Corrado Di Mambro

Ventricular pre-excitation: symptomatic and asymptomatic children have the same potential risk of sudden cardiac death

AIMS Children and adolescents with ventricular pre-excitation (VPE) are at increased risk for sudden cardiac death (SCD). Although antiarrhythmic therapy and catheter ablation are well established temporary or definitive treatments for patients with Wolff-Parkinson-White (WPW) syndrome, the optimal management of children with asymptomatic VPE remains to be clearly defined. On the basis of the most recent guidelines and recommendations, the aim of this study was to determine the electrophysiological characteristics of young patients with VPE and WPW syndrome to assess and compare their potential risk of SCD. METHODS AND RESULTS We retrospectively investigated 124 consecutive young patients with VPE (51 with WPW syndrome and 73 asymptomatic) who underwent transoesophageal electrophysiological study. At baseline, atrioventricular reentrant tachycardia (AVRT) was induced in 13 WPW vs. 10 asymptomatic patients (25.5 vs. 13.7%, P = NS). Atrial fibrillation (AF) was induced in 13 WPW vs. 15 asymptomatic patients (25.5 vs. 20.5%, P = NS). A shortest pre-excited R-R interval (SPERRI) ≤250 ms during AF was found in four WPW vs. six asymptomatic patients (30.8 vs. 40%, P = NS). During isoproterenol infusion or stress testing, AVRT was induced in 31 of 44 WPW vs. 33 of 69 asymptomatic patients (70.4 vs. 47.8%, P = 0.018). Atrial fibrillation was induced in 12 of 44 WPW vs. 21 of 69 asymptomatic patients (27.3 vs. 30.4%, P = NS). A SPERRI ≤ 210 ms was found in 6 of 12 WPW vs. 10 of 21 asymptomatic patients (50 vs. 47.6%, P = NS). No statistically significant correlation was observed between accessory pathway location and symptoms, AVRT/AF inducibility, or mean APERP/SPERRI values. CONCLUSION Children and adolescents with WPW syndrome have a higher rate of AVRT inducibility than asymptomatic patients. However, no differences between the two groups were found in atrial vulnerability and parameters related to the risk of SCD.

Left ventricular pacing in neonates and infants with isolated congenital complete or advanced atrioventricular block: short- and medium-term outcome

AIMS Right ventricular (RV) pacing may induce left ventricular (LV) dysfunction: neonates and infants with isolated congenital complete/advanced atrioventricular block (CCAVB) are at high risk of developing RV pacing-induced LV dyssynchrony, remodelling, and dysfunction. We prospectively investigated whether LV pacing results in normal LV function and good clinical status in the short/medium term. METHODS AND RESULTS In this single-centre, prospective study, 10 consecutive patients with CCAVB (median age 4 months, range: 0.1-16) underwent pacemaker implantation (4 VVIR, 6 DDD) using epicardial leads (on the LV apex in 8, on the LV free wall in 2). Data were collected at implantation and at 1- and 12-month follow-up. Echocardiographic evaluation included two-dimensional/three-dimensional assessment of LV dimensions, function (ejection fraction, EF), and ventricular synchrony (interventricular and intraventricular dyssynchrony). Prior to pacemaker implantation, EF was normal in six patients, 50% in two, ≤40% in two. All patients showed good clinical status and normal LV dimensions at follow-up. Patients with LV dilatation and impaired EF at implantation showed LV reverse remodelling and enhanced LV function. Normal LV function and synchrony were observed in most patients (one patient with EF 53% and three patients with mild dyssynchrony at 12-month follow-up). Paced QRS complex tended to be wider than native QRS complexes (P = 0.07); QTc duration of paced complexes was within normal limits or only slightly prolonged, without significant differences compared with QTc interval of native complexes. CONCLUSION At short- and medium-term follow-up, LV pacing results in satisfactory LV electromechanical function and synchrony in neonates and infants with CCAVB.

Cryoablation of AVNRT in children and adolescents: early intervention leads to a better outcome.

Currently, transcatheter cryoablation is a well‐established technique for the treatment of atrioventricular nodal reentry tachycardia (AVNRT) in children. In the past decade, many studies have focused on identifying factors that may affect acute and long‐term outcome, but none has been shown to be strongly predictive of treatment success. The aim of our study was to determine whether patient age and cryoablation method correlate with acute and long‐term success.

