Case Report: Transcatheter interventional procedure to innominate vein turn-down procedure for failing fontan circulation

Abstract

Fontan physiology creates a chronic state of decreased cardiac output and systemic venous congestion, leading to liver cirrhosis/malignancy, protein-losing enteropathy, chylothorax, or plastic bronchitis. Creating a fenestration improves cardiac output and relieves some venous congestion. The anatomic connection of the thoracic duct to the subclavian-jugular vein junction exposes the lymphatic system to systemic venous hypertension and could induce plastic bronchitis. To address this complication, two techniques have been developed. A surgical method that decompresses the thoracic duct by diverting the innominate vein to the atrium, and a percutaneous endovascular procedure that uses a covered stent to create an extravascular connection between the innominate vein and the left atrium. We report a novel variant transcatheter intervention of the innominate vein turn-down procedure without creating an extravascular connection in a 39-month-old patient with failing Fontan circulation complicated by plastic bronchitis and a 2-year post-intervention follow-up.

Keywords: case report; failing fontan; hypoplastic left heart syndrome; modified fenestration; plastic bronchitis; transcatheter interventional procedure.

Figure 1

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).

Figure 2

(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.

Figure 3

(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.

Figure 4

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).