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.

Lyme carditis presenting as paroxysmal junctional tachycardia and complete atrioventricular block in an adolescent.

We describe a case of a previously healthy adolescent who presented with junctional tachycardia and complete atrioventricular (AV) block due to Lyme carditis. The simultaneous presence of these findings suggested significant inflammation of the AV junction. Junctional tachycardia, particularly if seen in a patient with conduction abnormalities and potential tick exposure, should increase suspicion for Lyme carditis.

Electrocardiogram changes in pediatric patients with myocarditis.

BACKGROUND: It is traditionally taught that pediatric patients with myocarditis almost always have an abnormal electrocardiogram (ECG) at presentation. However, there has never been a study to objectively evaluate ECG changes in pediatric myocarditis patients compared to healthy controls or explore if specific ECG changes correlate with clinical outcomes. METHODS: Pediatric patients diagnosed with acute myocarditis were age and sex matched 1:2 with healthy controls in this retrospective case-control study spanning a seven-year period. ECGs from presentation through discharge were interpreted by electrophysiologists blinded to the patients' diagnoses. RESULTS: Thirty-nine patients with myocarditis were identified. Twenty-eight (72%) had an abnormal ECG at presentation, 11 (28%) had a completely normal ECG. In this second group, six patients had an abnormal ECG at some point during their hospital course for a total of 34 (88%). Myocarditis patients who had an abnormal ECG at presentation spent more time in the hospital, 5 (2-19) versus 2 ((1-3) days (p < 0.01), and in the ICU, 1 (0-6) versus 0 (0-1) days (p < 0.01). Myocarditis patients were more likely to have ST elevation on their ECG compared to control patients (41% versus 17%, p < 0.01). Patients with ST elevation at presentation had a higher peak troponin level, 18.4 (5.8-31.0) versus 7.7 (0-19.1) ng/ml, (p < 0.01). CONCLUSIONS: Over a quarter of patients with myocarditis had a normal ECG at presentation to the emergency department. Patients with an abnormal ECG at presentation spent more time in the hospital. The presenting ECG, particularly the presence of ST elevation, may correlate with other clinical markers and help direct early management decisions.

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.

Paroxysmal complete atrioventricular block in pediatric heart transplant recipients following cardiac catheterization: A case series.

Late-onset paroxysmal AVB has been described as a rare complication after HT and has been associated with AR or CAV. We describe 4 pediatric HT recipients who developed paroxysmal AVB hours after routine cardiac catheterization in the absence of AR, CAV, or underlying conduction system disease. Four pediatric HT recipients who were >1 year post-transplant had episodes of paroxysmal AVB hours after surveillance cardiac catheterization with EMB. Telemetry demonstrated high-grade block, ranging from 2:1 AVB to complete AVB without ventricular escape for several seconds. None of the patients had significant AR or rapidly progressive CAV. Supplemental testing did not reveal underlying conduction system disease. Three of the 4 patients received permanent pacemakers, although subsequent interrogations showed minimal ventricular pacing. These pediatric HT recipients had paroxysmal AVB hours after cardiac catheterization in the absence of significant AR, CAV, or underlying conduction system disease. Subsequent pacemaker interrogations showed minimal ventricular pacing, suggesting these were isolated episodes. These cases suggest that mechanisms in addition to AR and CAV may cause paroxysmal AVB in pediatric HT recipients, warranting further investigation.

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.

Airway ciliary dysfunction and respiratory symptoms in patients with transposition of the great arteries.

