Surgical and Cardiac Catheterization Outcomes of Scimitar Syndrome Patients: A Three Decade Single-Center Experience.

Scimitar syndrome (SS) is a rare congenital condition which includes partial anomalous pulmonary venous return (PAPVR) and a variable degree of pulmonary hypoplasia. We describe the clinical features, therapeutic approach and outcomes of patients who underwent cardiac catheterization and/or surgical repair of the scimitar vein at a single institution in the United States. This retrospective cohort study included all patients with SS who underwent scimitar vein surgical repair or cardiac catheterization from October 1989 through August 2021 in a tertiary care center. A total of 84 patients with SS were included and median follow-up time was 74 months. Patients diagnosed with SS under the age of one year had a significantly greater incidence of congenital heart defects (CHD) (p < 0.001), non-cardiac anomalies (p = 0.02), pulmonary hypertension (p = 0.02), and mortality (p = 0.04) compared to those diagnosed over the age of 1 year. Twenty-eight patients underwent surgical repair of the scimitar vein. Overall, eight (10%) patients died. Compared to surviving patients, deceased patients had a significantly higher incidence of pulmonary hypertension (PH), neonatal SS diagnosis, and extracorporeal membrane oxygenation (ECMO) support. Median scimitar vein pressure (20 mmHg) of deceased patients was significantly higher compared to pressures in surviving patients (11 mmHg; p = 0.02). PH, CHD, neonatal SS diagnosis, ECMO support, and markedly elevated scimitar vein pressure are associated with mortality. Scimitar vein surgical repair during infancy is commonly associated with PH and restenosis that requires re-intervention.

Fostering Engagement With Health and Housing Innovation: Development of Participant Personas in a Social Housing Cohort.

BACKGROUND: Personas, based on customer or population data, are widely used to inform design decisions in the commercial sector. The variety of methods available means that personas can be produced from projects of different types and scale. OBJECTIVE: This study aims to experiment with the use of personas that bring together data from a survey, household air measurements and electricity usage sensors, and an interview within a research and innovation project, with the aim of supporting eHealth and eWell-being product, process, and service development through broadening the engagement with and understanding of the data about the local community. METHODS: The project participants were social housing residents (adults only) living in central Cornwall, a rural unitary authority in the United Kingdom. A total of 329 households were recruited between September 2017 and November 2018, with 235 (71.4%) providing complete baseline survey data on demographics, socioeconomic position, household composition, home environment, technology ownership, pet ownership, smoking, social cohesion, volunteering, caring, mental well-being, physical and mental health-related quality of life, and activity. K-prototype cluster analysis was used to identify 8 clusters among the baseline survey responses. The sensor and interview data were subsequently analyzed by cluster and the insights from all 3 data sources were brought together to produce the personas, known as the Smartline Archetypes. RESULTS: The Smartline Archetypes proved to be an engaging way of presenting data, accessible to a broader group of stakeholders than those who accessed the raw anonymized data, thereby providing a vehicle for greater research engagement, innovation, and impact. CONCLUSIONS: Through the adoption of a tool widely used in practice, research projects could generate greater policy and practical impact, while also becoming more transparent and open to the public.

White Matter Damage Relates to Oxygen Saturation in Children With Sickle Cell Anemia Without Silent Cerebral Infarcts.

BACKGROUND AND PURPOSE: Sickle cell anemia is associated with compromised oxygen-carrying capability of hemoglobin and a high incidence of overt and silent stroke. However, in children with no evidence of cerebral infarction, there are changes in brain morphometry relative to healthy controls, which may be related to chronic anemia and oxygen desaturation. METHODS: A whole-brain tract-based spatial statistics analysis was carried out in 25 children with sickle cell anemia with no evidence of abnormality on T2-weighted magnetic resonance imaging (13 male, age range: 8-18 years) and 14 age- and race-matched controls (7 male, age range: 10-19 years) to determine the extent of white matter injury. The hypotheses that white matter damage is related to daytime peripheral oxygen saturation and steady-state hemoglobin were tested. RESULTS: Fractional anisotropy was found to be significantly lower in patients in the subcortical white matter (corticospinal tract and cerebellum), whereas mean diffusivity and radial diffusivity were higher in patients in widespread areas. There was a significant negative relationship between radial diffusivity and oxygen saturation (P<0.05) in the anterior corpus callosum and a trend-level negative relationship between radial diffusivity and hemoglobin (P<0.1) in the midbody of the corpus callosum. CONCLUSIONS: These data show widespread white matter abnormalities in a sample of asymptomatic children with sickle cell anemia, and provides for the first time direct evidence of a relationship between brain microstructure and markers of disease severity (eg, peripheral oxygen saturation and steady-state hemoglobin). This study suggests that diffusion tensor imaging metrics may serve as a biomarker for future trials of reducing hypoxic exposure.