Mitral Valve Intervention, Pulmonary Hypertension, and Survival in 219 Shone's Syndrome Patients.

Background: Relationships between pulmonary hypertension (PHTN), mitral valve intervention (MVI), and death among patients with Shone's syndrome (SS) are unclear. Our recent data suggested temporal progression of MV disease and early survival benefit with MVI at initial operation. In this expanded cohort, we characterized trajectory and impact of PHTN on survival, and its relationship to MVI(s).

Reading tea leaves worldwide: Decoupled drivers of initial litter decomposition mass-loss rate and stabilization.

The breakdown of plant material fuels soil functioning and biodiversity. Currently, process understanding of global decomposition patterns and the drivers of such patterns are hampered by the lack of coherent large-scale datasets. We buried 36,000 individual litterbags (tea bags) worldwide and found an overall negative correlation between initial mass-loss rates and stabilization factors of plant-derived carbon, using the Tea Bag Index (TBI).

Perioperative sildenafil therapy in pulmonary hypertension associated with congenital cardiac disease: An updated meta-analysis.

To provide an updated meta-analysis to evaluate the efficacy and safety of sildenafil on pediatric patients with pulmonary hypertension (PH) associated with congenital heart disease (CHD). To assess the efficacy and safety of sildenafil, five outcomes, time duration of post-operative need for mechanical ventilation, time duration of post-operative ICU stay, length of hospitalization (LOH), the incidence of mortalities and pulmonary arterial pressure to aortic pressure ratio (PAP/AoP) were regarded as primary efficacy outcomes. Standardized mean difference (SMD) was calculated for continuous data.

Surgical strategies to address re-operative complex left ventricular outflow tract and thoracic aortic pathology: Cleveland Clinic children's experience.

Background: Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them.

Methods: Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children's between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed.

Anterior Scalene Anomaly in a Patient With Arterial and Neurogenic Thoracic Outlet Syndrome.

Compression of the neurovascular structures of the upper extremity as they pass through the thoracic outlet result in thoracic outlet syndrome. The myriad of symptoms associated with the syndrome vary based on the structure(s) compressed: the subclavian artery/vein or the inferior trunk of the brachial plexus. This is a common site of compression especially in the presence of upper extremity injury, overuse or anatomical abnormalities.

Coronary artery disease in adults with anomalous aortic origin of a coronary artery.

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins.

Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021).

Characterizing the anatomic spectrum, surgical treatment, and long-term clinical outcomes for patients with Shone's syndrome.

Objective: Shone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival.

Methods: In total, 121 patients with SS who underwent operation at Cleveland Clinic between 1956 and 2021 were reviewed.

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs.

We describe the management of a 2 month-old male (BSA 0.27 m) with congenitally corrected transposition of the great arteries (CCTGA), left and right ventricular outflow tract obstruction, incomplete atrioventricular (AV) canal defect, Ebsteinoid left AV valve, and multiple VSDs. In short, the patient presented with respiratory failure, metabolic acidosis with worsening AV valve regurgitation, and acute decompensation from pulmonary overcirculation.

Critical Care Among Disadvantaged Minority Groups Made Equitable: Trends Throughout the COVID-19 Pandemic.

Background: US racial and ethnic minorities have well-established elevated rates of comorbidities, which, compounded with healthcare access inequity, often lead to worse health outcomes. In the current COVID-19 pandemic, it is important to understand existing disparities in minority groups' critical care outcomes and mechanisms behind these-topics that have yet to be well-explored.

Objective: Assess for disparities in racial and ethnic minority groups' COVID-19 critical care outcomes.

Evaluation of the relationship of cholinergic metabolites in urine and urgency urinary incontinence.

Introduction And Hypothesis: To investigate differences in urine cholinergic metabolites in women with urinary urgency incontinence (UUI) and responders (R) and non-responders (NR) to anti-cholinergic medications (AC).

Methods: Patients with UUI and age-matched controls were evaluated pre- and post-treatment using OABSS, UDI-6 and IIQ-7. Controls were defined as having a cumulative OABSS of zero.

Pandemic Superimposed on Epidemic: Covid-19 Disparities in Black Americans.

Health and healthcare disparities are variances in the health of a population or the care rendered to a population. Disparities result in a disproportionately higher prevalence of disease or lower standard of care provided to the index group. Multiple theories exist regarding the genesis of this disturbing finding.

Children with Narcolepsy type 1 have increased T-cell responses to orexins.

Narcolepsy type 1 (NT1) is caused by severe loss of the orexin neurons, and is highly associated with HLA DQB1*06:02. Using intracellular cytokine staining, we observed a higher frequency of IFN-γ- and TNF-α-producing CD4 and CD8 T-cells in response to orexins in 27 children with NT1 compared to 15 healthy control children. Conversely, no such difference was observed between 14 NT1 and 16 HC adults.