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Surgical repair in anomalous aortic origin of a coronary artery aims at mitigating the risk of sudden cardiac death in a subset of patients. The pattern and behavior of electrocardiogram changes in a large cohort of these patients are lacking. We aim to describe postoperative electrocardiogram changes in this population and its clinical implications on follow-up.All patients aged less than 21 years who underwent surgical repair for anomalous aortic origin of a coronary artery between December 2012 and June 2020 at our institution were considered for inclusion. Electrocardiograms were reviewed at 5 defined time intervals, from preoperative to 90-day follow-up, with attention to significant findings of ST-segment changes, abnormal T waves, and pathologic Q waves. The electrocardiogram changes were analyzed for correlation with surgical reintervention and medium-term outcomes.Sixty-two patients met inclusion criteria (median age 13.7 years, 61% male). ST-segment changes in the initial postoperative period were seen in 52 patients (84%), all resolving over time. Abnormal T waves were seen in 19 patients (31%), occurred commonly at the predischarge period, and mostly resolved over time. Pathologic Q waves were observed in only 1 patient and associated with reintervention due to coronary artery stenosis. There was no association between postoperative electrocardiogram changes and inducible myocardial ischemia, ventricular dysfunction, or restriction from exercise at follow-up.ST-segment changes and T-wave abnormalities are commonly seen in the postoperative period after anomalous aortic origin of a coronary artery repair, tend to resolve over time, and are not associated with adverse medium-term outcomes. Pathologic Q waves were associated with the need for early coronary reintervention.

Atrial arrhythmia's (AA) following lung transplant in adults are a well-described clinical finding. In pediatrics, however, there are limited data with some reports suggesting that arrhythmias are rare.We performed a single-center retrospective review of lung transplant recipients from January 2013 to June 2020. A detailed evaluation of clinical characteristics, presence of arrhythmias, and outcomes was completed. Arrhythmias were documented based on inpatient telemetry or remote Holter monitoring. Analyses assessing risk factors for arrhythmias and associations with clinical outcomes were performed.Ninety-one lung transplants were performed in 90 patients. Post-operative AA occurred following 19% transplants. Ectopic atrial tachycardia was seen in 14%, atrial flutter in 2%, and a combination in 2%. The majority of these arrhythmias occurred within the first 45 days post-operatively. Antiarrhythmic treatment was required in 59%, but none required ablation or electrical cardioversion. In patients followed for a year or more, 88% had resolution of their arrhythmia. Arrhythmias were not associated with mortality. In further analysis, however, the presence of arrhythmia was associated with an increased length of ICU stay (median of 12 days (IQR 6, 23) versus 5 days (IQR 4, 9); p = .019) and overall length of hospital stay (median of 26 days (IQR 19, 36) versus 17 days (IQR 19, 36); p = .043).Atrial tachyarrhythmias after lung transplantation are common in the pediatric population and usually occur early. Although they frequently require medical therapy and are associated with longer stays, there is no associated increased mortality. In addition, the arrhythmias typically self-resolve.

Hypoglycemia is a rare but recognized side effect of anti-beta-adrenergic medications (beta-blockers) commonly used to manage arrhythmias in children. In 2014 a commercially available premixed solution of sotalol became available, and the use of oral sotalol in children has increased. Hypoglycemia associated with sotalol use has not been previously reported. Sotalol contains a racemic mixture of its D- and L-enantiomers and although it primarily acts to block Ikr (potassium channels), the L-enantiomer has nonselective anti-beta-adrenergic effects. Common sources such as Lexidrugs® and UpToDate®, used to obtain information regarding adverse drug events and precautions, do not list hypoglycemia as a side effect of sotalol. We report 2 unrelated toddlers with severe hypoglycemia, altered mental status, and seizures with sotalol use requiring hospitalization. In both children, hypoglycemia occurred in morning hours after decreased oral intake. Complete work-up for alternative etiologies for hypoglycemia was negative. It is important for prescribing physicians to be aware of this rare but serious side effect and to counsel patients and families appropriately. Key Teaching Points • Sotalol is a racemic mixture of 2 enantiomers. In addition to potassium channel blockade, sotalol has nonselective beta-adrenergic blocking effects. • Severe hypoglycemia can occur with sotalol use, particularly in young children and toddlers after decreased oral intake, after prolonged fasting, or with intercurrent illness. • Counseling should be provided to parents regarding hypoglycemia risks.

