Ando Makoto, Takahashi Yukihiro, Kikuchi Toshio, Tatsuno Katsuhiko
Department of Pediatric Cardiac Surgery, Sakakibara Heart Institute, Tokyo, Japan.
Ann Thorac Surg. 2003 Oct;76(4):1059-64; discussion 1064-5. doi: 10.1016/s0003-4975(03)00836-1.
Tetralogy of Fallot with subarterial ventricular septal defect is frequently seen among Asians. Compared with infracristal ventricular septal defect, postoperative right ventricular outflow obstruction is more likely because of subpulmonary extension of the defect. Moreover the incidence of aortic regurgitation is a concern because of the absence of a supporting infundibulum.
Four hundred cases of classic tetralogy were reviewed, 61 of which had subarterial ventricular septal defect.
Aortic regurgitation (of more than mild degree) was identified in 7 cases with subarterial and 7 with infracristal ventricular septal defects. The mechanism of infracristal defect was predominantly an annular dilation before surgery. In contrast 5 cases with subarterial defect had progression of aortic regurgitation after operation yielding an actuarial incidence of 29.7% at 20 years. In 2 patients the cause seemed to be fixation or plication of the aortic valve annulus by the ventricular septal patch. Compared with infracristal defect, subarterial defect was associated with increased incidence of reoperation (12.0% versus 1.9% at 10 years, p = 0.01), frequent use of transannular patch (70.5% versus 45.7%, p = 0.0004), and worse New York Heart Association (NYHA) functional class (p = 0.007). Right ventricular outflow obstruction was the reason for reoperation in 3 patients with subarterial defect and was associated with worse NYHA classification in the long-term, on multivariate analysis (p = 0.0002).
Tetralogy with subarterial ventricular septal defect was associated with worse functional outcome. To prevent adverse outcomes, precise suturing of the distal ventricular septal patch, extensive infundibulectomy, lower threshold for transannular incision, and smaller-sized ventricular septal patch placement are warranted.
动脉下室间隔缺损型法洛四联症在亚洲人中较为常见。与嵴下室间隔缺损相比，由于缺损的肺动脉下延伸，术后右心室流出道梗阻的可能性更大。此外，由于缺乏支持性的漏斗部，主动脉瓣关闭不全的发生率也令人担忧。
回顾了400例典型法洛四联症病例，其中61例有动脉下室间隔缺损。
在7例动脉下室间隔缺损和7例嵴下室间隔缺损患者中发现了主动脉瓣关闭不全（程度超过轻度）。嵴下缺损的机制主要是术前瓣环扩张。相比之下，5例动脉下缺损患者术后主动脉瓣关闭不全进展，20年时精算发病率为29.7%。在2例患者中，原因似乎是室间隔补片对主动脉瓣环的固定或折叠。与嵴下缺损相比，动脉下缺损与再次手术发生率增加相关（10年时分别为12.0%和1.9%，p = 0.01），跨环补片的使用频率更高（分别为70.5%和45.7%，p = 0.0004），纽约心脏协会（NYHA）功能分级更差（p = 0.007）。右心室流出道梗阻是3例动脉下缺损患者再次手术的原因，多因素分析显示长期与NYHA分级更差相关（p = 0.0002）。
动脉下室间隔缺损型法洛四联症的功能结局较差。为预防不良结局，有必要精确缝合室间隔远端补片、广泛切除漏斗部、降低跨环切口阈值以及放置较小尺寸的室间隔补片。
