Universal ECG changes in pediatric patients undergoing procainamide challenge.

BACKGROUND: Procainamide is known to prolong PR, QRS, and QTc intervals in adults and is utilized to unmask the distinct electrocardiographic signatures, including right bundle branch block pattern and right precordial ST segment elevation seen with Brugada syndrome. This study analyzes a pediatric case series of procainamide challenges done to evaluate for possible Brugada syndrome. Our goal was to quantify the impact of procainamide on electrocardiographic intervals in all pediatric patients, regardless of the eventual determination of Brugada syndrome status. METHODS: We retrospectively reviewed all children undergoing procainamide challenge, consecutively from March 2009 to September 2012. Patients received intravenous procainamide over 15 minutes. All electrocardiograms (ECGs) performed during the drug challenge were reviewed. Analysis and ECG measurements were performed by investigators blinded to clinical diagnoses. RESULTS: All patients undergoing procainamide infusion had prolongation of their PR (mean +14%; 0-45%), QRS (+13%; 2-49%), and QTc (+15%; 4-30%) intervals regardless of testing outcome. QTc prolongation to >460 ms following procainamide infusion occurred in 76% of patients. No sustained arrhythmias or adverse events occurred. CONCLUSIONS: ECG changes including prolongation of the PR, QRS, and QTc intervals were seen in all children who underwent procainamide challenge for evaluation of possible Brugada syndrome. An increase in the PR, QRS, and QTc intervals of at least 20% occurred in all children without any adverse events. The QTc prolongation was not completely explained by the QRS prolongation.

"What Is the Point of Human Beings?"

Climate change caused by human activities is a serious threat to the survival of our species, and one that we have not yet mustered the perspective and the will to address. Our vision tends to focus on what is useful to ourselves as individuals in the short term, instead of more broadly on what is needed for the long-term good of our species. We do not consider what our purpose and role could be in the life of our planet. We do not ask "What is the point of human beings?" In this time of climate crisis, answering this question by developing a shared sense of purpose, a purpose centered on caring for the world as a whole, might give our species the perspective and will we need to survive.

"Part of Who You Are": Reflections on Being a Military Psychiatrist.

This personal narrative reflects on the writer's formation of a military identity. It discusses the author's experience with the effects of military culture on both personal and group identity and the challenges of leaving the military.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Sports-Related Sudden Cardiac Injury or Death.

Symptoms such as syncope and chest pain, especially if they are accompanied by palpitations or occur with exercise in any combination, require cardiac evaluation before adolescent athletes are allowed to return to the sports field. Some life-threatening conditions will likely be associated with a family history of HCM or LQTS, but the family history may not be discovered at the first medical visit. A family history of CPVT, for example, is hard to elicit unless this diagnosis has already been established in an affected family member. The keys will be the timing of symptoms and the documentation of arrhythmia with exercise. The ECG at baseline in CPVT may be deceptively normal. Hypertrophic cardiomyopathy is progressive, so evaluation during early childhood may be negative. Long QT syndrome may not always result in an abnormal ECG, even in genetically positive individuals. A high index of suspicion is needed to make these diagnoses, especially if the family history is not available.

Sports participation and quality of life in adolescents and young adults with congenital heart disease.

BACKGROUND: Adolescents and young adults with congenital heart disease (CHD) are often restricted from physical activity and sports participation, which may have adverse effects. OBJECTIVES: To determine the amount of physical activity, type of sports participation, and reasons for sports restrictions, and to evaluate the effect of sports participation on quality of life (QoL) in a cohort of patients with CHD. METHODS: Individuals with CHD aged 13-30 years were recruited at outpatient visits or via mailings. They completed a questionnaire addressing physical activity, sports participation, sports restrictions, and QoL (Pediatric Quality of Life Inventory). We also reviewed the patient's medical record. RESULTS: Of the 177 patients who responded (mean age 20 years), 31% have mild CHD, 40% have moderate CHD, and 29% have severe CHD. In the cohort, 52% participate in competitive sports, 25% recreational sports, and 23% no sports. Among patients with severe CHD, 29% participate in competitive sports that would be restricted by published guidelines (36th Bethesda Conference). After controlling for age, sex, CHD severity, residual hemodynamic disease, and comorbidities, participation in competitive sports and increased frequency of physical activity are independently associated with a higher QoL (P = .003 and P = .001, respectively). In an identical model, competitive sports participation and frequency of physical activity are associated with higher maximum predicted oxygen consumption (VO2 ) (n = 40; P = .002 and .02) and slightly lower body mass index (BMI) (P = .02 and .01). All findings were similar when analyses were stratified by recruitment method. CONCLUSIONS: Patients with CHD commonly participate in competitive sports, and such participation is associated with higher QoL, improved exercise capacity, and lower BMI.

Propranolol vs prednisolone for symptomatic proliferating infantile hemangiomas: a randomized clinical trial.

IMPORTANCE: While propranolol is touted as superior to prednisolone for treating infantile hemangiomas (IH), a randomized clinical trial (RCT) comparing the outcome and tolerability of these medications for symptomatic, proliferating IH has not been reported. OBJECTIVES: To determine if oral propranolol is more efficacious and better tolerated than prednisolone in treating symptomatic, proliferating IH and to determine the feasibility of conducting a multi-institutional, RCT comparing efficacy and tolerability of both medications. DESIGN, SETTING, AND PARTICIPANTS: Phase 2, investigator-blinded, multi-institutional RCT conducted in 3 academic vascular anomalies clinics on 19 of 44 eligible infants aged between 2 weeks and 6 months. All participating patients had symptomatic proliferating IH treated between September 1, 2010, and August 1, 2012. INTERVENTIONS: Treatment with oral propranolol vs prednisolone (2.0 mg/kg/d) until halted owing to toxic effects or clinical response. MAIN OUTCOMES AND MEASURES: Primary outcome was change in IH size after 4 months of therapy. Secondary outcomes were response rate and frequency and severity of adverse events (AEs). RESULTS: The primary outcome showed no difference in lesion size or affected skin area after 4 months of therapy: 41% and 1.32 mm2 for prednisolone vs 64% and 0.55 mm2 for propranolol (P = .12 for lesion size, and P = .56 for affected skin area). Longitudinal analyses showed a faster response in total lesion outer dimension with prednisolone (P = .03), but this advantage over time was not noted when central clearing and outer dimension were included in the analysis (P = .91). The overall frequency of AEs was similar (44 for prednisolone vs 32 for propranolol) (P = .84), but prednisolone-treated participants had more grade 3 severe AEs (11 vs 1) (P = .01), particularly growth retardation resulting in size and weight below the fifth percentile. Early study withdrawal owing to AEs occurred in 6 (75%) of 8 patients in the prednisolone group but 0 of 11 propranolol-treated participants. The mean duration of therapy was shorter for prednisolone (141 vs 265 days), reflecting the higher rate of early withdrawals. CONCLUSIONS AND RELEVANCE: Both medications show similar efficacy for reducing the area of symptomatic, proliferating IH. Although prednisolone showed a faster response rate, propranolol was better tolerated with significantly fewer severe AEs. Propranolol should be the first line of therapy for symptomatic IH unless contraindicated or unless future studies demonstrate severe AEs from propranolol. Recruiting participants for a phase 3 RCT would be difficult owing to safety profiles measured here and emerging trends favoring propranolol. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00967226.