Ablation for atrioventricular nodal reentrant tachycardia (AVNRT) classically utilizes evaluation of signal morphology within the anatomic region of the slow pathway (SP), which involves subjectivity. Ripple mapping (RM; CARTO-3Patients ≤18 years with AVNRT in the EP laboratory between 2017 and 2021 were evaluated. RM was performed to define region of SP conduction in patients from 2019 to 2021, whereas standard electro-anatomical mapping was used from 2017 to 2019. All ablations were performed using cryotherapy. Demographics, outcomes, and analysis of variance in number of test lesions until success was compared between groups.A total of 115 patients underwent AVRNT ablation during the study; 46 patients were in the RM group and 69 were in the control group. There were no demographic differences between groups. All procedures, in both groups, were acutely successful. In RM group, 89% of first successful lesions were within 4 mm of the predicted site. There was significantly reduced variability in number of test lesions until success in the RM group (p = .01).RM is a novel technique that can help identify SP location, allowing for successful ablation of AVNRT with decreased variability.

This study sought to evaluate the safety and efficacy of transvenous lead extraction (TLE) at a single pediatric/congenital heart disease (CHD) center.Data on TLE in pediatric and CHD patients are limited.Retrospective cohort study evaluating TLE from 2008 to 2019.A total of 113 patients underwent TLE with 162 leads removed (including 38 high-voltage leads). Median age at lead implantation was 13 years (range 0.6-61.8 years), with a median age at extraction of 21.6 years (6.4-64.3 years). Median lead age was 7.2 years (1.0-35.3 years). Successful extraction occurred in 110 (97%) patients, 159 (98%) leads. Complex extraction was needed for 120 leads; 52 (44%) using laser alone, 27 (23%) mechanical sheath alone, and 21 (18%) using both. Femoral extraction was used for 19 (16%) leads. Risk factors for complex extraction were ≥2 leads extracted (odds ratio: 3.36; 95% confidence interval [CI]: 1.2-9.36; P = 0.021), lead within the right ventricle (odds ratio: 2.8; 95% CI: 1.2-6.5; P = 0.017), and a combination of younger patient age at implant and older lead age at extraction, with patients ≤12 years of age at implant and leads ≥7 years of age having an odds ratio: of 10.1 (95% CI: 2.21-45.9; P = 0.003). Major complications occurred in 5 (4.4%) of patients, with no mortality, but a high incidence of tricuspid valve injury.TLE can be performed successfully and safely in a pediatric and CHD center. Patient age at lead implantation, not age at extraction, is an important predictor of needing a complex extraction, with younger patients at implant and older leads at extraction having the highest risk.

Junctional ectopic tachycardia (JET) is a common arrhythmia after congenital heart disease surgery. There is variability in the choice of antiarrhythmic therapy, with amiodarone used commonly. Intravenous (IV) sotalol is a newly available agent that may be useful for JET.The purpose of this study was to evaluate the safety and efficacy of IV sotalol for postoperative JET and compare outcomes with IV amiodarone.This is a retrospective single-center study of all patients who received IV sotalol or IV amiodarone for postoperative JET at Texas Children's Hospital from December 15, 2015, to December 15, 2020. Data included antiarrhythmic efficacy, hemodynamics, and adverse effects. Successful JET control was defined as a decrease in JET rate to170 beats/min (or decrease by20%), or conversion to sinus rhythm, with persistent control over 24 hours without requiring alternative antiarrhythmics or mechanical support.A total of 32 patients (median age 71 days; interquartile range 17-221 days) received IV amiodarone (n = 20 [62%]) or IV sotalol (n = 12 [38%]) for postoperative JET. Amiodarone was successful in treating JET in 75% of cases; sotalol was successful in 83%. The JET rate decreased faster over the first 90 minutes after a sotalol bolus (25 beats/min per hour) than after an amiodarone bolus (8 beats/min per hour) (P.01); no heart rate difference was seen after 24 hours. Amiodarone infusion was discontinued early because of hypotension/bradycardia in 2 patients; this was not required in any patients receiving sotalol.For children with postoperative JET, both IV sotalol and amiodarone are safe and efficacious. IV sotalol may lead to a faster improvement in heart rate.

