Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.

INTRODUCTION: Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life. METHODS: In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life. RESULTS: Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 - 176 days, 3rd = 178 - 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year. CONCLUSIONS: Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

Results of international assistance for a paediatric heart surgery programme in a single Ukrainian centre.

BACKGROUND: Surgery for CHD has been slow to develop in parts of the former Soviet Union. The impact of an 8-year surgical assistance programme between an emerging centre and a multi-disciplinary international team that comprised healthcare professionals from developed cardiac programmes is analysed and presented.Material and methodsThe international paediatric assistance programme included five main components - intermittent clinical visits to the site annually, medical education, biomedical engineering support, nurse empowerment, and team-based practice development. Data were analysed from visiting teams and local databases before and since commencement of assistance in 2007 (era A: 2000-2007; era B: 2008-2015). The following variables were compared between periods: annual case volume, operative mortality, case complexity based on Risk Adjustment for Congenital Heart Surgery (RACHS-1), and RACHS-adjusted standardised mortality ratio. RESULTS: A total of 154 RACHS-classifiable operations were performed during era A, with a mean annual case volume by local surgeons of 19.3 at 95% confidence interval 14.3-24.2, with an operative mortality of 4.6% and a standardised mortality ratio of 2.1. In era B, surgical volume increased to a mean of 103.1 annual cases (95% confidence interval 69.1-137.2, p<0.0001). There was a non-significant (p=0.84) increase in operative mortality (5.7%), but a decrease in standardised mortality ratio (1.2) owing to an increase in case complexity. In era B, the proportion of local surgeon-led surgeries during visits from the international team increased from 0% (0/27) in 2008 to 98% (58/59) in the final year of analysis. CONCLUSIONS: The model of assistance described in this report led to improved adjusted mortality, increased case volume, complexity, and independent operating skills.

Down Syndrome and Postoperative Complications in Children Undergoing Intestinal Operations.

OBJECTIVE: This paper intends to evaluate the association between Down Syndrome (DS) and postoperative medical and surgical complications and inpatient postoperative mortality in pediatric patients undergoing intestinal operations. METHODS: The 2012 Kids' Inpatient Database was queried to compare short-term postoperative medical and surgical complications and in-patient mortality among patients with DS undergoing intestinal operations to a cohort without DS using inverse probability weighting. Subset analysis was performed for patients undergoing intestinal operations exclusive of gastrostomy placement. Adverse treatment effects were calculated for the outcomes of interest. RESULTS: Of 17,026 pediatric patients undergoing intestinal operations, 444 had DS. In unadjusted analysis, medical complications (urinary tract infection, deep venous thrombosis, sepsis, pneumonia) occurred in 7.9% of patients with DS, compared to 14.1% of those without (p < 0.001). Surgical complications (wound disruption, hemorrhage, superficial or deep wound infection) occurred in 3.5% of patients with DS, compared to 4.6% of those without (p = 0.34), and in-patient mortality occurred in 0.3% of patients with DS, compared to 2.7% of those without (p = 0.009). Adverse treatment effects (ATE) calculated after inverse probability weighting demonstrated no difference for medical or surgical complications but a significantly decreased mortality with DS. CONCLUSIONS: Contrary to common perception and data extrapolated from the adult literature, pediatric patients with DS have neither higher medical nor surgical complication rates after intestinal operations. Similar to patients undergoing congenital heart surgery, pediatric patients with DS have a lower postoperative inpatient mortality after these general operations compared to those without DS. Mechanisms influencing risks in DS patient remain unknown. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Circadian Variation of Ventricular Arrhythmias in Catecholaminergic Polymorphic Ventricular Tachycardia.

OBJECTIVES: The aim of this paper was to investigate whether ventricular arrhythmias in children with catecholaminergic polymorphic ventricular tachycardia (CPVT) show circadian patterns. BACKGROUND: Circadian arrhythmic patterns have been established in long QT, Brugada, and early repolarization, but have not been investigated in CPVT. METHODS: This is a multicenter, retrospective review of pediatric CPVT patients, age <21 years at diagnosis. Timing of ventricular tachycardia (VT ≥3 beats) was assessed during 24-h continuous monitoring (Holter, implantable loop recorder, implantable cardioverter defibrillator) and by eliminating sleep hours, in addition to sporadic exercise stress tests. Morning was defined as 6:00 am to 11:59 am, afternoon 12:00 pm to 5:59 pm, and evening 6:00 pm to 11:59 pm. Distribution of VT events was compared by time of day, day of week, age, and sex. RESULTS: Eighty patients (53% male), 61% with an ICD, experienced 423 VT events during a median follow-up time of 6 years (interquartile range: 2 to 10 years). When compared to morning hours, VT was more likely to occur in the afternoon (odds ratio [OR]: 2.54; 95% confidence interval [CI]: 1.69 to 3.83) or evening hours (OR: 2.91; 95% CI: 1.82 to 4.67). The predominance of afternoon/evening events persisted regardless of age, gender, or day of the week. Among 50 patients who underwent exercise stress tests, VT was significantly more likely to occur in the afternoon (OR: 3.00; 95% CI: 1.39 to 6.48). CONCLUSIONS: In pediatric CPVT patients, ventricular arrhythmias are more likely to occur in the afternoon and evening hours. Because children's activity levels peak in both the morning and afternoon, the lack of arrhythmias in the morning hours raises questions whether factors other than adrenergic stimulation influence arrhythmia induction in pediatric patients with CPVT.

