Thromboembolism prophylaxis practices of pediatric and congenital electrophysiologists during invasive electrophysiology studies: A PACES survey.

BACKGROUND: Thromboembolic events related to invasive electrophysiology studies, while rare, can have devastating consequences. Use of systemic anticoagulation for a pediatric or adult-congenital invasive electrophysiology study is recommended, however there is no established standard of practice in this population. OBJECTIVE: To report on procedural practices for thromboembolism prophylaxis during invasive electrophysiology studies for pediatric patients and adults with congenital heart disease. METHODS: An anonymous web-based survey was sent to the members of the Pediatric and Congenital Electrophysiology Society. The survey focused on pre-procedural, intra-procedural, and post-procedural thromboembolism prophylaxis practices during invasive electrophysiology studies. Significant practice variation was defined as <90% concordance among respondents. RESULTS: Survey was completed by 73 members; 52 (71%) practicing in the United States, 65 (89%) practicing in an academic institution, and 14 (19%) in an institution that performs more than 200 invasive electrophysiology procedures annually. Responses showed significant variation in practice. Prior to an invasive electrophysiology procedure, 25% discontinue aspirin while 47% discontinue anticoagulants. Heparin is given for all procedures by 32%. When heparin is administered, the first dose is given by 32% after sheaths are placed, 42% after crossing into the systemic atrium, and 26% just prior to systemic-side ablation. Most target an activated clotting time between 200-300 seconds. Post systemic-side ablation, 58% do not initiate a heparin infusion. Post-procedural oral agents were initiated on day of procedure by 34% of respondents and on post-procedure day 1 by 53%. If treating with aspirin, 74% use low-dose (3-5 mg/kg or 81 mg daily), and 68% treat for 4-6 weeks. CONCLUSION: There is significant variation in thromboembolism prophylaxis for invasive EP studies among pediatric and congenital electrophysiologists. Further studies are needed to optimize the management of thromboembolism prophylaxis in this population.

Use of supra-therapeutic phenytoin for management of ventricular arrhythmias in children: Case series and literature review.

Phenytoin is a versatile drug with utility in neurological, dermatological, and even cardiac disease processes. Though phenytoin is widely available due to its excellent anti-epileptic properties, it is now rarely used as an antiarrhythmic. Phenytoin has well-studied sodium-channel blocking abilities which can be taken advantage of to treat ventricular arrhythmias. Thus, it should remain in the arsenal of antiarrhythmics for any electrophysiologist. We present two cases of intractable ventricular arrhythmia in children that were controlled with phenytoin at supra-therapeutic serum levels, preventing the need for heart transplantation.

Preservation of Antegrade Pulmonary Blood Flow in Kawashima Procedure With Prior Right Ventricular Outflow Tract Stent.

Surgical management of single ventricle with interrupted inferior vena cava and azygos continuation typically requires a Kawashima procedure with subsequent completion of Fontan. However, this group is at risk of development of pulmonary arteriovenous malformations. Evidence suggests preservation of hepatic venous flow into the pulmonary circulation can potentially delay this development. We hereby describe a method of preserving antegrade pulmonary blood flow during the Kawashima procedure in the setting of prior right ventricular outflow tract stents.

Cardiac involvement in a pediatric patient with COVID-19: Looking beyond the nonspecific global cardiac injury.

We report a case of a 17-year-old healthy male presenting with multisystem hyperinflammatory shock temporally associated with COVID-19. Cardiac involvement was suspected based on evidence of significant cardiac injury (elevated cardiac biomarkers, electrocardiographic and echocardiographic abnormalities). Cardiac magnetic resonance imaging was performed demonstrating global biventricular systolic dysfunction, as well as a small area of T2 hyperintensity and mid-wall late gadolinium enhancement. This case discusses the varied cardiac involvement in pediatric patients with COVID-19 infection and highlights that cardiac injury is not just limited to hyperinflammatory syndrome related global dysfunction but a more focal myocarditis can also be seen.

Acquired acrodermatitis enteropathica due to zinc-depleted parenteral nutrition.

Well-known causes of zinc deficiency, also referred to as acrodermatitis enteropathica (AE), include defects in intestinal zinc transporters and inadequate intake, but a rare cause of acquired zinc deficiency discussed here is an iatrogenic nutritional deficiency caused by parenteral nutrition administered without trace elements. While zinc-depleted parenteral nutrition causing dermatosis of acquired zinc deficiency was first reported in the 1990s, it is now again relevant due to a national vitamin and trace element shortage. A high index of suspicion may be necessary to diagnose zinc deficiency, particularly because early clinical findings are nonspecific. We present this case of acquired zinc deficiency in a patient admitted to a pediatric intensive care unit for respiratory distress and atypical pneumonia, who subsequently developed a severe bullous eruption due to iatrogenic zinc deficiency but was treated effectively with enteral and parenteral zinc supplementation, allowing for rapid re-epithelialization of previously denuded skin.

Longitudinal Self-Management and/or Transition Readiness per the TRxANSITION Index among Patients with Chronic Conditions in Pediatric or Adult Care Settings.

OBJECTIVE: To evaluate the roles of key individual, family, and illness characteristics on the levels of and gains in longitudinal healthcare transition (HCT) readiness in the pediatric setting and/or self-management skills (SMS) in the adult-focused setting, we used a large dataset with longitudinal measurements from 2006 to 2015. STUDY DESIGN: This longitudinal observational study followed 566 adolescents and young adults with chronic conditions at University of North Carolina Hospitals. TRxANSITION Index measurements, which represent learning outcomes rather than health outcomes, were collected multiple times per patient and analyzed using a novel application of an education-based approach. RESULTS: Levels of and gains in HCT/SMS scores increased with age (P < .001) with smaller increases at older ages. Mastery of skills varied by age with self-management achieved after 20 years of age. Scores varied positively by father's education and negatively by mother's education and duration of diagnosis. Gains in scores further varied positively with private insurance and negatively with mother's education and duration of diagnosis. CONCLUSIONS: We found diminishing positive increases in HCT/SMS scores as patients become older and smaller levels of and gains in readiness among younger patients with more educated mothers. Risk factors for absolute level of HCT/SMS readiness and inadequate longitudinal gains are not always the same, which motivates a deeper understanding of this dynamic process through additional research. This information can guide providers to focus HCT/SMS preparation efforts on skills mastered at particular ages and to identify patients at risk for inadequate development of HCT/SMS skills.

Chronological age when healthcare transition skills are mastered in adolescents/young adults with inflammatory bowel disease.

AIM: To describe the longitudinal course of acquisition of healthcare transition skills among adolescents and young adults with inflammatory bowel diseases. METHODS: We recruited adolescents and young adults (AYA) with inflammatory bowel diseases (IBD), from the pediatric IBD clinic at the University of North Carolina. Participants completed the TRxANSITION Scale™ at least once during the study period (2006-2015). We used the electronic medical record to extract participants' clinical and demographic data. We used ordinary least square regressions with robust standard error clustered at patient level to explore the variations in the levels and growths of healthcare transition readiness. RESULTS: Our sample (n = 144) ranged in age from 14-22 years. Age was significantly and positively associated with both the level and growth of TRxANSITION Scale™ scores (P < 0.01). Many healthcare transition (HCT) skills were acquired between ages 12 and 14 years, but others were not mastered until after age 18, including self-management skills. CONCLUSION: This is one of the first studies to describe the longitudinal course of HCT skill acquisition among AYA with IBD, providing benchmarks for evaluating transition interventions.