Department of Pediatric and Congenital Cardiac Surgery, Athens Heart Surgery Institute, Iaso Children's Hospital, Athens, Greece.
Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, United Kingdom.
Semin Thorac Cardiovasc Surg. 2020;32(2):271-279. doi: 10.1053/j.semtcvs.2020.01.011. Epub 2020 Feb 10.
Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.
主动脉-左心室隧道(AoVT)是一种罕见的先天性畸形,是一条起自升主动脉窦上方,紧邻窦管交界的通道,通向左心室(AoLVT)腔,或罕见地通向右心室(AoRVT)腔。本研究回顾了我们在过去 30 年中治疗 AoVT 的经验。15 个参与中心提交了数据,共 42 例患者在 1987 年至 2018 年间接受了 AoVT 矫正手术。其中 36 例为 AoLVT,6 例为 AoRVT。隧道起源于 28 例(77.8%)患者的右冠状动脉窦上方。对于 AoLVT,大多数手术在早期进行(中位年龄 25 天,范围:1 天-25 岁)。相比之下,AoRVT 诊断和修复较晚(中位年龄 6 岁,范围:1 个月-12 岁)。重要的冠状动脉口移位很常见。AoLVT 最常见的手术修复是仅对主动脉口进行补片关闭(23 例),而在 AoRVT 中,两个口或仅心室口被关闭。11 例(30.5%)AoLVT 患者并存严重或中度主动脉瓣关闭不全,并对 8 例患者进行了主动脉瓣成形术,主要是由于主动脉瓣狭窄。几乎所有患者出院时的主动脉瓣关闭不全均为轻度至轻度。早期死亡率为 7.14%,其中 3 例 AoLVT 患者死于心力衰竭。有 2 例早期再次手术和 1 例晚期死亡。AoVT 是一种罕见的畸形。AoLVT 通常需要在婴儿期进行手术。AoRVT 更为罕见,在生命后期诊断和修复。通过补片关闭进行手术修复,并根据需要进行主动脉瓣修复,可获得良好的效果。