Epipericardial Fat Pad Necrosis-A Rare Cause of Chest Pain in an Adolescent.

ABSTRACT: Epipericardial fat necrosis is a rare cause of acute pleuritic chest pain reported in approximately 40 cases. This diagnosis mimics a myocardial infarction, pulmonary embolism, or pericarditis; however, the cardiac enzymes and electrocardiogram are usually normal. We present the first reported case of epipericardial fat necrosis in an adolescent.

Advanced Heart Block in Children with Lyme Disease.

BACKGROUND: The clinical course of children with advanced heart block secondary to Lyme disease has not been well characterized. OBJECTIVE: To review the presentation, management, and time to resolution of heart block due to Lyme disease in previously healthy children. METHODS: An IRB approved single-center retrospective study was conducted of all patients < 21 years old with confirmed Lyme disease and advanced second or third degree heart block between 2007 and 2017. RESULTS: Twelve patients (100% male) with a mean age of 15.9 years (range 13.2-18.1) were identified. Six patients (50%) had mild to moderate atrioventricular valve regurgitation and all had normal biventricular function. Five patients had advanced second degree heart block and 7 had complete heart block with an escape rate of 20-57 bpm. Isoproterenol was used in 4 patients for 3-4 days and one patient required transvenous pacing for 2 days. Patients were treated with 21 days (n = 6, 50%) or 28 days (n = 6, 50%) of antibiotics. Three patients received steroids for 3-4 days. Advanced heart block resolved in all patients within 2-5 days, and all had a normal PR interval within 3 days to 16 months from hospital discharge. CONCLUSION: Symptomatic children who present with new high-grade heart block from an endemic area should be tested for Lyme disease. Antibiotic therapy provides quick and complete resolution of advanced heart block within 5 days, while steroids did not appear to shorten the time course in this case series. Importantly, no patients required a permanent pacemaker.

Aortic Root Dilatation in Mucopolysaccharidosis I-VII.

The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children's Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions. Exact binomial 95% confidence intervals (CIs) were constructed for ARD, defined as a z-score greater than 2 at the SoV. The patient age ranged from 3.4-25.9 years (mean 13.3 ± 6.1), the height from 0.87-1.62 meters (mean 1.24 ± 0.21), and the weight from 14.1-84.5 kg (mean 34.4 ± 18.0). The prevalence of dilation at the AVA was 41% (14/34; 95% CI: 25%-59%); at the SoV was 35% (12/34; 95% CI: 20%-54%); and at the STJ was 30% (9/30; 95% CI: 15%-49%). The highest prevalence of ARD was in MPS IVa (87.5%). There was no significant difference between mean z-scores of MPS patients who received treatment with hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT) vs. untreated MPS patients at the AVA (z = 1.9 ± 2.5 vs. z = 1.5 ± 2.4; p = 0.62), SoV (z = 1.2 ± 1.6 vs. z = 1.3 ± 2.2; p = 0.79), or STJ (z = 1.0 ± 1.8 vs. z = 1.2 ± 1.6; p = 0.83). The prevalence of ARD was 35% in our cohort of MPS I-VII patients. Thus, we recommend screening for ARD on a routine basis in this patient population.

Neonatal arrhythmias in Turner syndrome: a case report and review of the literature.

BACKGROUND: While left-sided congenital heart defects have been well described in females with Turner syndrome (45, X), the literature is scarce regarding arrhythmias in this patient population. CASE SUMMARY: A full-term neonate referred to cardiology was found to have a non-apex forming left ventricle and partial anomalous pulmonary venous return. During the echocardiogram, she developed atrial flutter, followed by orthodromic reentrant supraventricular tachycardia (SVT). She was started on propranolol and eventually switched to sotalol due to breakthrough SVT. A genetics evaluation revealed Turner syndrome with complete monosomy X (45, X). The patient is now 18 months old and has not had any further arrhythmias. DISCUSSION: We present a rare case of atrial flutter followed by supraventricular tachycardia in a neonate with Turner syndrome and left-sided heart defects. This case highlights the importance of early and precise investigation of cardiac abnormalities in neonatal patients, especially among females with Turner syndrome given their relatively higher risk of cardiovascular disease compared to the general population.

Comparison of Holter With Zio Patch Electrocardiography Monitoring in Children.

