A Case of Persistent Junctional Reciprocating Tachycardia Coincident With Cor Triatriatum Sinister.

Persistent junctional reciprocating tachycardia is a rare form of refractory atrioventricular reentrant tachycardia that accounts for <1% of supraventricular tachycardia in pediatrics. The accessory pathways are generally isolated with few reported underlying structural heart defects. We present a case of a five-month-old male with refractory tachyarrhythmia found to have cor triatriatum sinister, which to our knowledge, is the first reported case of these two rare anomalies coexisting.

Double outlet right ventricle.

This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle.

Guidewire pacing during transcatheter aortic valve implantation in a patient with complex congenital heart disease.

We present a case of temporary guidewire pacing in a patient with Fontan anatomy during transcatheter aortic valve implantation. Temporary pacing was successfully achieved utilizing this method without complications. There is an increasing population of patients with complex congenital heart disease and expanding variety of transcatheter interventions. Due to limitations in vascular access and surgical anatomies, guidewire pacing may have a wide array of potential applications in pediatrics and the congenital heart disease population.

Utility and safety of adenosine challenge for subtle ventricular pre-excitation in the pediatric population.

INTRODUCTION: Adenosine challenge (AC) can be used to evaluate possible Wolff-Parkinson-White (WPW) pattern on an electrocardiogram (ECG). Despite the use of this technique, there is a paucity of studies in the pediatric population evaluating the efficacy, safety, and outcomes of this testing modality. METHODS AND RESULTS: All ACs performed from January 2009 to June 2017 were retrospectively reviewed. Patient demographics, adenosine dosing, results, adverse effects, and outcomes including results of electrophysiology studies (EPS) were reviewed. Analysis was conducted between AC positive and negative cohorts. ECG criteria of shortest PR interval, longest QRS duration, and the number of suspected pre-excited leads were evaluated for inter-rater reliability and correlation to positive AC. Fifty-six AC (n = 51) were performed (median age, 13.8; range, 0.3-20 years). Forty-one AC were pre-EPS and 15 post-EPS due to concern for recurrent WPW. Thirty-one (76%) pre-EPS AC were negative, 9 (22%) positive, and 1 (2%) equivocal. EPS was performed following seven positive AC revealing 5 (71%) left posterior and 2 (29%) right posteroseptal AP. The 15 post-EPS AC were all found to be negative. Mean effective adenosine dose was 0.2 ± 0.11 mg/kg. No adverse events were reported. Mean follow up duration after AC was 314 ± 482 days with no documented arrhythmias. CONCLUSION: Adenosine challenge is an effective and safe testing modality for subtle WPW in the pediatric population. In our population, there were no adverse events or documented arrhythmias in patients following a negative study.

Marked First-Degree Atrioventricular Block and Pseudo-Pacemaker Syndrome in a Pediatric Patient.

We describe a 17-year-old female who presented with 3 weeks of abdominal pain, exercise intolerance, and an episode of altered mental status found to have marked first-degree atrioventricular block. Exercise stress test and cardiac catheterization demonstrated pseudo-pacemaker syndrome, and a permanent pacemaker was placed. Following placement, she has resolution of symptoms and markedly improved exercise tolerance.

Automated QT analysis on Holter monitors in pediatric patients can differentiate long QT syndrome from controls.

