Abstract Body (Do not enter title and authors here): Case description: A 64-year-old male was referred for transcatheter aortic valve replacement (TAVR) due to critical bicuspid aortic valve stenosis (Figure 1A/B) with multiple episodes of unexplained syncope. A multidisciplinary discussion determined that he was at high surgical risk due to multiple comorbidities, including developmental delay, and prior renal transplantation with baseline serum creatinine of 1.8 mg/dL.

Transfemoral and trans-subclavian arterial approaches for valvular replacement were not anatomically feasible due to the circumflex aortic arch (Figure 2A/B). Left transcarotid approach was considered due to the location of the vessel as the first branch of the ascending aorta (Figure 2B, 3A) but was unsuitable due to significant left carotid artery calcification. Thus, the decision was to perform a transaortic approach under general anesthesia.

A right femoral artery sheath was inserted, and a wire was advanced to the aortic root (Figure 2B) for pigtail placement used to guide positioning during TAVR. A mini upper hemi-sternotomy was performed and J-ed into the right second intercostal space. The pericardium was suspended, and two concentric purse string sutures were placed in the aorta at the site of sheath placement. The valve was crossed and following valvuloplasty, the 29-mm Edwards SAPIEN 3 valve was delivered across the aortic valve annulus and deployed following balloon inflation with rapid ventricular pacing (Figure 3A). Postoperative echocardiography showed no prosthetic stenosis (Figure 3B) and no valvular or paravalvular regurgitation.

Discussion: To our knowledge, this is the first report of a transaortic TAVR performed on a patient with a circumflex aortic arch. Circumflex aortic arch is a rare congenital anomaly and poses challenges to transcatheter aortic valve interventions from the extremities. This case illustrates the potential of transaortic TAVR as treatment for symptomatic aortic stenosis when there are no other options for access. Multidisciplinary collaboration and comprehensive imaging are critical in tailoring individualized cardiovascular intervention in patients with congenital anatomical variations.