Cohen Meryl S, Marino Bradley S, McElhinney Doff B, Robbers-Visser Daniëlle, van der Woerd Wendy, Gaynor J William, Spray Thomas L, Wernovsky Gil
Department of Pediatrics, University of Pennsylvania School of Medicine, Cardiac Center at Children's Hospital of Philadelphia, 19104, USA.
J Am Coll Cardiol. 2003 Aug 6;42(3):533-40. doi: 10.1016/s0735-1097(03)00715-0.
We sought to assess the prevalence and progression of neo-aortic root dilation and valvar regurgitation after staged reconstruction for hypoplastic left heart syndrome (HLHS).
In HLHS, the pulmonary valve functions as the neo-aortic valve. Neo-aortic valve dysfunction has been observed after arterial switch operation and the Ross procedure.
Patients with HLHS born before January 1995 who had the Fontan operation and had serial echocardiograms were included. Echocardiograms were reviewed preoperatively, after each surgical reconstruction, and at most recent follow-up for neo-aortic root size and severity of neo-aortic regurgitation (AR). Potential risk factors for neo-aortic valve dysfunction were assessed.
Fifty-three patients met inclusion criteria. Bidirectional superior cavopulmonary anastomosis as an interim procedure was performed in 39 patients (74%). Median duration of follow-up was 9.2 (range 5.1 to 21) years. During follow-up, the neo-aortic root progressively dilated out of proportion to body size over time, with 52 patients (98%) having a Z-score >2 at most recent follow-up. Neo-AR was present in 61% of patients at most recent follow-up, with progression over time in 26 patients (49%). However, neo-AR was more than mild in only three patients. Significantly larger neo-aortic root Z-scores were observed in patients with any degree of neo-AR at most recent follow-up. No other anatomic or clinical variables correlated with severity of neo-AR or root dilation.
After staged reconstruction for HLHS, neo-aortic root dilation and neo-AR progress over time. Early volume unloading does not have a beneficial impact on dilation of the neo-aortic root. These findings raise concerns about neo-aortic valve function into adulthood.
我们试图评估左心发育不全综合征（HLHS）分期重建术后新主动脉根部扩张及瓣膜反流的发生率和进展情况。
在HLHS中，肺动脉瓣起到新主动脉瓣的作用。在动脉调转术和罗斯手术之后，已观察到新主动脉瓣功能障碍。
纳入1995年1月之前出生、接受了Fontan手术且有系列超声心动图检查结果的HLHS患者。对术前、每次手术重建后以及最近一次随访时的超声心动图进行回顾，以评估新主动脉根部大小及新主动脉反流（AR）的严重程度。评估新主动脉瓣功能障碍的潜在危险因素。
53例患者符合纳入标准。39例患者（74%）接受了双向腔肺吻合术作为中间手术。中位随访时间为9.2年（范围5.1至21年）。随访期间，新主动脉根部随时间逐渐扩张，与身体大小不成比例，52例患者（98%）在最近一次随访时Z值>2。在最近一次随访时，61%的患者存在新AR，26例患者（49%）随时间病情进展。然而，只有3例患者的新AR超过轻度。在最近一次随访时，任何程度新AR的患者新主动脉根部Z值均显著更大。没有其他解剖或临床变量与新AR严重程度或根部扩张相关。
HLHS分期重建术后，新主动脉根部扩张和新AR随时间进展。早期容量负荷减轻对新主动脉根部扩张没有有益影响。这些发现引发了对成年期新主动脉瓣功能的担忧。
