Endovascular repair of intercostal patch aneurysms in a patient with Loyes-Dietz syndrome.

Open repair has remained the first-line of treatment of patients with genetically triggered aortic diseases. Up to 30% of patients could require additional aortic operations after the first aortic repair. Reoperative open surgical repair for recurrent thoracoabdominal aortic, intercostal, or visceral patch aneurysms carries a mortality rate as high as 23%. Patients with genetic triggered aortic diseases are prone to develop patch aneurysms owing to weakening of the native aorta. Fenestrated-branched endovascular aortic repair has been used selectively, provided the proximal landing zone can be based on an open surgical graft. We report the case of a 45-year-old female patient with Loeys-Dietz syndrome and multiple prior aortic repairs. She had presented with multiple saccular aortic patch aneurysms. The patient provided written informed consent for enrollment in a prospective investigational device exemption study and for the report of her case details.

Computed tomography angiography demonstrated three aortic patch aneurysms involving a proximal intercostal patch (2.5 cm), distal intercostal patch (7 cm), and left renal artery patch (5 cm). The patient had two left-sided renal arteries incorporated as a single Carrel patch. Her medical history was notable for prior repair of a DeBakey I aortic dissection with total arch and aortic valve replacement, open thoracoabdominal aortic aneurysm repair, and infrarenal endovascular aortic repair.

A stent graft was designed with three directional branches for the celiac axis, superior mesenteric artery, and right renal artery and two fenestrations for the two left renal arteries. Both left renal arteries were targeted using a preloaded catheter system. The delivery system was exchanged for a coaxial system, which was used for sequential catheterization and stenting of the right renal and mesenteric branches using a total femoral approach. The coaxial system included an 8.5F steerable sheath prepared with a 0.014-in. guidewire, which was inserted via a puncture in the valve and looped around the sheath. A 6F shuttle was used as the dilator of the 8.5F steerable sheath, and this system was introduced inside a 12F DrySeal (W.L. Gore & Associates, Flagstaff, AZ). When the steerable sheath exited the 12F DrySeal and the curve was created, a hemostatic clamp was applied to both ends of the guidewire to optimize support for advancement of the bridging stent grafts (Fig 1). Procedure details are illustrated in the Video.

Fig 1

Illustration showing the steerable sheath system.

The patient was discharged home on postoperative day 4 without complications. At the 6-month follow-up examination, the patient reported no complications, and repeat computed tomography angiography demonstrated widely patent target vessels, no endoleak, and a decrease in the largest patch aneurysm diameter to 3.5 cm.