Introduction: Surgical repair in anomalous aortic origin of a coronary artery (AAOCA) aims at mitigating the risk of sudden cardiac death in a subset of patients (pts). The pattern and behavior of electrocardiogram (ECG) changes in a large cohort of these pts is lacking. We aim to describe post-operative (post-op) ECG changes in this population with medium term follow up (f/u). Methods: Observational study evaluating ECGs before and after surgery to describe peri-operative changes through f/u. All patients Results: A total of 64 pts were identified (median age 13.7 yrs, 64% male). Two patients had evidence of STE and TWI pre-op and were not included. Of 62 pts: 47 (75%) had new STE early post-op and all but 1 had resolution at 90-day f/u; 50 (81%) had no evidence of new TWI or q waves early post-op and 96% of these remained stable at 90-day f/u. Conversely, 12 (19%) had new TWI in the early post-op: 8 had isolated changes (75% resolved at 90 days) and 4 associated with new q waves (50% resolved at 90 days). Including all ECG changes in the total cohort, 92% resolved completely at 90-day f/u. In those pts (8%) with persistent changes, none required late reintervention and all but 1 were cleared for return to exercise. All patients were alive at medium f/u of 4 yrs (IQR 2-6) with normal ventricular function. Conclusions: New STE is a common finding in the early post-op period following repair of AAOCA and is not correlated with persistent ECG changes at 90-day follow up. TWI is infrequently seen but less likely to resolve if associated with new q waves. Despite these changes in the early post-op, clinical outcomes medium-term are excellent.

Introduction: The current AATS and AHA/ACC guidelines recommend maximal exercise stress test (mEST) to identify ischemia and direct decision-making in patients (pts) with anomalous aortic origin of a coronary artery (AAOCA). Stress cardiac magnetic resonance imaging (sCMR) has reliably identified myocardial perfusion abnormalities. Hypothesis: We hypothesize that EST and sCMR do not agree in the detection of inducible ischemia in AAOCA. Methods: AAOCA pts 1.05. Abnormal mEST included: significant ST changes (≥1 mm horizontal or downsloping ST-depression, ≥2 mm upsloping ST depression, ST elevation), high-grade arrhythmia, abnormal peak VO2 ( Results: Of 147 pts with AAOCA and both EST and sCMR, 140 achieved max HR ≥85%ile on EST. Table 1 compares demographics and EST parameters in pts with inducible ischemia on sCMR (+sCMR) vs without (-sCMR). Significant ST changes were seen in 2/26 (7.7%) pts with +sCMR compared to 8/114 (7%) pts with -sCMR. An abnormal mEST did not agree with sCMR in identifying inducible ischemia (McNemar p < 0.001) in all AAOCA patients who achieved max HR, nor in the sub-group with RER >1.05 (n = 88). Conclusions: mEST does not agree with sCMR in identifying inducible ischemia in patients with AAOCA. Our data suggest that mEST should not be used alone for the detection of inducible ischemia.

Fontan-associated protein-losing enteropathy is difficult to treat and associated with poor prognosis. Cardiac rehabilitation and exercise are thought to have beneficial effects for patients with Fontan circulation. We report the case of a young adult patient palliated to Fontan circulation, with a decade-long history of symptoms related to protein-losing enteropathy. At age 23 years, he appreciated an improvement in symptoms and laboratory values after cardiac rehabilitation and prescriptive exercise.

Wolff-Parkinson-White (WPW) syndrome is a ventricular pre-excitation syndrome affecting approximately 1–3 in 1000 individuals and carries a low, but clinically important, risk of sudden cardiac death. The clinical presentation can vary from asymptomatic WPW to cardiac arrest as the sentinel event. Practice guidelines have been developed for the management of patients with asymptomatic WPW. These guidelines include noninvasive and invasive testing and have ascribed an important role to exercise testing.

Ventricular volumes and ejection fraction are often used in clinical decision making in patients with congenital heart disease (CHD). The referral diagnosis, radiation exposure and image quality of functional cardiac computed tomography (CT) in a relatively large cohort of patients of CHD has not been reported. This is a retrospective evaluation of functional CT studies performed in CHD patients from three institutions (1/2007–3/2013). Patient and scanner characteristics, radiation dose estimates and image quality were compared. Two hundred ninety-eight functional CT studies were evaluated. The most common referral diagnosis were tetralogy of Fallot (33 %), transposition complexes (24 %) single ventricle heart disease (15 %), and left sided obstruction (15 %). The reason for cardiac CT was presence of pacemaker (60 %), need for detailed coronary artery imaging (18 %), metallic artifact in CMR (12 %), evaluation of prosthetic valve function (4 %), and claustrophobia or BMI too large for the available MR scanner (6 %). 266 (89.3 %) scans allowed quantification of ventricular function, 25 (8.4 %) scans allowed qualitative assessment of function, and 7 (2.3 %) of the scans were non-diagnostic for functional analysis. Median DLP was 399 mGy cm (186, 614), and median effective dose was 5.5 mSv (2.6, 8.5). Radiation dose and image quality varied across institutions. Cardiac CT function imaging can be performed in patients with congenital heart disease when CMR is contraindicated or has poor image quality. Radiation dose and image quality varies across institutions.