Drug-Induced Loss of Preexcitation in Pediatric Patients with WPW Pattern During Electrophysiologic Study.

Ablation of accessory pathways (AP) is one of the most often performed procedures in pediatric electrophysiology. In pediatric patients these procedures are mostly performed in anaesthesia or sedation. In some of these patients who are referred for electrophysiologic (EP) study, we could observe disappearance of the preexcitation, i.e. antegrade conduction of an AP during introduction of sedation. As a suppression of AP conduction capacities has been reported as negative side effect of propofol and other anaesthetics, the aim of this study was to evaluate risk factors for drug-induced suppression of AP conduction properties. Consecutive, pediatric patients with Wolff-Parkinson-White (WPW) pattern referred for EP study in the period of 2016-2017 were reviewed in retrospect. Patients with complex congenital heart disease were excluded. An entire chart review including ECG, bicycle stress testing, and periprocedural data was performed. In 4 of 37 patients included into the study, loss of preexcitation could be observed during sedation. Data analysis showed weaker conduction capacities of the AP as a risk factor (p = 0.009). Interestingly, absolute (p = 0.11) or adjusted to body weight (p = 0.92) drug doses were not a relevant risk factor. Patients with WPW and weaker conduction capacities of the AP, as implied by an early disappearance of preexcitation during exercise stress testing, seem to be more prone to drug-induced suppression of an AP.

Características electrocardiográficas de vías fascículo-ventriculares en pediatría: estudio comparativo con las vías accesorias anteroseptalesderechas / Electrocardiographic characteristics of fasciculo-ventricular accessory pathways in children: A comparative study with right anteroseptal accessory pathways

Resumen Objetivos: Las vías accesorias (VAc) fascículo-ventriculares (FV) tienen una localización anatómica similar a las VAcanteroseptales derechas (ASD) y comparten características electrocardiográficas. El objetivo es comparar características electrocardiográficas de las VAC FV con las de las ASD en pediatría. Métodos: Se incluyeron pacientes con preexcitación manifiesta sometidos a estudio electrofisiológico. Las VAc FV se definieron por un intervalo HV ≤ 32ms y un alargamiento del AH sin modificación del HV, del grado o patrón de preexcitación ventricular durante la estimulación auricular. Tres observadores independientes y ciegos analizaron los ECG en cada grupo. Resultados: De 288 pacientes, 15 (5.2%) presentaban VAC FV y 14 VAC ASD (4.9%). El intervalo PR fue más largo en las VAc FV que en las ASD (113 ± 21 vs. 86 ± 13 ms respectivamente; p = < 0.001) y la duración del QRS fue menor (95 ± 12 vs. 137 ± 24 ms respectivamente; p = < 0.001). El ECG de las VAc FV presentó una deflexión rápida de baja amplitud previa al inicio del QRS en 13 de 15 pacientes (87%) y en 2 con VAc AV ASD (14%); (p = 0.003). Conclusiones: El intervalo PR fue más largo y el complejo QRS más angosto en la VAC FV respecto de las ASD. La presencia de una deflexión rápida de baja amplitud previa al inicio del QRS permitiría diferenciarlas de las aurículo-ventriculares ASD de manera no invasiva.

Abstract Objectives: Fasciculo-ventricular (FV) accessory pathways (AP's) and right anteroseptal (RAS) AP's share similar anatomic locations and electrocardiographic characteristics. The objective of this article is to compare these features in children. Methods: All patients with manifest pre-excitation who underwent an electrophysiological study were included. Fasciculo-ventricular AP's were defined by the presence of an HV inter- val ≤ 32 ms and a prolongation of the AH without changes in the HV interval, or the level of pre-excitation during atrial pacing. Three independent and blind observers analysed the ECG's in both groups. Results: Out of 288 patients, 15 (5.2%) had FV AP's and 14 (4.9%) right AS AP's. The PR interval was longer in FV AP's than in RAS (113 ± 21 vs 86 ± 13 ms respectively; P < .001) and the QRS was narrower (95 ± 12 vs 137 ± 24 ms respectively; P < .001). The ECG in patients with FV AP's showed a rapid low amplitude deflection at the begining of the QRS in 13 out of 15 patients (87%) and in 2 (14%) the RAS AP group (P = .003). Conclusions: The PR interval was longer and the QRS complex was narrower in patients with FV AP's. The presence of a rapid low amplitude deflection at the beginning of the QRS complex would allow to differentiate them from RAS AP's non-invasively.

[Electrocardiographic characteristics of fasciculo-ventricular accessory pathways in children: A comparative study with right anteroseptal accessory pathways]. / Características electrocardiográficas de vías fascículo-ventriculares en pediatría: estudio comparativo con las vías accesorias anteroseptales derechas.