BACKGROUND: Borderline QTc is a common referral to the pediatric cardiology clinic. Evaluation is challenging due to significant overlap of normal and abnormal QTc ranges. We hypothesized that automated QT analysis on Holter could differentiate between patients with long QT syndrome (LQTS) and healthy controls. METHODS: We conducted a retrospective review of 39 patients with known genotype-positive, phenotype-positive LQTS who underwent Holter monitoring between January 2010 and January 2016. They were compared 2:1 to age- and sex-matched controls. Automated QT analysis data were analyzed. RESULTS: Significant differences were found in all automated QT and QTc fields, except minimum QTc interval (P = 0.57). Mean QTc interval (LQTS 479 ± 28 ms vs controls 429 ± 16 ms; P ≤ 0.001) and percent QTc intervals (%QTc) >450 ms (LQTS 80 ± 28% vs controls 14 ± 16%; P ≤ 0.001) were selected for further analysis. A receiver operating characteristic curve was generated for each variable demonstrating high area under the curve values of 0.9494 and 0.9540, respectively. Threshold values of ≥461 ms for mean QTc (sensitivity 79.49%, specificity 98.72%) and ≥65% of %QTc >450 ms (sensitivity 79.49%, specificity 98.72%) allowed highly specific discrimination between cohorts (false positive rate 1.09%). Similarly, thresholds of <434 ms (sensitivity 97.44, specificity 61.54) for mean QTc and <32% (sensitivity 89.74, specificity 87.18) for %QTc >450 ms resulted in highly sensitive discrimination (false negative rates 2.17% and 8.7%). CONCLUSION: Holter monitor testing with automated QT analysis may be a useful tool to differentiate LQTS and control patients.

Adenosine-sensitive Wolff-Parkinson-White: Longer time across the atrioventricular groove.

BACKGROUND: Successful ablation sites in Wolff-Parkinson-White syndrome (WPW) are characterized by short atrioventricular (AV) intervals. Approximately 15% of patients with WPW have adenosine-sensitive accessory pathways (APs). We sought to determine if local AV intervals of adenosine-sensitive APs are different from those of adenosine-insensitive APs in patients with WPW. METHODS: Patients ≤21 years with WPW and adenosine-sensitive APs who underwent successful ablation over a 9-year period were included. Patients with WPW and adenosine-insensitive APs were matched by age and weight in a 1:2 case-control design. AP location, antegrade and retrograde conduction properties, supraventricular tachycardia (SVT) inducibility, local AV interval, interval from delta wave onset to local ventricular activation (del-V), and time to loss of preexcitation were reviewed. RESULTS: Fourteen patients with adenosine-sensitive APs and 28 with adenosine-insensitive APs were included. Patients with adenosine-sensitive APs had minimum 1:1 antegrade AP conduction at a longer median paced cycle length (380, interquartile range [IQR] 295 to 585 ms vs 290, IQR 250 to 330 ms, P = 0.046), were less likely to have inducible SVT (35.7% vs 75.0%, P = 0.035), and had a longer median local AV interval (40.5, IQR 30.8 to 58.3 ms vs 32.0, IQR 29.3 to 37.8 ms, P = 0.029) when compared to those with adenosine-insensitive APs. CONCLUSION: Patients with WPW and adenosine-sensitive APs have 1:1 antegrade AP conduction at longer cycle lengths, lower likelihood of SVT induction, and longer local AV intervals when compared to those with adenosine-insensitive APs. In patients with WPW, it may be important to consider adenosine response when selecting appropriate ablation targets.

Effect of household children on adult ED smokers' motivation to quit.

OBJECTIVE: We hypothesized adult parenting smokers in the emergency department (ED) have a higher interest in quitting and may be more amenable to tobacco cessation counseling than smokers without children. STUDY DESIGN: Cross-sectional survey study of adult smokers in 8 US academic EDs. RESULTS: One thousand one hundred sixty-eight smokers enrolled, 441 (37.8%) with household children (total of 973 exposed children). Compared to smokers without household children, smokers with children were younger (mean age, 37.4 vs 42.8 years), more female (60.3% vs 40.3%), and nonwhite (57.5% vs 44.5%) (all P < .006). Groups did not differ in nicotine addiction (median Fagerstrom score, 4 vs 4; P = .31). Parenting smokers were more interested in quitting (mean Ladder of Contemplation score, 4.8 vs 5.1 [P = .02]), felt it more important to quit (median score, 9 vs 8 [P = .01]), and more confident to quit (7 vs 6 [P = .004]) than nonparenting smokers. Smoking inside the home was banned by 45% of smokers with children vs 30% without household children (P < .001). CONCLUSIONS: Adult ED parenting smokers are interested in quitting and taking steps to limit their children's secondhand smoke exposure. Asking adult ED smokers about household children may enhance motivation to quit.