BACKGROUND: Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning. As CHD comprising transposition of the great arteries (TGA) is commonly thought to involve disturbance of left-right patterning, especially L-TGA with left-right ventricular inversion, we hypothesize CHD patients with transposition of great arteries (TGA) may have high prevalence of airway CD with increased respiratory symptoms. METHODS AND RESULTS: We recruited 75 CHD patients with isolated TGA, 28% L and 72% D-TGA. Patients were assessed using two tests typically used for evaluating airway ciliary dysfunction in patients with primary ciliary dyskinesia (PCD), a recessive sinopulmonary disease caused by respiratory ciliary dysfunction. This entailed the measurement of nasal nitric oxide (nNO), which is typically low with PCD. We also obtained nasal scrapes and conducted videomicroscopy to assess respiratory ciliary motion (CM). We observed low nNO in 29% of the patients, and abnormal CM in 57%, with 22% showing both low nNO and abnormal CM. No difference was observed for the prevalence of either low nNO or abnormal ciliary motion between patients with D vs. L-TGA. Respiratory symptoms were increased with abnormal CM, but not low nNO. Sequencing analysis showed no compound heterozygous or homozygous mutations in 39 genes known to cause PCD, nor in CFTR, gene causing cystic fibrosis. As both are recessive disorders, these results indicate TGA patients with ciliary dysfunction do not have PCD or cystic fibrosis (which can cause low nNO or abnormal ciliary motion). CONCLUSIONS: TGA patients have high prevalence of abnormal CM and low nNO, but ciliary dysfunction was not correlated with TGA type. Differing from PCD, respiratory symptoms were increased with abnormal CM, but not low nNO. Together with the negative findings from exome sequencing analysis, this would suggest TGA patients with ciliary dysfunction do not have PCD but nevertheless may suffer from milder airway clearance deficiency. Further studies are needed to investigate whether such ciliary dysfunction is associated with increased postsurgical complications as previously observed in CHD patients with heterotaxy.

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.

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.

Use of three-dimensional mapping in young patients decreases radiation exposure even without a goal of zero fluoroscopy.

At present, three-dimensional mapping is often used during cardiac ablations with an explicit goal of decreasing radiation exposure; three-dimensional mapping was introduced in our institution in 2007, but not specifically to decrease fluoroscopy time. We document fluoroscopy use and catheterisation times in this setting. Data were obtained retrospectively from patients who underwent ablation for atrioventricular nodal re-entrant tachycardia from January, 2004 to December, 2011. A total of 93 patients were included in the study. Among them, 18 patients who underwent radiofrequency ablation without three-dimensional mapping were included in Group 1, 13 patients who underwent cryoablation without three-dimensional mapping were included in Group 2, and 62 patients who underwent cryoablation with three-dimensional mapping were included in Group 3. Mean fluoroscopy times differed significantly (34.3, 23.4, and 20.3 minutes, p<0.001) when all the groups were compared. Group 3 had a shorter average fluoroscopy time that did not reach significance when compared directly with Group 2 (p=0.29). An unadjusted linear regression model showed a progressive decrease in fluoroscopy time (p=0.002). Mean total catheterisation times differed significantly (180, 211, and 210 minutes, p=0.02) and were related to increased ablation times inherent to cryoablation techniques. Acute success was achieved in 89, 100, and 97% of patients (p=0.25), and chronic success was achieved in 80, 92, and 93% of patients (p=0.38). Complication rates were similar (17, 23, and 7%, p=0.14). In conclusion, three-dimensional mapping systems decrease fluoroscopy times even without an explicit goal of zero fluoroscopy. Efficacy and safety of the procedure have not changed.

Ventricular tachycardia in a child with diabetic ketoacidosis without heart disease.

Ventricular tachycardia is uncommon in children without CHD. We present the case of a 15-year-old boy who presented with severe diabetic ketoacidosis and ventricular tachycardia and was not responsive to traditional anti-arrhythmic therapy. This case highlights the importance of identification of the underlying metabolic derangement causing the arrhythmia to provide appropriate management.

Accelerated Idioventricular Rhythm in a Child With Status Asthmaticus.

Acute asthma exacerbations occur relatively frequently in children. We present the case of a 4-year-old boy who was admitted to our hospital in status asthmaticus and found to have a wide complex rhythm while being treated with inhaled albuterol and intravenous methylprednisolone. This rhythm was diagnosed as accelerated idioventricular rhythm (AIVR), which carries a benign prognosis. It resolved as the medications used to treat his asthma exacerbation were weaned. There was no ventricular ectopy seen on a 24-hour Holter monitor performed 3 months after his hospitalization, suggesting that the AIVR was related to the medications the patient was receiving at the time. This case suggests that albuterol may be a risk factor for the development of AIVR and highlights the importance of recognizing this rhythm to avoid unnecessary and potentially harmful therapies.

Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association.