The standard for ambulatory arrhythmia detection in children is the Holter monitor. The Zio XT (Zio) patch has been FDA-approved for use in adults. However, its utility in children has not been directly compared with the Holter. We studied the ability to detect arrhythmias and patient comfort of the Zio versus the Holter in children. Patients <22 years old were prospectively enrolled to wear the Holter and Zio simultaneously for 48 hours at our institution. Detection of clinically significant arrhythmias was compared using McNemar's test. Wear-time and artifact time was compared using Wilcoxon sign test. Patient satisfaction ratings were analyzed with paired t tests. Two hundred patients (57% male) were included for analysis. The median age was 13.5 years (range 23 days to 21.7 years), and 40% had heart disease. The Zio and Holter had comparable median wear-times, 48.2 hours (interquartile range [IQR] 45.8 to 50.2]) versus 48.0 (48.0 to 48.0), respectively, p = 0.14, but the Zio had less artifact than the Holter, 2.8% (IQR 1.1 to 8.6) versus 5.6% (2.4 to 15.7), respectively, p <0.001. There was no difference in detection of clinically significant arrhythmias for the Zio versus the Holter (p = 0.23), however 75% of patients preferred the Zio over the Holter (p <0.001) due to lack of wires and the ability to shower. In conclusion, the Zio patch is as good as the Holter monitor in detection of clinically significant arrhythmias in children with less artifact. Patients/parents more often preferred the Zio over the Holter.

Diagnostic yield of patch ambulatory electrocardiogram monitoring in children (from a national registry).

The diagnostic yield of continuous electrocardiographic (ECG) monitoring in children for periods longer than a Holter monitor is unclear. The aim of this study was to characterize diagnostic yield, arrhythmia type, and time to first arrhythmia using a clinical repository of national ambulatory ECG data in children. A cross-sectional study was performed in 3,209 consecutive children receiving a 14-day adhesive patch monitor (Zio Patch) for clinical indications from January 2011 to December 2013. Of the 3,209 children (56% female, mean age 12.5 ± 4.4 years, range 1 month to 17 years), 390 had arrhythmias detected, making the diagnostic yield 12.2%. Of these, 137 patients (4.3%) had arrhythmias deemed clinically significant to warrant urgent physician notification. The most frequent indications for monitoring were palpitations (n = 1,138 [35.5%]), syncope (n = 450 [14.0%]), unspecified tachycardia (n = 291 [9.1%]), supraventricular tachycardia (n = 264 [8.2%]), and chest pain (n = 261 [8.1%]). Arrhythmias were detected in 10.0% of patients with palpitations, 6.7% of patients with syncope, 14.8% of patients with tachycardia, 22.7% of patients with supraventricular tachycardia, and 6.5% of patients with chest pain. The mean times to first detected and first symptom-triggered arrhythmias were 2.7 ± 3.0 and 3.3 ± 3.3 days, respectively. Forty-four percent of first detected arrhythmias and 50.4% of the first symptom-triggered arrhythmias occurred beyond 48 hours of monitoring. In conclusion, the diagnostic yield of continuous ECG patch monitoring in children was substantial beyond 48 hours and should be considered in children who are candidates for longer term ECG monitoring.

Monitoring universal protocol compliance through real-time clandestine observation by medical students results in performance improvement.

OBJECTIVE: To measure universal protocol compliance through real-time, clandestine observation by medical students compared with chart audit reviews, and to enable medical students the opportunity to become conscious of the importance of medical errors and safety initiatives. DESIGN: With endorsement from Tufts Medical Center's (TMC's) Chief Medical Officer and Surgeon-in-Chief, 8 medical students performed clandestine observation audits of 98 cases from April to August 2009. A compliance checklist was based on TMC's presurgical checklist. Our initial results led to interventions to improve our universal protocol procedures, including modifications to the operating room white board and presurgical checklist, and specific feedback to surgical departments. One year later, 6 medical students performed observations of 100 cases from June to August 2010. SETTING: Tufts Medical Center, Boston, Massachusetts, which is an academic medical center and the principal teaching hospital for Tufts University School of Medicine. PARTICIPANTS: An operating room coordinator placed the medical students into 1 of our 25 operating rooms with students entering under the premise of observing the anesthesiologist for clinical education. The observations were performed Monday to Friday between 7 am and 4 pm. Although observations were not randomized, no single service or type of surgery was targeted for observation. RESULTS: A broad range of departments was observed. In 8.2% of cases, the surgical site was unmarked. A Time Out occurred in 89.7% of cases. The entire surgical team was attentive during the time out in 82% of cases. The presurgical checklist was incomplete before incision in 13 cases. Images were displayed in 82% of cases. The operating room "white board" was filled out completely in 49% of cases. Team introductions occurred in 13 cases. One year later, compliance increased in all Universal Protocol dimensions. CONCLUSIONS: Direct, real-time observation by medical students provides an accurate and granular assessment of compliance with specific components of the universal protocol and engages medical students in the quality improvement process, raises their awareness of the gravity of medical errors, and ensures appreciation of the importance of quality and safety initiatives.