Publications by authors named "R D Hirsch"

Background: Pulmonary vein stenosis in children is associated with a poor prognosis. However, the cause and risk factors for mortality remain uncertain.

Methods: This retrospective, single-center study identified children with primary and secondary pulmonary vein stenosis through a cardiac catheterization database.

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Background: Advancements in cardiac catheterization have improved survival for pediatric congenital heart disease patients, but the associated ionizing radiation risks necessitate ethical consideration.

Methods: This study presents an empirical model, developed from 3131 unique pediatric procedures, to establish alert levels based on a patient's lateral thickness of the thorax for various procedural categories during diagnostic or interventional cardiac catheterization. The model uses linear regression of logarithmic reference air kinetic energy released per unit mass (KERMA) and air KERMA area product, also referred to as dose area product, to set alert levels at the top 95% and 99% of patient data.

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Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).

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This research letter evaluates the diagnostic accuracy of three physician groups-pediatric cardiologists, adult congenital heart disease (ACHD) specialists, and general cardiologists-and compares it to the performance of an AI (Artificial Intelligence) LLM model. Based on eight targeted questions in adult congenital heart disease, the analysis highlights significant disparities in correct answer rates, with congenital specialists achieving the highest accuracy and the AI model - the lowest.

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Missense mutations in exon 3 of , the gene encoding β-catenin, are associated with poor outcomes in endometrial carcinomas (EC). Clinically, mutation status has been difficult to use as a predictive biomarker as β-catenin oncogenic activity is modified by other factors, and these determinants are unknown. Here we reveal that CD73 restrains the oncogenic activity of exon 3 β-catenin mutants, and its loss associates with recurrence.

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