Left Ventricular Outflow Tract Gradient Is Associated With Coronary Artery Obstruction in Children With Williams-Beuren Syndrome.

OBJECTIVES: Patients with Williams-Beuren syndrome are associated with a high risk of hemodynamic collapse during sedation and/or anesthesia, presumably due to occult coronary obstruction. The objective of this study was to determine the association between transthoracic echocardiogram findings and the presence of coronary obstruction to examine if coronary obstruction can be predicted by transthoracic echocardiogram before anesthesia. DESIGN: Retrospective data analysis of patients with Williams-Beuren syndrome who underwent transthoracic echocardiogram, cardiac catheterization, and/or surgical interventions to determine the correlation between echocardiogram findings and the presence of coronary obstruction determined by cardiac catheterization and/or surgery. SETTING: Single-center university teaching hospital. PARTICIPANTS: The study included 49 patients with Williams-Beuren syndrome who underwent transthoracic echocardiogram, cardiac catheterization, and/or surgical interventions. MEASUREMENTS AND MAIN RESULTS: The only variable associated with coronary artery obstruction was the maximum instantaneous gradient (MIG) across the left ventricular outflow tract (LVOT) on a transthoracic echocardiogram. LVOT MIG ≥ 75 mmHg as the optimal cutoff value was associated with coronary artery obstruction (area under the curve 0.659, odds ratio 6.71, 95% CI 1.31-34.35, p = 0.022). CONCLUSION: LVOT gradient can serve as a good predictor of the presence of coronary obstruction in patients with Williams-Beuren syndrome.

Publisher Correction: The Extended Polydimensional Immunome Characterization (EPIC) web-based reference and discovery tool for cytometry data.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

An unusual route taken by a central venous catheter resulting in inadvertent subclavian artery cannulation: a case report.

Ultrasound-guided cannulation of a central venous catheter into the internal jugular vein (IJV) was performed in the intensive care unit for a critically ill patient. The catheter was inserted into the subclavian artery distally, despite prior ultrasound confirmation of the guidewire position using both the in-plane and out-of-plane views. The catheter was removed successfully by the interventional radiologist with a closure device. To our knowledge, there have been previous case reports of subclavian artery injury during IJV cannulation with ultrasound guidance, but rarely in the setting whereby the guidewire was visualized before dilatation and railroading of the catheter. This case demonstrates that the confirmation of the guidewire in the proximal segment of the vein is insufficient to exclude arterial cannulation.