Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection (MIS-C): A Multi-institutional Study from New York City.

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.

Can the 12-lead ECG distinguish RVOT from aortic cusp PVCs in pediatric patients?

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Ultrasound-guided axillary venous access for pediatric and adult congenital lead implantation.

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.

Incidence of Echocardiographic Abnormalities Following Pediatric SVT Ablation: Comparison of Cases Utilizing Fluoroscopy Alone to Cases with Adjunctive 3D Electroanatomic Mapping.

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.

A Prospective Assessment of Optimal Mechanical Ventilation Parameters for Pediatric Catheter Ablation.

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 < 0.0001; right lateral: 2.7 ± 1.6 vs. 6.3 ± 3.5 mm, p < 0.0001; left lateral: 1.8 ± 1.0 vs. 4.3 ± 1.9 mm, p < 0.0001). The presence or absence of PEEP had no effect on catheter movement. In multivariable analysis, STV was associated with a 3.1-mm reduction in movement (95% CI 2.6-3.5, p < 0.0001), adjusting for end-expiratory pressure, annular location, and patient size. We conclude that STV ventilation is associated with reduced ablation catheter movement compared to a LTV strategy, independent of PEEP and annular position.

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

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 (

A multicenter review of ablation in the aortic cusps in young people.

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 <21 years of age who underwent ablation in the aortic cusps were included. Factors analyzed included patient demographics, procedural details, outcomes, and complications. RESULTS: Thirteen patients met inclusion criteria (median age 16 years [range 10-20.5] and median body surface area 1.58 m2 [range 1.12-2.33]). Substrates for ablation included: nine premature ventricular contractions or sustained ventricular tachycardia (69%), two concealed anteroseptal accessory pathways (APs) (15%), one Wolff-Parkinson-White with an anteroseptal AP (8%), and one ectopic atrial tachycardia (8%). Three-dimensional electroanatomic mapping in combination with fluoroscopy was used in 12/13 (92%) patients. Standard 4-mm-tip radiofrequency (RF) current was used in 11/13 (85%) and low-power irrigated-tip RF in 2/13 (15%). Angiography was used in 13/13 and intracardiac echocardiography was additionally utilized in 3/13 (23%). Ablation locations included: eight noncoronary (62%), three left (23%), and two right (15%) cusps. Ablation was acutely successful in all patients. At median follow-up of 20 months, there was one recurrence of PVCs (8%). There were no ablation-related complications and no valvular injuries observed. CONCLUSION: Arrhythmias originating from the coronary cusps in this series were successfully and safely ablated in young people without injury to the coronary arteries or the aortic valve.

Tricking CARTO: Cryoablation of Supraventricular Tachycardia in Children with Minimal Radiation Exposure Using the CARTO3 System.

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.

Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.

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.

Success rates in pediatric WPW ablation are improved with 3-dimensional mapping systems compared with fluoroscopy alone: a multicenter study.

INTRODUCTION: 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.

Three-Catheter Technique for Ablation of Left-Sided Accessory Pathways in Wolff-Parkinson-White is Less Expensive and Equally Successful When Compared to a Five-Catheter Technique.

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.

The prevalence of arrhythmias, predictors for arrhythmias, and safety of exercise stress testing in children.

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.

Reducing patient radiation exposure during paediatric SVT ablations: use of CARTO® 3 in concert with "ALARA" principles profoundly lowers total dose.

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 (p<0.001). Mean dose area product was 92.2±179 uGym2 in ALARA+C3 compared with 584±687 uGym2 in ALARA (p<0.001). Of the 33 subjects (42%) in the ALARA+C3 group, 14 received ⩽1 mGy exposure. The ALARA+C3 dosages are the lowest reported for a combined electroanatomical-fluoroscopy technique. CONCLUSIONS: 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.

Intervening for RPA stenosis following Waterston shunt: Importance of anatomical definition of the coronary arteries.

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.

Pediatric patient radiation dosage during endomyocardial biopsies and right heart catheterization using a standard "ALARA" radiation reduction protocol in the modern fluoroscopic era.

