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Endovascular Management Of An Intercostal Patch Aneurysm In A Patient With Loeys-Dietz Syndrome
Guilherme B. Lima, MD, PhD, Diego S. Rodrigues, MD, Randall R. DeMartino, MD, Bernardo C. Mendes, MD.
Mayo Clinic, Rochester, MN, USA.
Fenestrated branched endovascular aneurysm repair (FB-EVAR) is an important strategy for patients with genetically triggered aortic diseases. Althought open repair remains the standard of care for these patients, procedures often require additional interventions due to the native vessels fragility. Re-do open approach in these situations is challenging with mortality as high as 20%. FB-EVAR has emerged as a viable strategy for these patients, utilizing open surgical grafts as secure landing zones for the endovascular repair. We report a 49-year-old female patient with Loeys-Dietz Syndrome secondary to a TGFBR1 mutation and a 6.4 cm intercostal patch aneurysm eight years after an open thoracoabdominal aortic aneurysm (TAAA) repair and multiple previous aortic operations secondary to a DeBakey I aortic dissection. Written informed consent was provided by the patient. Her previous open repair involved a custom-made trifurcated graft that incorporated the celiac axis (CA), superior mesenteric artery (SMA), and left renal artery (LRA), along with a separate 7-millimeter Dacron graft connecting the main graft to the right renal artery (RRA). At the same level as the trifurcated graft, an intercostal patch was created to include the T9 to T12 intercostal arteries, which gradually expanded to 6.4 cm over the years. A physician-modified stent-graft was then constructed using a tapered Cook TX-2 dissection stent-graft, featuring three inner branches for the CA, SMA, and LRA, and one fenestration for the RRA.kissing balloon maneuver was performed in the SMA and LRA to ensure no compression would compromise the future bridging stents. Using through-and-through access, the stent-graft was advanced from the left femoral artery and deployed in a staggered fashion. A self-expandable stent-graft was deployed in the CA avoiding compression when deploying the SMA and LRA bridging stents. The CA was extended distally with a balloon-expandable stent-graft (BESG). The SMA and LRA were bridged with two BESGs in a kissing maneuver fashion to avoid compression. Finally, the RRA was also bridged with a BESG.The patient required a return to the operating room five days post-procedure for the repair of a type IB endoleak. She was discharged on postoperative day seven, neurologically intact. At six-month follow-up, computed tomography angiography showed that the target vessels remained widely patent, with no evidence of endoleak and a reduction in aneurysm size to 5.8 cm.
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