARA (pConclusions: Addition of CARTO® 3 to ALARA protocols markedly reduced radiation exposure to young people undergoing supraventricular tachycardia ablation while allowing for equivalent procedural efficacy and safety.

Introduction There are little data on the effect of catheter position and mechanical ventilation on ablation catheter stability during electrophysiology study in children. We sought to determine the magnitude of catheter movement with mechanical ventilation, the effect of ventilation maneuvers on catheter movement, and to compare the degree of movement observed between the right lateral (RL) and right posteroseptal (RPS) regions. Methods From June 2012 to June 2013, patients ≤21 years of age undergoing ablation for supraventricular tachycardia with CARTO® 3 (Biosense Webster, Diamond Bar, CA, USA) were included. During mapping the ablation catheter was placed in the RPS and RL regions and the magnitude of catheter movement (mm) was measured using CARTO® 3. Measurements were made during routine ventilation and with a maximal inspiration maneuver between end-expiration (ENDEX) and peak-inspiration (PEAKINS). Results Twenty-one patients were included: 12 males (57%), age 13 ± 3 years, weight 55 ± 14 kg. Indications for ablation were: 10 Wolff-Parkinson-White, seven atrioventricular node re-entry tachycardia, four concealed accessory pathway. Mechanical ventilation was used in all cases. The magnitude of catheter movement was 3.6 ± 1.7 mm with routine ventilation and 6.2 ± 4.1 mm between ENDEX and PEAKINS (P ≤ 0.01). Catheter movement was greater in the RL compared to the RPS region with routine ventilation (RL 4.3 ± 1.6 vs RPS 3.0 ± 1.5; P < 0.01) and between ENDEX and PEAKINS (RL 8.3 ± 4.7 vs RPS 4.0 ± 1.7; P < 0.01). Conclusions Ventilation and catheter position both have significant impact on the degree of catheter movement during ablation. Movement was greatest in the RL position. This may partially explain the lower success rates of ablation in the RL region.

BackgroundIn previous pilot work we demonstrated that a novel automated signal analysis tool could accurately identify successful ablation sites during Wolff-Parkinson-White (WPW) ablation at a single center.ObjectiveWe sought to validate and refine this signal analysis tool in a larger multi-center cohort of children with WPW.MethodsA retrospective review was performed of signal data from children with WPW who underwent ablation at two pediatric arrhythmia centers from 2008-2015. All patients with WPW ≤ 21 years who underwent invasive electrophysiology study and ablation with ablation signals available for review were included. Signals were excluded if temperature or power delivery was inadequate or lesion time was < 5 seconds. Ablation lesions were reviewed for each patient. Signals were classified as successful if there was loss of antegrade and retrograde accessory pathway (AP) conduction or unsuccessful if ablation did not eliminate AP conduction. Custom signal analysis software analyzed intracardiac electrograms for amplitudes, high and low frequency components, integrated area, and signal timing components to create a signal score. We validated the previously published signal score threshold 3.1 in this larger, more diverse cohort and explored additional scoring options. Logistic regression with lasso regularization using Youden's index criterion and a cost-benefit criterion to identify thresholds was considered as a refinement to this score.Results347 signals (141 successful, 206 unsuccessful) in 144 pts were analyzed [mean age 13.2 ± 3.9 years, 96 (67%) male, 66 (45%) left sided APs]. The software correctly identified the signals as successful or unsuccessful in 276/347 (80%) at a threshold of 3.1. The performance of other thresholds did not significantly improve the predictive ability. A signal score threshold of 3.1 provided the following diagnostic accuracy for distinguishing a successful from unsuccessful signal: sensitivity 83%, specificity 77%, PPV 71%, NPV 87%.ConclusionsAn automated signal analysis software tool reliably distinguished successful versus unsuccessful ablation electrograms in children with WPW when validated in a large, diverse cohort. Refining the tools using an alternative threshold and statistical method did not improve the original signal score at a threshold of 3.1. This software was effective across two centers and multiple operators and may be an effective tool for ablation of WPW.

After heart transplantation (HT) in infants and young children, environmental and intrinsic factors may lead to changes in the geometry and compliance of the donor heart. Serial demographic, clinical, hemodynamic, and echocardiographic data were obtained from HT recipients younger than 4 years of age. Echocardiographic chamber measurement z-scores were compared using recipient body surface area from the time of HT to 1 week, 3 months, and last follow-up visit. Left ventricular end-diastolic volume (LVEDV) z-scores were correlated with pulmonary capillary wedge pressure (PCWP) at each time point. Heart transplantation was performed for 13 children between March 2009 and December 2012, 9 of whom (69%) were boys. The median age at HT was 8 months (range, 4-43 months), and the mean follow-up period was 13 ± 7 months. Left ventricular end-diastolic dimension z-scores decreased significantly (p = 0.03) between HT and 1 week, then increased from 1 week to 3 and 12 months. (-1.32 ± 1.7, -0.71 ± 1.8, 0.41 ± 2.1, 0.79 ± 2.3, respectively). A positive relationship (R(2) = 0.48) between the LVEDV z-score and PCPW was present at the last follow-up visit. For infants and young children, the allograft demonstrates appropriate growth by 1 year after HT. Left ventricular compliance improves over time.

Right pulmonary artery (RPA) stenosis following Waterston shunt is common. We report a case of RPA stenosis many years following tetralogy of Fallot repair with take-down of a Waterston shunt and demonstrate an unusual and important anatomic variant of proximity of the left coronary artery to the mid-portion of the RPA. © 2013 Wiley Periodicals, Inc.

Background: Surveillance endomyocardial biopsy (EMB) with right heart catheterization (RHC) is the standard of care for the assessment of post cardiac transplantation rejection. This procedure has traditionally relied upon fluoroscopy, which exposes both patient and staff to the risks of ionizing radiation. These risks may be of particular concern in the transplant patient who must undergo many such procedures lifelong. We present data on a new “ALARA – As Low As Reasonably Achievable” protocol to reduce radiation exposure during the performance of RHC with EMB. Methods: All cardiac transplantation patients