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 < 21 years of age who underwent RHC with EMB at The Children's Hospital at Montefiore from 6/11-12/11 were included. EMB was performed after all right heart pressures including wedge pressure and thermodilution cardiac output were measured. A novel ALARA protocol consisting of multiple features including ultra-low frame rates (2-3 fps), low fluoro dose/frame (10-18 nGy/frame), use of the "air-gap" technique for patients < 20 kg, and multiple other techniques aimed at minimizing use of fluoroscopy were employed in all cases. Demographics, procedural data and patient radiation exposure levels were collected and analyzed. RESULTS: 18 patients underwent 45 surveillance RHC with EMB in the study period and were the subject of this analysis. The mean age was 5.9 ± 6.1 years, weight was 20.4 kg ± 16.6 kg, and BSA was 0.75 ± 45 m(2) . PA fluoroscopy was used exclusively in 45/45. Vascular access was RFV (21/45; 47%), RIJV (17/45; 38%), LFV (4/45; 9%) and LIJV (3/45; 7%). The median number of EMB specimens obtained was 5 (range, 4-7). The median fluoroscopy time was 3.7 min (range, 1.2-9). The median air Kerma product (K) was 1.4 mGy (range, 0.4-14), and dose area product (DAP) was 15.8 uGym(2) (range, 3.5-144.5). The K and DAP are substantially lower than any prior published data for RHC/EMB in this patient group. There were no procedural complications. CONCLUSIONS: The use of a novel ALARA protocol for RHC and EMB in pediatric cardiac transplantation patients markedly reduced radiation exposure to levels far below any previously reported values without negatively affecting the safety or efficacy of these procedures.

Effects of ventilation and catheter position on catheter movement on the tricuspid annulus during ablation in children.

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.

Reverse ventricular remodeling and improved ventricular compliance after heart transplantation in infants and young children.

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.

Quantitative Prediction of Right Ventricular Size and Function From the ECG.

BACKGROUND: Right ventricular ejection fraction (RVEF) and end-diastolic volume (RVEDV) are not readily assessed through traditional modalities. Deep learning-enabled ECG analysis for estimation of right ventricular (RV) size or function is unexplored. METHODS AND RESULTS: We trained a deep learning-ECG model to predict RV dilation (RVEDV >120 mL/m2), RV dysfunction (RVEF ≤40%), and numerical RVEDV and RVEF from a 12-lead ECG paired with reference-standard cardiac magnetic resonance imaging volumetric measurements in UK Biobank (UKBB; n=42 938). We fine-tuned in a multicenter health system (MSHoriginal [Mount Sinai Hospital]; n=3019) with prospective validation over 4 months (MSHvalidation; n=115). We evaluated performance with area under the receiver operating characteristic curve for categorical and mean absolute error for continuous measures overall and in key subgroups. We assessed the association of RVEF prediction with transplant-free survival with Cox proportional hazards models. The prevalence of RV dysfunction for UKBB/MSHoriginal/MSHvalidation cohorts was 1.0%/18.0%/15.7%, respectively. RV dysfunction model area under the receiver operating characteristic curve for UKBB/MSHoriginal/MSHvalidation cohorts was 0.86/0.81/0.77, respectively. The prevalence of RV dilation for UKBB/MSHoriginal/MSHvalidation cohorts was 1.6%/10.6%/4.3%. RV dilation model area under the receiver operating characteristic curve for UKBB/MSHoriginal/MSHvalidation cohorts was 0.91/0.81/0.92, respectively. MSHoriginal mean absolute error was RVEF=7.8% and RVEDV=17.6 mL/m2. The performance of the RVEF model was similar in key subgroups including with and without left ventricular dysfunction. Over a median follow-up of 2.3 years, predicted RVEF was associated with adjusted transplant-free survival (hazard ratio, 1.40 for each 10% decrease; P=0.031). CONCLUSIONS: Deep learning-ECG analysis can identify significant cardiac magnetic resonance imaging RV dysfunction and dilation with good performance. Predicted RVEF is associated with clinical outcome.

Lone atrial fibrillation in the young - perhaps not so "lone"?

OBJECTIVE: To determine if pediatric patients with a history of lone atrial fibrillation (AF) have other forms of supraventricular tachycardia (SVT) that may potentially trigger AF. STUDY DESIGN: A multicenter review of patients with lone AF who underwent electrophysiology (EP) study from 2006-2011 was performed. INCLUSION CRITERIA: age ≤21 years, normal ventricular function, structurally normal heart, history of AF, and EP study and/or ablation performed. EXCLUSION CRITERIA: congenital heart disease or cardiomyopathy. Patient demographics, findings at EP study and follow-up data were recorded. RESULTS: Eighteen patients met inclusion criteria. The mean age was 17.9 ± 2.2 years, weight was 82 ± 21 kg, body mass index was 27 ± 6, and 15 (83%) were males. Eleven (61%) were overweight or obese. Seven (39%) had inducible SVT during EP study: 5 atrioventricular nodal re-entry tachycardia (71%) and 2 concealed accessory pathways with inducible atrioventricular re-entry tachycardia (29%). All 7 patients with inducible SVT underwent radiofrequency ablation. There were no complications during EP study and/or ablation for all 18 patients. The mean follow-up was 1.7 ± 1.5 years and there were no recurrences in the 7 patients who underwent ablation. There were 2 recurrences of AF in patients with no other form of SVT during EP study. CONCLUSIONS: Inducible SVT was found in 39% of pediatric patients undergoing EP study for lone AF. EP study should be considered for pediatric patients presenting with lone AF.