OBJECTIVES: Fasciculo-ventricular (FV) accessory pathways (AP's) and right anteroseptal (RAS) AP's share similar anatomic locations and electrocardiographic characteristics. The objective of this article is to compare these features in children. METHODS: All patients with manifest pre-excitation who underwent an electrophysiological study were included. Fasciculo-ventricular AP's were defined by the presence of an HV interval≤32ms and a prolongation of the AH without changes in the HV interval, or the level of pre-excitation during atrial pacing. Three independent and blind observers analysed the ECG's in both groups. RESULTS: Out of 288 patients, 15 (5.2%) had FV AP's and 14 (4.9%) right AS AP's. The PR interval was longer in FV AP's than in RAS (113±21 vs 86±13ms respectively; P<.001) and the QRS was narrower (95±12 vs 137±24ms respectively; P<.001). The ECG in patients with FV AP's showed a rapid low amplitude deflection at the begining of the QRS in 13 out of 15 patients (87%) and in 2 (14%) the RAS AP group (P=.003). CONCLUSIONS: The PR interval was longer and the QRS complex was narrower in patients with FV AP's. The presence of a rapid low amplitude deflection at the beginning of the QRS complex would allow to differentiate them from RAS AP's non-invasively.

Characteristic features of patients with multiple accessory pathways.

Objective Only limited clinical and electrophysiological data concerning patients (pts) with multiple accessory pathways (MAP) in comparison to large control groups are available. The aim of our study was to analyse these data from the largest cohort of patients with multiple accessory pathways and a large control group. Method and results We analysed data from pts with MAP (group 1) and pts with a single accessory pathway (AP) (group 2) referred for radiofrequency catheter ablation (RFCA) at our tertiary centre. Group 1 consisted of 124 pts (M 62.10%, mean age 33.00 ± 5.26) with MAP and RFCA. Group 2 consisted of 376 pts (M 51.20%, mean age 35.87 ± 16.15) with a single accessory pathway and RF ablation. Group 1 exhibited a higher incidence of overt APs (P < 0.0001), Ebstein anomaly (P = 0.001), ventricular fibrillation (P = 0.012), antidromic atrioventricular re-entrant tachycardia (A AVRT) (P = 0.025) and male gender (P = 0.038). The mean age at the first documented atrioventricular re-entrant tachycardia (AVRT) episode was lower in pts with MAP than in pts with single APs: 16.79 ± 13.41 vs 20.84 ± 14.29, respectively (P = 0.001). Concealed accessory pathways (P < 0.0001) occurred more frequently in the control group. Group 1 had more right-lateral (P = 0.0001), mid-septal (P = 0.0001), left-posterior (P = 0.01), left-anterior (P = 0.013) and left-lateral localizations of AP (P < 0.037). Conclusions The MAP group included statistically significantly more men, Ebstein anomaly and overt APs. The mean age of the first episode of atrioventricular re-entrant tachycardia was lower in pts with MAP. Certain distribution patterns are apparent for single and MAP. Pts with MAP are at higher risk of VF and antidromic atrioventricular re-entrant tachycardia.

Long term risk of Wolff-Parkinson-White pattern and syndrome.

For years, conventional wisdom has held that patients with asymptomatic ventricular pre-excitation (asymptomatic WPW or WPW pattern) were at low risk for adverse outcomes. This assumption has been challenged more recently in a number of observational/natural history studies as well as in prospective trials in which patients were more aggressively studied via invasive electrophysiology study (EPS) and more aggressively treated, in some cases, with pre-emptive catheter ablation, despite the lack of symptoms. In sum, the data do not definitively support one approach (early, up-stream EPS and/or ablation) vs. the other (watchful waiting with close monitoring). The most recent pediatric and adult guidelines reflect this ambiguity with a broad spectrum of approaches endorsed.

Cardiac arrhythmias in patients with Danon disease.

AIMS: Different cardiac arrhythmias have been suggested to be associated with Danon disease, e.g. Wolff-Parkinson-White syndrome. However, a systematic electrophysiological investigation of patients with Danon disease is lacking thus far. METHODS AND RESULTS: Seven patients with Danon disease (4 males, 35.8 ± 10.8 years; 3 females, 51.3 ± 19.9 years) from 3 different families were studied. In all patients, the presence of Danon disease was confirmed by western blot of biopsy material or genetic testing. The patients were characterized by 12-lead electrocardiogram (ECG), Holter ECG, echocardiography, and serial implantable cardioverter defibrillator (ICD) interrogations (in ICD recipients). All male patients underwent electrophysiological investigation (EP study). Asymptomatic ventricular tachyarrhythmias were documented in six of the seven patients. Moreover, 5 of the 7 patients suffered from atrial fibrillation (AF), with 1 of them experiencing thromboembolic stroke at the age of 30 years. In male patients, the initial QRS complex was characterized by a slurring upstroke and shortened PQ interval mimicking ventricular pre-excitation. One male patient showed initial QRS complex slurring with prolonged PR interval. However, the presence of an accessory pathway was excluded by an EP study in all patients. In female patients, initial QRS complex slurring was significantly less distinct. In four patients, ICD implantation was performed for primary prevention of sudden cardiac death. However, sustained ventricular arrhythmias were not documented in any of the patients. CONCLUSIONS: The present study indicates that the distinct surface ECG pattern in Danon disease is not associated with ventricular pre-excitation. Atrial fibrillation is frequently observed in these patients and may be associated with thromboembolic events in the young, while sustained ventricular arrhythmias occur less frequently than previously reported.