Reverse Szabo technique for stenting a single major aorto-pulmonary collateral vessel in pulmonary atresia with ventricular septal defect.

Management of pulmonary atresia with ventricular septal defect (PA-VSD) in the neonatal period presents numerous challenges. Endovascular stenting of the ductus arteriosus or of a collateral vessel in ductal-dependent pulmonary circulation as an alternative to the Blalock-Taussig (BT) shunt has become increasingly popular in the last decades. The utilization of the reverse Szabo (anchor-wire) technique for single collateral vessel stenting in a case of PA-VSD is described.

Atrial tachycardias in young adults and adolescents with congenital heart disease: conversion using single dose oral sotalol.

UNLABELLED: Atrial tachyarrhythmias are a chronic long-term hazard in patients with congenital heart disease (CHD). These arrhythmias contribute to ventricular dysfunction, heart failure can contribute to sudden death. We performed a prospective study of oral sotalol for the conversion of atrial tachyarrhythmias in adults and adolescents with congenital heart disease and stable hemodynamics. METHODS: Patients were admitted and given oral sotalol in an inpatient, monitored setting. The initial dose was targeted at 2 mg/kg. Antiarrhythmic drugs other than digoxin were stopped. RESULTS: Nineteen patients were enrolled. The average patient age was 20 years (12-39). Four had atrial ectopic tachycardia (AET) and 15 had atrial reentry tachycardia (IART). Nine had Fontan physiology. Permanent pacing therapies had failed to restore sinus or paced rhythm consistently in 6 patients. Overall 16 of 19 atrial tachyarrhythmias (84%) converted with single dose oral sotalol. AET converted to sinus or paced rhythm in 3/4 patients and IART in 13/15 patients. The average times to conversion were 98 and 145 min, respectively. Two patients required pacemakers due to sinus bradycardia. One patient had a lethal thromboembolic event 2 days after conversion. CONCLUSIONS: Oral sotalol offers an effective alternative to direct current cardioversion in adults and adolescents with CHD and hemodynamically stable atrial tachyarrhythmias. Conversion with sotalol at ~2 mg/kg generally occurred within 2 h. Vigilance for thromboembolism must be maintained as well as caution for those with bradycardia without pacemakers in this patient population. There are theoretical and practical advantages of sotalol over cardioversion.

Paediatric cardiac assistance in developing and transitional countries: the impact of a fourteen year effort.

BACKGROUND: Paediatric cardiac services are poorly developed or totally absent in underdeveloped countries. Institutions, foundations and interested individuals in those nations in which sophisticated paediatric cardiac surgery is practised have the ability to alleviate this problem by sponsoring paediatric cardio-surgical missions to provide care, and train local caregivers in developing, transitional, and third world countries. The ultimate benefit of such a programme is to improve the surgical abilities of the host institution. The purpose of this report is to present the impact of our programme over a period of 14 years. METHODS: We specifically reviewed our database of patients from our missions, our team lists, surgical results, and the number and type of personnel trained in the institutions that we have assisted. In order for the institution to be entered into the study, the foundation had to provide at least 2 months of training. In addition, the institution had to respond to a simple questionnaire concerning the number and types of surgery performed at their facility before and after intervention by the foundation. RESULTS: We made 140 trips to 27 institutions in 19 countries, with 12 of the visited institutions qualifying for inclusion. Of these, 9 institutions reported an increase in the number and complexity of cases currently being performed in their facility since the team intervened. This goal had not been accomplished in 3 institutions. The reasons for failure included the economic situation of the country, hospital and national politics, personality conflicts, and continued lack of hardware and disposables. CONCLUSIONS: Paediatric cardiac service assistance can improve local services. A significant commitment is required by all parties involved.

The pediatric ECG.

Knowledge of the basics of pediatric ECG interpretation is helpful in differentiating normal from abnormal findings. These basics include familiarity with the age-related normal findings in heart rate, intervals, axis, and waveform morphologies; an understand-ing of cardiac physiologic changes associated with age and maturation, particularly the adaptation from right to left ventricular predominance; and a rudimentary understanding of common pediatric dysrhythmias and findings associated with congenital heart diseases.