Cardiología Pediátrica, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, España.
Cardiología Pediátrica, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, España.
Med Clin (Barc). 2017 Nov 9;149(9):391-396. doi: 10.1016/j.medcli.2017.03.041. Epub 2017 Jun 16.
Dilatation of the ascending aorta associated with bicuspid aortic valve is a major cause of morbidity and mortality in adults. The main objective was to recognize the aortic involvement in children, its characteristics and risk factors.
Aortic measures of all pediatric patients with bicuspid aortic valve followed in a tertiary pediatric hospital between 1997 and 2015 were retrospectively taken. Patients with syndromes associated with aortic dilatation were excluded (n=17).
Two hundred and six patients were included, 67.9% males. The commonest opening pattern was horizontal: 137 (66.7%). Half of the patients (101) had a history of surgical aortic coarctation, 46 (22.3%) had≥moderate aortic valve stenosis and 13 (6%) had≥moderate aortic insufficiency. Mean follow-up time was 6.1 (4.9) years; diagnosis of aortic dilatation was made during the first year of follow-up. Progression of the dilatation of the ascending aorta was noted in 17.1%, and of the aortic root in 2.5%. More than one-third (80/206) had aortic dilatation (z-score>2). The ascending aorta was exclusively affected in 70/80 patients, with sparing of the aortic root. In the multivariate analysis, patients with dilatation of the ascending aorta were associated with absence of coarctation (P=.001) and vertical opening pattern (P=.007).
Pediatric patients with bicuspid aortic valve warrant medical follow-up for the frequent association with valve impairment and/or dilatation of the ascending aorta.
与二叶式主动脉瓣相关的升主动脉扩张是成年人发病率和死亡率的主要原因。主要目的是识别儿童的主动脉受累、其特征和危险因素。
回顾性地分析了 1997 年至 2015 年期间在一家三级儿科医院接受治疗的所有二叶式主动脉瓣患儿的主动脉测量值。排除与主动脉扩张相关的综合征患者（n=17）。
共纳入 206 例患者，其中 67.9%为男性。最常见的开口模式为水平型：137 例（66.7%）。半数患者（101 例）有主动脉缩窄手术史，46 例（22.3%）有≥中度主动脉瓣狭窄，13 例（6%）有≥中度主动脉瓣关闭不全。平均随访时间为 6.1（4.9）年；在随访的第一年诊断出主动脉扩张。17.1%的患者升主动脉扩张进展，2.5%的患者主动脉根部扩张。超过三分之一（80/206）的患者有主动脉扩张（z 评分>2）。80 例患者中有 70 例升主动脉单纯受累，主动脉根部未受累。多变量分析显示，升主动脉扩张的患者与无缩窄（P=.001）和垂直开口模式（P=.007）相关。
二叶式主动脉瓣患儿需要进行医学随访，因为其常伴有瓣膜损害和/或升主动脉扩张。
