Background: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings.
Methods: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used.
Results: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p = .04). The length of stay (16 vs. 13 days, p = .05) and total charges incurred ($283, 280 vs. $234, 106, p = .03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality.
Conclusion: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non-ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome.
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http://dx.doi.org/10.1111/jocs.16742 | DOI Listing |
Rheumatology (Oxford)
January 2025
Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy.
Objective: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that exhibits a wide spectrum of clinical manifestations. The recent identification of activating mutations in the MAPK-ERK pathway in patients with ECD has led to the introduction of targeted therapies. The most commonly used targeted therapies are BRAF- and MEK-inhibitors, which are highly effective but also carry significant toxicity.
View Article and Find Full Text PDFKidney Int Rep
January 2025
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Introduction: The aim of this study was to describe the clinical features and treatment responses of Rosai-Dorfman disease (RDD) and Erdheim-Chester disease (ECD) with kidney involvement.
Methods: We retrospectively analyzed patients with RDD and ECD with kidney involvement from 2005 to 2023, evaluating kidney function changes, as well as computed tomography (CT), and metabolic responses.
Results: The study included 4 patients with RDD and 44 with ECD, with median ages of 58 and 51 years, respectively.
Metastatic triple-negative breast cancer has a poor prognosis and poses significant therapeutic challenges. Until recently, limited therapeutic options have been available for patients with advanced disease after failure of first-line chemotherapy. The aim of this review is to assess the current evidence supporting second-line treatment options in patients with metastatic triple-negative breast cancer.
View Article and Find Full Text PDFClin Ophthalmol
January 2025
Department of Pediatrics, Faculty of Medicine, Chulalongkorn University Bangkok, Bangkok, Thailand.
Purpose: To describe the demographic profile, clinical characteristics, and treatment trends of Fuchs endothelial corneal dystrophy (FECD) in Thai patients, reflecting the evolving landscape of corneal transplantation in this region.
Patients And Methods: This retrospective cohort study included 900 patients (1,743 eyes) diagnosed with FECD at a tertiary referral center in Thailand between January 2017 and June 2023. Demographic, clinical, and surgical data were analyzed, focusing on best-corrected visual acuity (BCVA), central corneal thickness (CCT), endothelial cell density (ECD), surgical interventions, and graft survival rate.
J Fr Ophtalmol
January 2025
Service d'ophtalmologie, hôpital La Timone, 264, rue Saint-Pierre, 13005 Marseille, France.
Purpose: To analyze the outcomes and frequency of complications after Descemet's membrane endothelial keratoplasty (DMEK) and evaluate the parameters associated with the occurrence of these complications.
Methods: A multicenter retrospective study of 143 DMEK procedures performed consecutively by five surgeons between June 2018 and March 2021 was performed. Surgeon-specific surgical and graft characteristics were also assessed.
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