Aortic Root Dilation in Patients with 22q11.2 Deletion Syndrome Without Intracardiac Anomalies.

Aortic root dilation (ARD) has been reported in patients with 22q11.2 deletion syndrome (22q11.2DS) with and without congenital heart defects (CHDs). However, the long-term implications of isolated ARD in 22q11.2DS remain undefined. In this study, we measured aortic root size and estimated the probability of changing between normal aortic root size and ARD during follow up to understand the prevalence, longitudinal course, and clinical risk factors for ARD in patients with 22q11.2DS without intracardiac CHDs. Aortic root size was measured in 251 patients with 432 studies. Forty-one patients (16.3%) had ARD on at least one echocardiogram and the cohort sinus Z-score was increased on the last echocardiogram [mean (1.09, SD 1.24) and median (1.20, min - 1.90 and max 5.40)]. Transition probability analysis showed that 8.1% of patients developed ARD and 45.4% of patients with ARD reverted to normal at the next echocardiogram. The risk of ARD over time was significantly associated with male sex (OR 3.06, 95% CI 1.41-6.65; p = 0.004), but not with age or presence of an aortic arch anomaly. Compared to a sinus Z-score ≥ 2, initial Z-score < 2 was associated with 14.3 times lower risk of developing sinus Z-score ≥ 3 at follow up. Sinus Z-score overall decreased by age, and males had a higher Z-score than females (ß = 0.72, SE = 0.14, p < 0.001). Though only a few patients had a Z-score > 4, and patients with initial Z-scores < 2 seem unlikely to develop clinically significant disease, screening practices remain incompletely defined such that periodic evaluation appears warranted.

Parental Wartime Deployment and Socioemotional Adjustment in Early Childhood: The Critical Role of Military Parents' Perceived Threat During Deployment.

Infants, toddlers, and preschool-aged children have unique developmental needs that render them vulnerable to challenges associated with parental military service. We used a sample of military-connected families with 3-6-year-old children (N = 104) to examine associations among children's socioemotional development and fathers' trauma-related deployment experiences, including perceived threat during deployment and exposure to combat and the aftermath of battle. Of these potential stressors, only paternal perceived threat during deployment was significantly associated with measures of mother-reported child adjustment. Fathers' perceived threat during deployment was associated with child behavior problems even after accounting for demographic variables and current paternal symptoms of posttraumatic stress, depression, and anxiety, ß = .36, p = .007. The association between fathers' perceived threat during deployment and child behavior problems was mediated by several family processes related to emotion socialization, including father-reported sensitive parenting, indirect effect (IE) B = 0.106, 95% CI [0.009, 0.236]; parent-child dysfunctional interaction, IE B = 0.119, 95% CI [0.014, 0.252]; and mother-reported family emotional responsiveness, IE B = 0.119, 95% CI [0.011, 0.258]. Implications for future research on the intergenerational transmission of traumatic stress as well as prevention and intervention efforts for military-connected families with young children are discussed.

Investing in Ex Ante Regulation: Evidence from Pharmaceutical Patent Examination.

We explore how the Patent Office may improve the quality of issued patents on "secondary" drug features by giving examiners more time to review drug-patent applications. Our findings suggest that current time allocations are causing examiners to issue low quality secondary patents on the margin. To assess the merits of expanding ex ante scrutiny of drug-patent applications at the agency, we set forth estimates of the various gains and losses associated with giving examiners more time, including reduced downstream litigation costs and added personnel expenses, along with both the static gains and dynamic innovation losses associated with earlier generic entry.

ASE Statement on Adapting Pediatric, Fetal, and Congenital Heart Disease Echocardiographic Services to the Evolving COVID-19 Pandemic.

Over the 12 months since the start of the coronavirus disease 2019 pandemic, an explosion of investigation and an increase in experience have led to vast improvement in our knowledge about this disease. However, coronavirus disease 2019 remains a huge public health threat.

Specific Considerations for Sonographers When Performing Echocardiography during the 2019 Novel Coronavirus Outbreak: Supplement to the American Society of Echocardiography Statement.

Sonographers need to be familiar with and prepared to implement strategies for reducing the risk of exposure to and transmission of the COVID-19 virus. Strategies to employ can be grouped into three broad categories: (1) whom to scan, (2) where to scan, and (3) how to scan. Whom to scan addresses sonographer strategies for determining essential and emergent scan status. Where to scan addresses sonographer practice strategies for selecting equipment, use of rooms, portable examinations, and training personnel. How to scan addresses the topics of scanning techniques (tailored protocols, right-handed scanning, use of barrier devices) and equipment cleaning and disinfecting.

Elevated left ventricular outflow tract velocities on exercise stress echocardiography may be a normal physiologic response in healthy youth.

BACKGROUND: Children with heart disease are at risk for sudden death during exercise, yet decisions regarding sports participation are often based on resting data. Acceleration across the left ventricular outflow tract (LVOT) assessed on stress echocardiography may suggest a diagnosis of hypertrophic cardiomyopathy in patients in whom it is not otherwise obvious. However, the range of peak velocities across the LVOT in healthy youth is unknown. The aim of this study was to describe LVOT velocities with maximal exercise in this age group. METHODS: Subjects up to 18 years old were prospectively enrolled if they had normal results on resting echocardiography and were undergoing exercise testing for other reasons. Subjects with significant comorbidities, suspected cardiomyopathy, or family histories of cardiomyopathy were excluded. Peak LVOT velocities were measured in the upright position using continuous-wave Doppler immediately after maximal exercise. RESULTS: Fifty subjects (mean age, 13.8 ± 2.8 years) were included. Twenty-eight (56%) were male, and 40 (80%) were Caucasian. The median peak LVOT velocity measured immediately after exercise was 2.5 m/sec (range, 1.3-5.9 m/sec). Sixteen subjects (32%) developed peak LVOT velocities of ≥3 m/sec. Twelve of the 16 (75%) with elevated velocities had a dynamic outflow tract Doppler pattern, of whom eight had evidence of intracavitary narrowing on two-dimensional echocardiography. CONCLUSIONS: The development of significant exercise-induced LVOT velocities may be a normal physiologic finding in healthy youth. The measurement of LVOT velocities alone with maximal exercise may not help distinguish patients with hypertrophic cardiomyopathy from healthy children.