Cryoablation of right-sided accessory pathways in children: report of efficacy and safety after 10-year experience and follow-up

AIMS The aim of this study was to report the 10-year experience of our Institution in cryoablation of supraventricular tachycardia due to a right accessory pathway (AP). METHODS AND RESULTS Seventy-one cryoablations of right AP were performed between July 2002 and October 2011 in our Institution in 66 patients (mean age 12 + 3 years, 56% males). Acute procedural success rate was 97%: 80% in patients with concealed AP and 100% in those with manifest AP (P < 0.05). Acute procedural success rate was not related to institutional experience. No permanent complication occurred. Sixteen patients had recurrences during the follow-up (18.6 ± 6.6 months; range 3-111), 13 within the first month of follow-up, 2 within the 6 months of follow-up, and 1 within 12 months of follow-up. Cox regression showed that sex, patient age, number of delivered cryo-bonus, and presence of manifest or concealed AP are not independent predictors of procedural success. Among the 16 patients with recurrences, in 10 a cryoablation redo was successful and with no further AP recurrences. The mean fluoroscopy time was 28.4 min (range 19.7-44.6) with a significant decrease (P = 0.033) in relation to the increase of the institutional experience. There were no permanent ablation-related complications. CONCLUSION Cryoablation of right-sided AP is effective and very safe in children. Better results are achieved in manifest AP. The learning curve has a real impact in the attempt to reduce the fluoroscopy time.

Comparison of cryoablation with 3D mapping versus conventional mapping for the treatment of atrioventricular re-entrant tachycardia and right-sided paraseptal accessory pathways.

UNLABELLED Aim Transcatheter cryoablation is a well-established technique for the treatment of atrioventricular nodal re-entry tachycardia and atrioventricular re-entry tachycardia in children. Fluoroscopy or three-dimensional mapping systems can be used to perform the ablation procedure. The aim of this study was to compare the success rate of cryoablation procedures for the treatment of right septal accessory pathways and atrioventricular nodal re-entry circuits in children using conventional or three-dimensional mapping and to evaluate whether three-dimensional mapping was associated with reduced patient radiation dose compared with traditional mapping. METHODS In 2013, 81 children underwent transcatheter cryoablation at our institution, using conventional mapping in 41 children - 32 atrioventricular nodal re-entry tachycardia and nine atrioventricular re-entry tachycardia - and three-dimensional mapping in 40 children - 24 atrioventricular nodal re-entry tachycardia and 16 atrioventricular re-entry tachycardia. RESULTS Using conventional mapping, the overall success rate was 78.1 and 66.7% in patients with atrioventricular nodal re-entry tachycardia or atrioventricular re-entry tachycardia, respectively. Using three-dimensional mapping, the overall success rate was 91.6 and 75%, respectively (p=ns). The use of three-dimensional mapping was associated with a reduction in cumulative air kerma and cumulative air kerma-area product of 76.4 and 67.3%, respectively (p<0.05). CONCLUSIONS The use of three-dimensional mapping compared with the conventional fluoroscopy-guided method for cryoablation of right septal accessory pathways and atrioventricular nodal re-entry circuits in children was associated with a significant reduction in patient radiation dose without an increase in success rate.

Miniaturized Implantable Loop Recorder in Small Patients: An Effective Approach to the Evaluation of Subjects at Risk of Sudden Death

The etiological diagnosis of syncope and/or palpitations in children is often challenging. However, when noninvasive conventional examinations are inconclusive, the subcutaneous miniaturized implantable loop recorder (ILR) is recommended. The aim of our study was to evaluate the efficacy of miniaturized cardiac implantable devices in the early diagnosis of arrhythmias in children ≤6 years.

Results of remote follow-up and monitoring in young patients with cardiac implantable electronic devices.

BACKGROUND Remote monitoring is increasingly used in the follow-up of patients with cardiac implantable electronic devices. Data on paediatric populations are still lacking. The aim of our study was to follow-up young patients both in-hospital and remotely to enhance device surveillance. METHODS This is an observational registry collecting data on consecutive patients followed-up with the CareLink system. Inclusion criteria were a Medtronic device implanted and patients willingness to receive CareLink. Patients were stratified according to age and presence of congenital/structural heart defects (CHD). RESULTS A total of 221 patients with a device - 200 pacemakers, 19 implantable cardioverter defibrillators, and two loop recorders--were enrolled (median age of 17 years, range 1-40); 58% of patients were younger than 18 years of age and 73% had CHD. During a follow-up of 12 months (range 4-18), 1361 transmissions (8.9% unscheduled) were reviewed by technicians. Time for review was 6 ± 2 minutes (mean ± standard deviation). Missed transmissions were 10.1%. Events were documented in 45% of transmissions, with 2.7% yellow alerts and 0.6% red alerts sent by wireless devices. No significant differences were found in transmission results according to age or presence of CHD. Physicians reviewed 6.3% of transmissions, 29 patients were contacted by phone, and 12 patients underwent unscheduled in-hospital visits. The event recognition with remote monitoring occurred 76 days (range 16-150) earlier than the next scheduled in-office follow-up. CONCLUSIONS Remote follow-up/monitoring with the CareLink system is useful to enhance device surveillance in young patients. The majority of events were not clinically relevant, and the remaining led to timely management of problems.

