Myocardial ischaemia and valve insufficiency caused by a dysplastic aortic valve cusp: a previously unreported unique morphologic anomaly.

Isolated aortic regurgitation and myocardial infarction are a rare congenital defect among neonatal patients. We present a case of a neonate with an unusual aortic valve morphology causing both regurgitation and obstruction of the left coronary artery ostium. Despite both non-invasive and invasive imaging modalities, accurate diagnosis of the valve morphology was only determined by direct visualisation at the time of surgical repair. To the knowledge of authors, this particular aortic valve morphology in neonatal population has not been previously reported in the literature.

Ten-Year Clinical Experience with the Lumenless, Catheter-Delivered, 4.1-Fr Diameter Pacing Lead in Patients with and without Congenital Heart.

BACKGROUND: Patients with congenital heart defects (CHD) often present more challenges to pacing therapy due to anatomy than those without CHD. The lumenless, 4.1Fr diameter M3830 pacing lead (Medtronic, Inc., Minneapolis, MN, USA), approved for use in 2005, has, to date, reported to have excellent short-term (<6 years) lead performance. Unfortunately, very long-term performance is unknown, especially among CHD patients and with implants at alternate pacing (AP) sites. This study reports a 10-year clinical experience with the M3830 lead. METHODS: Records of patients who received the M3830 lead were reviewed: patient demographics, implant techniques and locations, sensing and pacing characteristics, impedances (Imp), and any complications at implant and follow-up. RESULTS: From 2005 to 2015, 141 patients (ages 2-50, mean 20.1 years, 57% males) received 212 leads: atrial 115; ventricle 97. CHD was present in 62% of patients. Leads were inserted at AP sites in 96% of patients. Postimplant follow-up was from 3 months to 10 years (mean 56.3 months). Comparative implant versus follow-up values (mean ± standard deviation) were available on 196 leads (92.5%), showing persistently low (<1 v @ 0.4-0.5 ms) pacing thresholds (P = 0.57). Sensing was also comparable (atrial leads, P = 0.41; ventricular leads, P = 0.9). Impedances differed (P < 0.05) but remained within the normal range. Two A leads became dislodged and one was repositioned while two other leads (1 A, 1 V) were extracted. There are no differences observed in the pacing characteristics between the CHD and non-CHD groups on follow-up. CONCLUSIONS: The 4.1Fr lumenless pacing lead shows ease of implant regardless of CHD or AP site, excellent very long-term (10 years) stability, and performance indices with a very low rate of complications.

Human Metapneumovirus Infection in Immunocompromised Patients.

Human metapneumovirus (HMPV) is a pathogen associated with respiratory tract infection and is related to avian pneumovirus. Typically, children, the elderly, and those who are immunocompromised are the most susceptible to HMPV infection; however, the virus can infect persons of all ages. In otherwise healthy individuals, HMPV infection is generally self-limiting, but immunocompromised individuals can develop fatal complications. We present a case series of 3 severely immunocompromised patients who were infected with HMPV and describe their clinical course. All 3 patients had acute myeloid leukemia, histories of neutropenic fever, and prolonged hospitalization stays. This case series highlights the severe sequelae observed in individuals infected with HMPV, particularly among those who are immunocompromised.

Incidence of arrhythmias and electrocardiographic abnormalities in symptomatic pediatric patients with PCR-positive SARS-CoV-2 infection, including drug-induced changes in the corrected QT interval.

BACKGROUND: There is limited data regarding the electrophysiological abnormalities and arrhythmias in children with COVID-19, including those associated with treatment using potentially proarrhythmic hydroxychloroquine (HCQ) and azithromycin (AZN). OBJECTIVES: To describe the electrophysiologic findings and arrhythmias associated with pediatric COVID-19 and its treatment. METHODS: A single-center retrospective chart review was undertaken and included all patients with (1) symptoms of COVID-19 and (2) PCR-positive nasopharyngeal swabs for SARS-CoV-2 who were placed on continuous telemetry for the duration of their hospitalization during March through May, 2020. RESULTS: Thirty-six patients were included in the study. Significant arrhythmias were found in 6 (nonsustained ventricular tachycardia in 5 and sustained atrial tachycardia in 1). All were self-resolving and half prompted prophylactic antiarrhythmic therapy. Patients with significant arrhythmias were likely to have noncardiac comorbidities (4/6), but these were not more common than in patients without arrhythmias (20/30, P = 1). The use of HCQ was associated with statistically significant QTc prolongation (413 ± 19 ms vs 425 ± 16 ms, P =.005). QTc was not statistically different in patients with and without arrhythmias (425 ± 15 ms vs 425 ± 15 ms, P = 1). CONCLUSIONS: In pediatric patients with PCR-positive active COVID-19 infection, significant arrhythmias are infrequent, but are more common than expected in a general pediatric population. Comorbidities are not more common in patients with arrhythmias than in patients without arrhythmias. COVID-19 treatment using HCQ is associated with QTc prolongation but was not associated with arrhythmias in pediatric patients.

Pharmacologic management of attention deficit hyperactivity disorder in children and adolescents: a review for practitioners.

Attention-deficit/hyperactivity disorder (ADHD) is a common neurobehavioral disorder in children and adolescents. ADHD affects multiple aspects of an individual's life and functioning in family, social, and academic realms. Effective management of ADHD is necessary for children and adolescents and may include non-pharmacologic treatments, pharmacologic therapy including use of stimulant and non-stimulant medications, or a combination of the different treatment modalities. In general, medications used to treat ADHD are safe and effective. Medical practitioners can follow a step-wise approach in the selection and adjustment of pharmacologic agents to treat ADHD, while working closely with families, caregivers, and other medical and educational professionals to form appropriate treatment plans. This article reviews practical aspects of pharmacological treatment of ADHD in children and adolescents.

16 Years of Cardiac Resynchronization Pacing Among Congenital Heart Disease Patients: Direct Contractility (dP/dt-max) Screening When the Guidelines Do Not Apply.

OBJECTIVES: The purpose of this study was to use direct cardiac resynchronization therapy (CRT)-paced contractility (dP/dt-max) response as a pre-implantation evaluation among patients with congenital heart disease (CHD) and follow clinical parameters and contractility indexes after CRT implantation. BACKGROUND: Patients with CHD often develop early heart failure with few therapeutic options, leading to heart transplantation (HT). Unfortunately, guidelines for CRT do not apply, and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies. Therefore, which CHD patients would benefit from CRT remains an enigma. METHODS: From 1999 to 2015, 103 CHD patients with New York Heart Association (NYHA) functional class II to IV were listed for HT; 40 patients on optimal medical therapy were referred for paced contractility response cardiac catheterization before CRT consideration. If dP/dt-max improved ≥15% from baseline, these "responders" were given the option of CRT with continued follow-up after implantation. RESULTS: Of 40 patients studied, 26 (65%) (age 22 ± 8.2 years; 9 of 26 [35%] single or systemic right ventricle; 17 of 26 [65%] with pacemakers) met criteria for possible hemodynamic benefit and underwent CRT implantation. All 26 patients improved in NYHA functional classification: 5 of 26 patients (19%) were later relisted for HT (4 to 144 months, mean 55 months) after CRT implantation, whereas 21 of 26 (81%) continued with improved NYHA functional class (12 to 112 months, mean 44 months) later. A repeat dP/dt-max study following long-term CRT showed stable function or continued contractility improvement. CONCLUSIONS: Heart failure is common among CHD patients, and therapies are limited. CRT guidelines do not address clinical and anatomic issues of CHD. Short-term paced contractility response testing identifies those CHD patients who are likely to respond to CRT regardless of anatomy.