Double arch technique - a novel surgical procedure for the hypoplastic aortic arch syndrome

Abstract Background The hypoplastic aortic arch syndrome was defined as a combination of hypoplastic aortic arch and complex proximal descending aortic anomalies (such as aortic aneurysm, aortic coarctation, aortic tortuosity, or aortic dissection). In this study, we proposed a novel 'double arch' technique as a simple surgical procedure to treat the hypoplastic aortic arch syndrome, which could eliminate the requirement for cardiopulmonary bypass. Purpose This study aimed to investigate the surgical outcomes of patients who underwent the 'double arch' technique, and further evaluate the effectiveness and safety of this novel technique. Methods Briefly, J-shaped mini-sternotomy and left posterolateral thoracotomy were both performed in this technique. The ascending aorta, aortic arch, and proximal descending aorta were dissociated. After that, the ascending aorta was occluded using a side-biting clamp, followed by an end-to-side anastomosis between the proximal end of vascular graft and the ascending aorta. Similarly, the thoracic descending aorta was occluded with two aortic clamps beyond the opening of the left subclavian artery, and was then transected between the clamps, followed by an end-to-end anastomosis between the distal end of vascular graft and the distal descending aorta, while the proximal descending aorta was closed with sutures. The transection site of the aorta could be adjusted according to the extent of the lesions, and the vascular graft was placed in the left thoracic cavity. The operation was performed at room temperature. The primary endpoint was early mortality, which referred to in-hospital death and/or death within 30 days after surgery (including intraoperative death), whichever is longer. Critical secondary endpoints included aortic reoperation, newly developed aneurysms, and the blood pressure difference between the upper and lower extremities. Results Twelve patients with hypoplastic aortic arch syndrome were enrolled from December 2015 to February 2022. Patients were diagnosed using computed tomography. The median age (interquartile range, IQR) was 26.5 (17.0, 39.0) years (ranging from 12.0 to 55.0), and 8 patients were female (66.7%). The median operation time (IQR) was 4.7 (3.8, 5.5) hours (ranging from 2.8 to 7.3). All patients survived to discharge and completed the follow-up. The median follow-up time (IQR) was 5.42 (3.25, 5.97) years (ranging from 0.02 to 6.25). During the follow-up, no patient died or was required for reoperation. In addition, no newly developed aneurysm was found, and no severe adverse event was recorded. The median blood pressure difference (IQR) between the upper and lower extremities was 9 (4, 17) mmHg (ranging from 3 to 27). Conclusions The 'double arch' technique is a viable alternative surgical treatment for the hypoplastic aortic arch syndrome. This technique is expected to significantly simplify the surgical procedure and improve the long-term prognosis of patients.Diagram of the 'double arch' techniqueCT scans of pre- and post-operation