We report a case of a 6-year-old boy who developed intra-atrial re-entrant tachycardia after percutaneous atrial septal defect closure. Ablation was performed, and the circuit of tachycardia was identified. This was a rare complication caused by right atrial enlargement due to an atrial septal defect closure device.

Pulmonary hypertension is not the only cause of arterial desaturation in patients with atrial septal defect. Arterial desaturation can also occur with normal pulmonary artery pressure making it mandatory to understand the mechanism to avoid erroneous diagnosis. In this report, for the first time, we demonstrate atrial flutter as the cause of arterial desaturation in a patient with large atrial septal defect despite normal pulmonary artery pressure, which was normalised following successful radiofrequency ablation.

Noonan syndrome is the second most common genetic syndrome associated with congenital heart disease after Trisomy 21. The two most common cardiac lesions associated with Noonan syndrome are pulmonary stenosis and hypertrophic cardiomyopathy. Although the incidence of mitral valve disease in Noonan syndrome ranges between 2 and 6%, rapid progression of mitral valve dysplasia causing severe mitral valve regurgitation and left atrial dilatation is seldom seen. Most cases of mitral valve disease have been diagnosed either on routine echocardiographic surveillance or when presented with heart failure symptoms. We describe an 18-month-old boy with Noonan syndrome presenting in atrial flutter due to a massively enlarged left atrium caused by severe mitral valve regurgitation which developed and progressed in less than 17 months.

Introduction: For rhythm control of acute atrial flutter (AAFL) in the emergency department (ED), choices include initial drug therapy or initial electrical cardioversion (ECV). We compared the strategies of pharmacological cardioversion followed by ECV if necessary (Drug-Shock), and ECV alone (Shock Only). Methods: We conducted a randomized, blinded, placebo-controlled trial (1:1 allocation) comparing two rhythm control strategies at 11 academic EDs. We included stable adult patients with AAFL, where onset of symptoms was <48 hours. Patients underwent central web-based randomization stratified by site. The Drug-Shock group received an infusion of procainamide (15mg/kg over 30 minutes) followed 30 minutes later, if necessary, by ECV at 200 joules x 3 shocks. The Shock Only group received an infusion of saline followed, if necessary, by ECV x 3 shocks. The primary outcome was conversion to sinus rhythm for ≥30 minutes at any time following onset of infusion. Patients were followed for 14 days. The primary outcome was evaluated on an intention-to-treat basis. Statistical significance was assessed using chi-squared tests and multivariable logistic regression. Results: We randomized 76 patients, and none was lost to follow-up. The Drug-Shock (N = 33) and Shock Only (N = 43) groups were similar for all characteristics including mean age (66.3 vs 63.4 yrs), duration of AAFL (30.1 vs 24.5 hrs), previous AAFL (72.7% vs 69.8%), median CHADS2 score (1 vs 1), and mean initial heart rate (128.9 vs 126.0 bpm). The Drug-Shock and Shock only groups were similar for the primary outcome of conversion (100% vs 93%; absolute difference 7.0%, 95% CI -0.6;14.6; P = 0.25). The multivariable analyses confirmed the similarity of the two strategies (P = 0.19). In the Drug-Shock group 21.2% of patients converted with the infusion. There were no statistically significant differences for time to conversion (84.2 vs 97.6 minutes), total ED length of stay (9.4 vs 7.5 hours), disposition home (100% vs 95.3%), and stroke within 14 days (0 vs 0). Premature discontinuation of infusion (usually for transient hypotension) was more common in the Drug-Shock group (9.1% vs 0.0%) but there were no serious adverse events. Conclusion: Both the Drug-Shock and Shock Only strategies were highly effective and safe in allowing AAFL patients to go home in sinus rhythm. IV procainamide alone was effective in only one fifth of patients, much less than for acute AF.

Introduction: Atrial fibrillation (AF) and atrial flutter (AFL) are two common arrhythmias that present to the emergency department (ED) and are a major risk factor for stroke. The 2014 Canadian Cardiovascular Society (CCS) guidelines recommend starting oral anticoagulation (OAC) upon ED discharge for patients with CHADS65 scores of ≥1 to reduce stroke risk. The goal of this study was to identify whether the ED patient population presenting with new onset AF/AFL with CHADS65 ≥ 1 are appropriately initiated on OAC by ED physicians. Methods: This was a retrospective chart review (Jan-Dec 2017) of ED visits at two academic hospitals in Ontario. The year 2017 was chosen to allow for adequate time from the publishing of the CCS guidelines for uptake into clinical practice. Inclusion criteria: patients with a new diagnosis of AF/AFL who are discharged by ED physicians. Exclusion criteria: patients with a history of AF/AFL, already on OAC, admitted to hospital, presenting with arrhythmia other than AF/AFL, and charts without adequate information to calculate CHADS65 score. Charts were reviewed in detail to assess CHADS65 score, ED physician decision to prescribe OAC, referral rates to outpatient clinics and timing of follow up. Results: A total of 1272 charts were reviewed. 1124 were excluded. 148 charts were identified as patients with new onset AF/AFL presenting to the ED who were discharged by ED physicians. 24/148 (16%) were appropriately prescribed OAC. 124/148 (84%) were not prescribed OAC. Of these 40/124 (32%) were CHADS65 0 while the other 84/124 (67%) were CHADS65 ≥ 1 who should have been considered for OAC. Further review determined that 78/84 (92%) were referred to outpatient clinics for the decision regarding OAC with the mean (SD) number of days to follow up being 11(±15). Importantly 1/84 (1.2%) returned prior to their scheduled appointment with a stroke. Only 6/84 (7%) had no follow up arranged. Conclusion: Overall, we found that the rate of OAC prescription by ED physicians for patients being discharged with a new diagnosis of AF/AFL with a CHADS65 score ≥1 was 16%. This is despite the CCS 2014 recommendation of starting OAC for all patients with a CHADS65 score ≥1. It appears that ED physicians are continuing to defer the decision to prescribe OAC to outpatient clinics. Further projects can explore barriers to application of the CCS guidelines and create knowledge translation tools.