Harder Roads to Trek? Paradoxical Slowing of an Atrioventricular Reentrant Tachycardia With Contralateral Bundle Branch Block.

Slowing of the tachycardia with increase in cycle length and increase in VA time with ipsilateral bundle branch block is a characteristic feature of accessory pathway mediated AVRT. Contralateral bundle branch block has no effect on the tachycardia as it is not a part of the tachycardia circuit. We present an interesting phenomenon in which contralateral bundle branch block resulted in tachycardia slowing in a case of WPW syndrome.

Depression masquerading as chest pain in a patient with Wolff Parkinson White syndrome.

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire-9 (PHQ-9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology.

Depression masquerading as chest pain in a patient with Wolff Parkinson White syndrome

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire–9 (PHQ–9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology.

Depression masquerading as chest pain in a patient with Wolff Parkinson White syndrome

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire–9 (PHQ–9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology.

Electrophysiological Characteristics and Radiofrequency Catheter Ablation of Accessory Pathway Connecting the Right Atrial Appendage and the Right Ventricle.

BACKGROUND: The accessory pathway (AP) connecting the right atrial appendage (RAA) and the right ventricle (RV) is rare. OBJECTIVE: We sought to investigate the feature of the AP connecting the RAA and the RV and the efficacy of radiofrequency catheter ablation via the endocardial access. METHODS: We retrospectively analyzed 14 consecutive patients with 14 APs connecting the RAA and the RV managed by 15 procedures between January 2003 and December 2014. RESULTS: Ten patients presented as preexcitation during sinus rhythm. All APs had retrograde conduction. None had either antegrade or retrograde decremental conduction property. Ablation targeting the sites at the tricuspid annulus failed in all patients. They were successfully managed by ablating the atrial insertion sites with a median of 10.5 (range 5-28) radiofrequency applications. Electrograms at the successful target showed high amplitude atrial electrogram and low amplitude or no ventricular electrogram. The atrial insertion sites were at the floor of the RAA in 10 patients and inside the lower lobe of the RAA in the remaining 4 patients. The shortest distance between the successful target and the tricuspid annulus in the right anterior oblique projection was 19.7 ± 4.0 mm. There were no complications or recurrences during a median follow-up period of 4.3 (range 0.2-11.8) years. CONCLUSION: The APs connecting the RAA and the RV had typical conduction properties. The atrial insertion site favored the floor and the lower lobe of the RAA. Ablation targeting the atrial insertion sites was effective and safe, albeit multiple radiofrequency applications were needed.

Frequency of fractionated ventricular activation and atrial/ventricular electrogram amplitude ratio at successful ablation target of accessory pathways in patients with Ebstein's anomaly.

INTRODUCTION: Knowledge about local electrogram and atrial/ventricular electrogram amplitude ratio (A/V ratio) at the true atrioventricular ring for successful ablation of accessory pathways (APs) in patients with Ebstein's anomaly is limited. METHODS: Twenty-two adults with Ebstein's anomaly and APs were managed by 24 sessions of radiofrequency catheter ablation (RFCA). A right atrial/ventricular angiogram with or without 3-dimensional electroanatomic mapping was performed to delineate the true atrioventricular ring and atrialized right ventricle (ARV). Electrograms of successful targets were analyzed. For each right-sided AP target, 2 AP targets matched by location and conduction property from normal hearts were selected as controls, and their A/V ratios were compared. RESULTS: In 32 right-sided APs of all 22 patients, local ventricular activation at successful site presented as normal electrogram in 14, fractionated ventricular electrogram (FVE) in 16, and double ventricular potentials in 2. The bipolar voltage of ARV was markedly lower than in normal hearts. The A/V ratio at the successful target was significantly higher in APs with FVE than in control (1.64 ± 1.34 vs. 0.47 ± 0.19, P < 0.01), and was similar in APs with normal ventricular electrogram and in the control (0.38 ± 0.15 vs. 0.46 ± 0.15, P = 0.1726). The A/V ratio at the successful target with double ventricular potentials (after surgical correction) was 0.30 ± 0.20. All APs were successfully ablated without complications. CONCLUSIONS: In patients with Ebstein's anomaly, FVE was found in 50% of the successful targets of APs. High A/V ratio at successful sites with FVE and normal A/V ratio at targets with normal ventricular electrogram can facilitate target recognition and ablation.

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.