Abstract Body (Do not enter title and authors here): Background: The hypoplastic aortic arch syndrome (HAAS) was defined as a combination of hypoplastic aortic arch and complex proximal descending aortic anomalies (such as aortic aneurysms, aortic coarctation, or aortic dissection). In this study, we proposed a novel 'double arch' technique as a simple surgical procedure to treat HAAS, 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 technique.
Methods: J-shaped mini-sternotomy and left posterolateral thoracotomy were both performed. After dissociating the aorta to the proximal descending aorta, 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 the descending aorta was 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 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, whichever is longer. Secondary endpoints included aortic reoperation, newly developed aneurysms, and the blood pressure difference between the upper and lower extremities.
Results: Twelve patients with HAAS were enrolled from 2015 to 2022. Patients were diagnosed using computed tomography. The median age (interquartile range, IQR) was 26.5 (17.0, 39.0) years, and 8 patients were female (66.7%). The median operation time (IQR) was 4.7 (3.8, 5.5) hours. All patients survived to discharge and completed the follow-up. The median follow-up time (IQR) was 5.42 (3.25, 5.97) years. During the follow-up, no patient died or was required for reoperation. In addition, no newly developed aneurysm and severe adverse event was recorded. The median blood pressure difference (IQR) between the upper and lower extremities was 9 (4, 17) mmHg.
Conclusions: The 'double arch' technique is a viable alternative surgical treatment for the HAAS. This technique is expected to significantly simplify the surgical procedure and improve the long-term prognosis of patients.