Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercisewere explored in the Pediatric Heart Network Fontan Cross-Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration −4.9 ± 3.8 v. nonfenestration −3 ± 3.5; P < .001). Physiological dead space at peak exercise was higher in fenestrated v. nonfenestrated (25.2 ± 16.1 v. 21.4 ± 15.2; P = .03). There was a weak association between fenestration patency and maximal work and heart rate. Fenestration patency was also weakly correlated with oxygen pulse, work and VE/VCO2 at AT. The effect of persistent fenestration on CPET measurements was minimal in this study, likely due to the cross-sectional design.
Traumatic pulmonary artery pseudoaneurysms (PAPs) are rare in the pediatric population. Although open surgical repair was once the most viable means of care, endovascular approaches have become more favorable as techniques and devices have improved. Currently, endovascular management varies, as covered stent grafts and coil embolization with and without stent coverage are among the various approaches taken to treat PAPs. 1-4 A large-diameter, balloon-expandable, covered endovascular stent has recently been approved by the US Food and Drug Administration as commercially available for use in the United States. At the time of our case, the NuMED covered Cheatham platinum balloon-expandable stent (NuMED, Inc, Hopkinton, NY) was available to centers participating in the Coarctation of the Aorta Stent Trial (COAST) and the Pulmonary Artery Repair with Covered Stents (PARCS) trial. This case demonstrates the youngest reported patient to have undergone successful covered stent implantation for a PAP under emergency use guidelines, performed with permission from the US Food and Drug Administration.
We present a case of a neonate with trisomy 21, ductal-dependent aortic coarctation, and severe respiratory failure secondary to coronavirus disease 2019 (COVID-19) pneumonia. The neonate was managed with venoarterial extracorporeal membrane oxygenation (VA ECMO), palliative stenting of the coarctation, and a vascular plug occlusion of a large patent ductus arteriosus. The patient was successfully weaned off extracorporeal membrane oxygenation (ECMO). The patient is currently awaiting a definitive surgical repair in the near future.
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