case_images: PMC10876887_01
case_images: Coronal images from a three-dimensional, heavily T2-weighted MRI sequence (siemens magnetom vida 3 T). Cisterna chyli (A;  ) and the lower third of the thoracic duct (A,B; arrowheads) are demonstrated. The tubular aspect of the thoracic duct is lost in its middle and upper portions with an increased abnormal signal intensity at these levels (B; arrows). Increased abnormal signal intensity is also seen in the bilateral supraclavicular regions, in particular on the left side (B; *) extending into the mediastinum and with an interstitial pattern into the right lung (A-C), surrounding the right bronchus (C; arrow).
case_images: fped-12-1341443-g001.jpg
case_images: PMC10876887_01_fped-12-1341443-g001.jpg
case_images: F1
case_images: Primarily, a magnetic resonance lymphangiography (Siemens Magnetom Vida 3 T, Erlangen, Germany) showed a re-permeabilization of the TD just above the subdiaphragmatic clip and a pulmonary lymphatic perfusion syndrome type 4 mainly from the right upper lobe (Figure 1).
case_images: (A) native fontan circulation without any stenosis (B) permeable fenestration connecting the innominate vein to the single atrium (C) stenting of the fenestration (formula  418 7/16 mm) (D) placement of ADO I  10/8 mm to close the distal part of the innominate vein.
case_images: fped-12-1341443-g002.jpg
case_images: PMC10876887_01_fped-12-1341443-g002.jpg
case_images: F2
case_images: Afterward, an Amplatzer Duct Occluder I (ADO I) 10/8 mm (Abbott, Illinois, USA) was placed at the distal part of the innominate vein (Figure 2).
case_images: (A) objectivation of the fenestration thrombosis (B) removal of the ADO and reconfirmation of the fenestration thrombosis (C) local thrombolysis with alteplase (D) covered stent (gore viabahn  7/19 mm) in the fenestration and ADO I  10/8 mm closing the distal innominate vein creating a transcatheter innominate vein turn-down procedure.
case_images: fped-12-1341443-g003.jpg
case_images: PMC10876887_01_fped-12-1341443-g003.jpg
case_images: F3
case_images: The device was released after a fluoroscopic check of the position and absence of occlusion of the stented fenestration (Figure 3).
case_images: Unenhanced CT image of the ADO I  10/8 mm closing the distal innominate vein and the covered stent (gore viabahn  7/19 mm) in the fenestration with proximal stenosis of the fenestration (arrow).
case_images: fped-12-1341443-g004.jpg
case_images: PMC10876887_01_fped-12-1341443-g004.jpg
case_images: F4
case_images: An unenhanced chest CT showed significant proximal stenosis of the fenestration (Figure 4). The innominate vein-RA fenestration was stenotic (Figure 4) with an invasive mean gradient of 9 mmHg (15 mmHg in the innominate vein vs. 6 mmHg in the RA).