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Background: Brugada syndrome (BrS) is a genetic disorder that increases the risk of ventricular tachyarrhythmias and sudden cardiac death (SCD). Certain drugs (propofol, local anaesthetics), fever, bradycardia, increased vagal tone and electrolyte imbalances can trigger or worsen BrS arrhythmias., Objective: To evaluate the incidence of malignant ventricular arrhythmias during the perioperative period in patients with BrS, hypothesising that common anaesthetic drugs may be safe to use during daily clinical practice., Design: The BRUGANAES study was an observational, retrospective project including BrS patients who underwent various types of anaesthesia., Setting: BrS patients undergoing any type of anaesthesia intervention from 1 January 2006, to 31 December 2023, from a tertiary hospital in Barcelona., Main Outcome Measures: The primary outcome was the occurrence of malignant ventricular arrhythmias and/or SCD during and up to 30 days postanaesthesia. Secondary outcomes included adverse events during hospitalisation, 30-day readmission rates and 30-day mortality rates., Results: Among 652 BrS patients registered in the hospital, 111 patients and 189 procedures were analysed. General anaesthesia was administered in 51.3% of cases, sedation in 36% and regional/neuraxial anaesthesia exclusively in 12.7%. Overall, nonrecommended drugs (propofol, ketamine and local anaesthetics) were used in 129 (68.3%) procedures, either bolus and/or continuous infusion. Epidural blocks were performed in 34% of regional anaesthesia cases, mostly in obstetrics, and subarachnoid blocks in 31.8%. The primary outcome occurred in two patients intraoperatively (1% of procedures): one with bradycardia-induced ventricular fibrillation after a nonrecommended drug and one with transient ventricular tachycardia after a drug not listed as potentially harmful., Conclusion: To date, this is one of the largest cohorts describing the perioperative approach for BrS patients, including a wide range of anaesthesia procedures and drugs. Most of the patients undergoing anaesthesia for an interventional procedure received an anaesthetic drug classified as not recommended., (Copyright © 2025 European Society of Anaesthesiology and Intensive Care. Unauthorized reproduction of this article is prohibited.)

Spontaneous coronary artery dissection is an uncommon, but not insignificant cause of acute coronary syndrome that overwhelmingly affects middle-aged women. The pathophysiology of coronary dissection appears to be an outside-in mechanism, where the initiating event is not an intimal tear but rather the formation of an intramural hematoma, which compromises blood flow by reducing the arterial lumen. Considering this mechanism, it is clear to see how intracoronary imaging techniques, such as optical coherence tomography and intravascular ultrasound, are most accurate in the diagnosis. However, they carry a high rate of complications and are therefore generally avoided when the clinical scenario and angiographic appearance both support the diagnosis of spontaneous coronary artery dissection. The natural history of the disease is toward healing of the vessel wall and restoration of blood flow. Therefore, conservative medical management is the preferred approach unless there are high-risk factors such as hemodynamic instability, signs of ischemia and severe proximal or multivessel lesions, in which percutaneous or surgical revascularization should be considered. Perioperative evaluation of these patients must take into account several aspects of this disease. Most of these patients will be receiving single or dual antiplatelet therapy, so one must consider the timing of the event and the surgical hemorrhagic risk when deciding to stop these therapies. Extracoronary vascular disease also must be assessed because it can have an effect on patient monitoring and risk of postoperative complications., Competing Interests: Conflict of Interest None., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The Publisher regrets that this article is an accidental duplication of an article that has already been published in Journal of Cardiothoracic and Vascular Anesthesia, 36 (2022) 2793-2802, http://dx.doi.org/10.1053/j.jvca.2021.10.030. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/our-business/policies/article-withdrawal., Competing Interests: Declaration of Competing Interest Dr. Neuburger is a consultant for Medtronic. The remaining authors do not have any financial or personal conflicts of interest to disclose.

Many patients experiencing a rare disease remain undiagnosed even after genomic testing. Reanalysis of existing genomic data has shown to increase diagnostic yield, although there are few systematic and comprehensive reanalysis efforts that enable collaborative interpretation and future reinterpretation. The Undiagnosed Rare Disease Program of Catalonia project collated previously inconclusive good quality genomic data (panels, exomes, and genomes) and standardized phenotypic profiles from 323 families (543 individuals) with a neurologic rare disease. The data were reanalyzed systematically to identify relatedness, runs of homozygosity, consanguinity, single-nucleotide variants, insertions and deletions, and copy number variants. Data were shared and collaboratively interpreted within the consortium through a customized Genome-Phenome Analysis Platform, which also enables future data reinterpretation. Reanalysis of existing genomic data provided a diagnosis for 20.7% of the patients, including 1.8% diagnosed after the generation of additional genomic data to identify a second pathogenic heterozygous variant. Diagnostic rate was significantly higher for family-based exome/genome reanalysis compared with singleton panels. Most new diagnoses were attributable to recent gene-disease associations (50.8%), additional or improved bioinformatic analysis (19.7%), and standardized phenotyping data integrated within the Undiagnosed Rare Disease Program of Catalonia Genome-Phenome Analysis Platform functionalities (18%)., (Copyright © 2022 Association for Molecular Pathology and American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Background: The extracellular volume (ECV) and intracellular volume (ICV) estimated by bioimpedance analysis (BIA) deviates markedly from the textbook volumes of 20% and 40% of the body weight (BW). We estimated the transcellular exchange of water by calculating solute equilibriums after fluid challenges to examine whether the BIA or the textbook volumes are likely to be most correct., Methods: Data was retrieved from 8 healthy male volunteers who received 25 mL/kg of Ringer's solution or 3-5 mL/kg of hypertonic (7.5%) saline over 30 min after the ECV and ICV had been estimated by BIA. The exchange of water between the ECV and the ICV was calculated according to a sodium equation and an osmolality equation. Simulations were performed, where deviating body fluid volumes were applied., Results: The mean ECV measured with BIA was 24.9% of BW (p < 0.05 versus the "textbook" volume). Mean ICV measured with BIA was 22.3% of BW (p < 0.05). The sodium and osmolality equations correlated closely with respect to the translocation of water across the cell membrane (r 2  = 0.86). By applying the "textbook" ECV, the sodium equation indicated that Ringer's solution exchanged negligible amounts of water, while hypertonic saline withdrew 1.4 L from the ICV to the ECV. By contrast, applying the BIA-derived ECV to the sodium equation implied that 3 L of water would be translocated from the ECV to the ICV once hypertonic saline was administered., Conclusion: The "textbook" ECV and ICV volumes but not the BIA-derived volumes were consistent with the fluid shifts obtained by two solute equations., (© 2022. The Author(s).)