Hybrid repair of a giant left subclavian artery aneurysm and arch coarctation in a patient with Larsen syndrome

A 34-year-old male was admitted to our emergency department with progressively aggravated hoarseness and sudden onset of chest and neck pain. General examination was remarkable for severe scoliosis (Panel A) and a blood pressure gradient of >20 mmHg between upper and lower extremities. Computed tomography angiography (CTA) revealed a giant left subclavian artery (LSA) aneurysm (55 × 47 mm, asterisk), aortic arch coarctation, and severely tortuous descending aorta. In addition, an isolated left vertebral artery (ILVA) arose from the arch (Panel B-C). Whole-exome sequencing analysis identified p.Gly488Ser mutation in FLNB, indicating a diagnosis of Larsen syndrome. Considering the patient’s refusal of open surgery and the acutely worsening symptoms due to the pending rupture of the LSA aneurysm, a less-invasive hybrid approach was accepted as reasonable by interdisciplinary consensus. First, through the right femoral artery, a 20 F sheath (W.L. Gore and Associates, Newark, Del) was advanced across the arch coarctation, facilitating the delivery of the aortic stent. Then, a Gore cTAG stent was implanted at the arch distal to the innominate artery orifice, followed by a balloon-expandable Cheatham-Platinum stent (NuMED, Hopkinton, USA) deploying at the coarctation segment. Second, the membrane of the cTAG stent was penetrated with an aspiration biopsy needle through the left common carotid artery (LCCA). The fenestration was then expanded by balloons and LCCA was stented with a 120 × 40 mm covered endograft (Panel D–F; Supplementary material online, Video S1). After the endovascular procedures, the ILVA was anastomosed end-to-side to LCCA. Finally, the distal outlet of LSA aneurysm was ligated, and a bypass from LCCA to distal LSA was performed with a 6 mm Gore-Tex prosthetic graft (Panel G). Completion angiography (Panel H; Supplementary material online, Video S2) and postoperative CTA (Panel I) confirmed exclusion of the aneurysm and patency of all the supra-arch vessels. The postoperative course was uneventful and the patient was discharged on the 4th day after surgery.