Transcatheter recanalisation for absent intra-pericardial pulmonary arteries.

Unilateral absence of intra-pericardial pulmonary artery is a rare congenital malformation. If untreated, it can lead to morbidity and mortality in adulthood. Early intervention and restoration of physiologic pulmonary blood flow is necessary. Transcatheter stenting as initial intervention has been rarely reported. We present transcatheter recanalisation and stenting of the obliterated ductus in two newborns with unilateral absence of intra-pericardial pulmonary artery with cross-sectional imaging, procedural details, angiography, and follow up to surgical repair. We believe that such procedure promotes ipsilateral pulmonary vasculature growth to facilitate unifocalization surgery at a later age.

Cardiopulmonary Exercise Testing Characterizes Silent Cardiovascular Abnormalities in Asymptomatic Pediatric Cancer Survivors.

Late-onset cardiovascular complications are serious concerns for pediatric cancer survivors (PCS) including those who are asymptomatic. We investigated whether cardiopulmonary exercise testing (CPET) can delineate the underlying pathophysiology of preclinical cardiovascular abnormalities in PCS. We examined CPET data via cycle ergometer in asymptomatic PCS with normal echocardiogram and age-matched controls. Peak and submaximal parameters were analyzed. Fifty-three PCS and 60 controls were studied. Peak oxygen consumption (VO2), peak work rate (WR), and ventilatory anaerobic threshold (VAT) were significantly lower in PCS than controls (1.86 ± 0.53 vs. 2.23 ± 0.61 L/min, 125 ± 45 vs. 154 ± 46 W, and 1.20 ± 0.35 vs. 1.42 ± 0.43 L/min, respectively; all p < 0.01), whereas peak heart rate (HR) and ventilatory efficiency (a slope of minute ventilation over CO2 production or ∆VE/∆VCO2) were comparable. Peak respiratory exchange ratio (RER) was significantly higher in PCS (p = 0.0006). Stroke volume (SV) reserve was decreased in PCS, indicated by simultaneous higher dependency on HR (higher ∆HR/∆WR) and lower peak oxygen pulse (OP). Twelve PCS with high peak RER (≥ 1.3) revealed lower pVO2 and VAT than the rest of PCS despite higher ventilatory efficiency (lower ∆VE/∆VCO2), suggesting fundamental deficiency in oxygen utilization in some PCS. Poor exercise performance in PCS may be mainly attributed to limited stroke volume reserve, but the underlying pathophysiology is multifactorial. Combined assessment of peak and submaximal CPET parameters provided critical information in delineating underlying exercise physiology of PCS.

Telehealth and Application of Appropriate Use Criteria for Pediatric Transthoracic Echocardiography.

Appropriate use criteria (AUC) for the performance of outpatient transthoracic echocardiography are based on history, physical examination and electrocardiograms. Telehealth provides access to clinical care in the absence of a physical examination. We assessed application of AUC for performance of echocardiography following a telehealth visit. In this single center study, we retrospectively reviewed the charts of pediatric patients whose originally scheduled in person visits were converted to telehealth visits between March and May 2020. Patients were referred for echocardiogram by the telehealth cardiologist. Echocardiograms requested during the telehealth visit were retrospectively classified as appropriate [A], may be appropriate [M], or rarely appropriate [R] by two independent reviewers. Findings from the echocardiogram were classified as normal, abnormal, or abnormal and motivating treatment by the same two independent reviewers based upon results from the echocardiogram report and physician office visit note. Of the 441 patients seen by telehealth, interquartile range of 1.9-15.3 years, 114 (26%) patients were referred for an echocardiogram. Of the patients for whom echocardiograms were requested, 56/114 (49%) were new to cardiology. Echocardiograms were rated: A-85 (75%), M-5 (4%), and R-24 (21%). Of the 108 completed studies, 61 (56%) were normal, 44 (41%) were abnormal, and three (3%) were abnormal motivating treatment. Abnormal TTE findings were not detected in the R encounters compared with abnormal findings in 46/82 (56%) of the A completed studies. Seventy-one percent (15/21) of the R-rated studies were performed for murmur evaluation.

A Combined Analysis of Peak and Submaximal Exercise Parameters in Delineating Underlying Mechanisms of Sex Differences in Healthy Adolescents.

Peak exercise parameters are considered the gold standard to quantify cardiac reserve in cardiopulmonary exercise testing (CPET). We studied whether submaximal parameters would add additional values in analyzing sex differences in CPET. We reviewed CPET of age-matched healthy male and female adolescents by cycle ergometer. Besides peak parameters, submaximal CPET parameters, including ventilatory anaerobic threshold (VAT), oxygen uptake efficiency slope (OUES), and submaximal slopes of Δoxygen consumption (ΔVO2)/Δwork rate (ΔWR), Δheart rate (ΔHR)/ΔWR, ΔVO2/ΔHR, and Δminute ventilation (ΔVE)/ΔCO2 production (ΔVCO2), were obtained. We studied 35 male and 40 female healthy adolescents. Peak VO2 (pVO2), peak oxygen pulse (pOP), and VAT were significantly lower in females than males (1.9 ± 0.4 vs. 2.5 ± 0.6 L/min; 10 ± 2.0 vs. 13.2 ± 3.5 ml/beat; 1.23 ± 0.3 vs. 1.52 ± 0.5 L/min, respectively, all p < 0.005). Females showed significantly lower pVO2, VAT, and OUES with the same body weight than males, implying higher skeletal muscle mass in males. When simultaneously examining ΔHR/ΔWR and pOP, females showed higher dependency on increases in HR than in stroke volume. Females demonstrated significantly lower pOP with the same levels of ΔVO2/ΔHR, suggesting more limited exercise persistence than males under an anaerobic condition at peak exercise. Oxygen uptake efficiency in relation to peak VE was significantly higher in males. There was no sex difference in either ΔVO2/ΔWR or ΔVE/ΔVCO2. Combinational assessment of peak and submaximal CPET parameters delineates the multiple mechanisms that contribute to the sex differences in exercise performance.

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.

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager.

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

Outcomes and Safety of Blenderized Tube Feedings in Pediatric Patients: A Single Center's Experience.

INTRODUCTION: Recently, significant interest from families and healthcare providers has arisen to use blenderized tube feedings (BTF). Although many institutions are providing this nutritional option, literature documenting outcomes and safety is lacking. METHODS: A retrospective chart review was performed on pediatric patients receiving BTF at Rutgers-Robert Wood Johnson University Hospital between January 2013 and April 2017. Demographic data and dietary information before and after BTF were collected. Reasons for diet initiation, symptoms, and anthropometrics were recorded. Adverse events and outcomes were assessed through physician documentation and relevant medication changes. RESULTS: Thirty-five patients (24 boys) received BTF. Age at initiation of BTF ranged from 1 to 19 years (mean 8.3 +/- 5.8 [SD] years). Length of follow-up ranged from 1 to 45 months (mean 15 +/- 12.2 months). The most common reason for starting BTF was gastroesophageal reflux disease (GERD) (N = 32). Almost all patients were on medications for GERD, constipation, or gastrointestinal dysmotility before starting BTF (N = 33). Majority of patients had improvement in relevant symptoms (N = 20); 13 of 33 patients on gastrointestinal medications were able to wean or stop medication(s). BMI z scores did not differ before and after BTF initiation (P = 0.558). No serious life-threatening adverse events were found. CONCLUSIONS: Our data suggest that BTF is a safe dietary intervention that may improve gastrointestinal symptoms in pediatric patients. Further prospective studies are needed to compare safety and efficacy of BTF and commercial formulas in pediatric patients.