Dysfunctional Behaviors in Children and Adolescents with NeurocardiogenicSyncope

Objective: We evaluate a large sample of children and adolescent with suspected neurocardiogenic syncope (NCS) and we compared the results with a healthy sample, with the aim to reveal a possible correlation between syncopal events and dysfunctional behaviors. Methods: A total number of ninety two patients with NCS (median age 14.7 years) were evaluated with Head-Up Tilt Test (HUTT) and psychological assessment at the same time. The results of the ninety two patients who underwent HUTT and completed psychological tests were compared with a normative group. The risk of psychosocial dysfunctions was assessed by using the standardized Italian version of the Child Behavior Checklist in Youth Self Report (YSR). Results: Compared with normative group, patients with NCS reported worse scores to the internalizing and total problem scales and to the syndrome scales of anxiety problems, depressive withdrawal problems, somatic complaints, social problems and thought problems. Conclusions: Patients with NCS present major emotional and behavioral dysfunctions than healthy sample. Our findings call for additional investigations on the possible pathophysiological association between psychosocial problems and the reflex mechanism that produces syncope. Furthermore, future clinical studies are necessary to plan an interventional strategy and optimize the clinical management.

Role of right ventricular three-dimensional electroanatomic voltage mapping for arrhythmic risk stratification of patients with corrected tetralogy of Fallot or other congenital heart disease involving the right ventricular outflow tract.

BACKGROUND The post-surgical history of repaired congenital heart disease (rCHD), in particular tetralogy of Fallot (TOF), is often complicated by sudden death. Electrical myocardial abnormalities could be a substrate for malignant ventricular arrhythmias. METHODS AND RESULTS 146 patients with TOF or other rCHD involving a subpulmonary right ventricle, considered to be at high arrhythmic risk, underwent right ventricular (RV) electroanatomic voltage mapping (EVM). Maps showed endocardial scars (<0.5mV) in all cases, mainly involving the RV outflow tract (n=141, 96.6%). In 28 cases (19.2%), other areas were involved. Total scar extension, expressed as % of total endocardial area, was significantly higher in patients with QRS ≥180ms [4.5% (±2.5) vs 2.8% (±2.4), p=0.014], left and right ventricular systolic dysfunction [4.5% (±3.2) vs 2.8% (±2.3), p=0.016 and 3.5% (±3.0) vs 2.6% (±1.9), p=0.03, respectively], premature ventricular contractions (PVCs) [3.2% (±2.6) vs 2.2% (±1.8), p<0.05], exercise-induced PVCs [3.8% (±2.4) vs 2.6% (±2.2), p=0.01], previous shunt [4.0% (±2.7) vs 2.6% (±2.2), p=0.01] and reintervention [4.2% (±3.2) vs 2.6% (±2.0), p=0.008]. Scar size also showed a positive correlation with duration of post-surgical follow-up (ρ=0.01), age at correction (ρ=0.01) and absolute QRS duration (ρ=0.05). CONCLUSIONS Patients with rCHD involving the right ventricle show electrical scars with variable distribution, not necessarily matching with sites of surgical lesions. Scar extension correlates with some of the risk factors for life-threatening arrhythmias in CHD, such as prolonged QRS. Thus EVM could be considered an additional tool in the assessment of risk stratification in this particular population.

Use of a Pediatric Syncope Unit Improves Diagnosis and Lowers Costs: A Hospital-Based Experience

Objectives To assess the effect of a dedicated pediatric syncope unit on the diagnostic and therapeutic management of children with suspected syncope. We also evaluated the effectiveness of the pediatric syncope unit model in decreasing unnecessary tests and hospitalizations, minimizing social costs, and improving diagnostic yield. Study design This single‐center cohort observational, prospective study enrolled 2278 consecutive children referred to Bambino Gesù Childrens Hospital from 2012 to 2017. Characteristics of the study population, number and type of admission examinations, and diagnostic findings before the pediatric syncope unit was implemented (2012‐2013) and after the pediatric syncope unit was implemented (2014‐2015 and 2016‐2017) were compared. Results The proportion of undefined syncope, number of unnecessary diagnostic tests performed, and number of hospital stay days decreased significantly (P < .0001), with an overall decrease in costs. A multivariable logistic regression analysis, adjusted for confounding variables (age, sex, number of diagnostic tests), the period after pediatric syncope unit (2016‐2017) resulted as the best independent predictor of effectiveness for a defined diagnosis of syncope (P < .0001). Conclusions Pediatric syncope unit organization with fast‐tracking access more appropriate diagnostic tests is effective in terms of accuracy of diagnostic yield and reduction of costs.