BACKGROUND: Acute rheumatic fever remains a serious healthcare concern for the majority of the world's population despite its decline in incidence in Europe and North America. The goal of this statement was to review the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and to update those criteria to also take into account recent evidence supporting the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever. METHODS AND RESULTS: To achieve this goal, the American Heart Association's Council on Cardiovascular Disease in the Young and its Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee organized a writing group to comprehensively review and evaluate the impact of population-specific differences in acute rheumatic fever presentation and changes in presentation that can result from the now worldwide availability of nonsteroidal anti-inflammatory drugs. In addition, a methodological assessment of the numerous published studies that support the use of Doppler echocardiography as a means to diagnose cardiac involvement in acute rheumatic fever, even when overt clinical findings are not apparent, was undertaken to determine the evidence basis for defining subclinical carditis and including it as a major criterion of the Jones criteria. This effort has resulted in the first substantial revision to the Jones criteria by the American Heart Association since 1992 and the first application of the Classification of Recommendations and Levels of Evidence categories developed by the American College of Cardiology/American Heart Association to the Jones criteria. CONCLUSIONS: This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.

Radiofrequency energy ablation in a child with an implanted vagus nerve stimulator.

An 8-year-old girl with supraventricular tachycardia and an implanted vagus nerve stimulator underwent radiofrequency ablation of her supraventricular tachycardia substrate. No known literature exists addressing the potential interaction of these two technologies, although there are reported cases of interaction between radiofrequency and other implanted stimulating devices such as pacemakers. The procedure was performed successfully without observed interaction, and the patient's family reported no significant change in frequency of seizure control.

Outcomes of pediatric patients undergoing cardiac catheterization while on extracorporeal membrane oxygenation.

The aim of the study is to explore the indications for cardiac catheterization while on extracorporeal membrane oxygenation (ECMO) and the various catheter interventions performed as well as assess the safety profile and determine the short- and intermediate-term survival. ECMO is a lifesaving intervention for pediatric patients with respiratory and/or cardiovascular failure. There is limited recent literature discussing the survival and outcomes of patients undergoing cardiac catheterization while on ECMO. A retrospective review of consecutive patients undergoing catheterization while on ECMO from 2004 to 2013 was performed. Thirty-six patients who underwent 40 cardiac catheterizations were identified. Indications for catheterization included hemodynamic/anatomic assessment of postoperative (16) and non-operative patients (7), planned catheter interventions (CI) (12), and cardiomyopathy assessment (5). CI were performed during 18 (45 %) catheterizations, including stenting of vessels/surgical shunts (9), balloon atrial septostomy (4), device closure of septal defects/vessels (3), thrombolysis of vessels (2), endomyocardial biopsy (2), and temporary pacer wire placement (1). Unexpected diagnostic information was found in 21 (52 %), and 13 patients were referred for surgical intervention. Successful decannulation was achieved in 86 % of patients. Survival to discharge was 72 % and intermediate survival was 69 % (median = 29 months). Survival was 88 % (15/17) among patients who underwent CI. There were six procedural complications (15 %); five vascular and one non-vascular. There were no complications related to patient transport. Cardiac catheterization and interventions while on ECMO are safe, with a survival to discharge of 72 %. Diagnostic information obtained from catheterization leads to management decisions which may impact survival.

Ventricular ectopy in children without known heart disease.

OBJECTIVE: To describe the presentation and clinical course of patients with ventricular ectopy (VE) without known heart disease seen at a single institution. STUDY DESIGN: Patients with VE were identified from the cardiology database. Patients with known hemodynamically significant heart disease or systemic diseases were excluded. RESULTS: A total of 219 patients constitute the study population, with 59% male and median age of diagnosis 11.3 years. A total of 138 patients had follow-up data. Median duration of follow-up was 3.1 years (n = 138, range 0-21 years) for a total of 587 patient-years. Simple VE was found in 83%, and 17% had ventricular tachycardia. Most patients were asymptomatic at presentation (77%) At presentation, echocardiograms were performed in 164 patients, with 98% normal. Of the 36 patients with sequential echocardiograms, 32 (88%) remained normal, 3 (9%) had abnormal echocardiograms which normalized, and 1 (3%) had progressive left ventricular dysfunction. On sequential Holter data (n = 48), 54% showed stable or decreased VE, 40% showed resolution, and 6% showed worsening. No cases of death or resuscitated sudden death occurred. CONCLUSIONS: Most patients were asymptomatic. There were rare cases of progression of VE and development of left ventricular dysfunction but the majority had stable findings. No deaths occurred.

Electrolyte screening in the evaluation of prolonged QTc interval.

We present a case of a previously healthy adolescent male for outpatient evaluation of prolonged QT interval. He was ultimately found to have acquired QT interval prolongation secondary to hypocalcaemia related to undiagnosed hypoparathyroidism. This case report highlights the importance of routine electrolyte analysis, even in the outpatient setting, during initial diagnostic workup for QT interval prolongation.