In critically ill children, multi-organ-system disease can influence the choice of antiarrhythmic medication. Intravenous therapy is often necessary. There is a scarcity of paediatric critical-care cases demonstrating the dosing, monitoring, and efficacy of intravenous sotalol. This case demonstrates the effective use of intravenous sotalol in an adolescent with renal, hepatic, and haematological dysfunctions.

Introduction: Rapid atrial fibrillation (AF) and flutter remains a common cause of emergency department (ED) visits. Canadian guidelines recommend a rhythm control strategy for patients presenting to ED within 48 hours of arrhythmia onset or who are anticoagulated. However, up to 70% of patients spontaneously convert within 24 hours, mitigating the need for urgent cardioversion. Moreover, education, risk stratification, appropriate anticoagulation, and follow-up may be challenging in the ED setting. Therefore, direct and rapid linkage to an AF clinic was proposed to address these gaps in care. Methods: A pilot evaluation of a “Short Duration AF Pathway” was performed at Kelowna General Hospital, B.C., from June 2014 to Feb 2015. This care pathway–consisting of a treatment algorithm, ED order set, and referral process–was applied to patients with AF≤48 hours or those who were anticoagulated. Patients received initial rate control medication in the ED and were referred for reassessment in a collaborative cardiologist/nurse practitioner AF clinic and seen within 24 hours. Data was collected prospectively; descriptive statistics are presented. Results: Twenty patients were enrolled during the pilot period. Mean age was 69 (SD=10) years, 6/20 (30%) female, mean CHADS65 score 1.35 (SD=1.1), with 15/20 (75%) CHADS65 ≥ 1. On presentation, 4/20 (20%) were taking anticoagulants and 12/20 (60%) had an AF history. All 20 patients were assessed in the AF clinic within 24 hours of referral. Upon assessment in the AF clinic, 10/20 (50%) had spontaneously converted to sinus rhythm and 5/20 (25%) were electrically cardioverted at the first AF clinic visit. The remaining 5/20 (25%) of patients were reclassified as AF of uncertain duration; one was admitted to hospital, the other four had delayed electrical cardioversion. All patients received education related to AF. No adverse events or readmissions to the ED were reported and 100% of patients with CHADS 65≥1 had received appropriate anticoagulation. Conclusion: A “Short Duration AF Pathway” is a viable alternate approach to immediate cardioversion within the ED. Potential advantages include avoiding unnecessary cardioversion, providing patient education, accessing timely specialty care, and initiating anticoagulation where appropriate.

We are reporting a case of a 37-year-old pregnant woman with a large secundum atrial septal defect with left-to-right shunt and severe pulmonary hypertension. Her atrial septal defect was undiagnosed before this pregnancy. After carefully considering all the options, we repaired her atrial septal defect with an open heart surgical closure at 20 weeks of gestation. A substantial and consistent reduction in pulmonary arterial pressure after the surgery and subsequent uneventful delivery indicate that surgical repair of atrial septal defects is a viable option that should be considered for such patients.

The reentrant circuit of common atrial flutter is known to located in the right atrium between two anatomical barriers. Recent electrophysiologic studies have defined the tricuspid annulus as the anterior barrier, and the terminal crest and its continuation as the eustachian ridge as the posterior barrier. Construction of a bidirectional block to conduction between these two barriers by means of lesions created with radiofrequency current have been shown to be effective in ablating the flutter. We now find that surgical creation of such a block to conduction between the barriers by a simple modification of the atrial incision line is equally effective. In a 6-year-old boy, who was admitted to our hospital for closure of an atrial septal defect and treatment of sustained atrial flutter, the atriotomy was performed perpendicular to the terminal groove and extended towards the tricuspid annulus, placing some crythermal lesions between the end of the incision and the annuals. The special defect was closed using a Dacron patch. The child was free of arrhythmia both during the postoperative stay and over the initial three months of follow-up. We conclude that this simple modification of the atrial incision line provides cure of atrial flutter in children who require atriotomy for repair of congential cardiac anomalies. It may also be beneficial in preventing ‘incisional’ reentrant tachycardia.