Merkx Remy, Duijnhouwer Anthonie L, Vink Evelien, Roos-Hesselink Jolien W, Schokking Michiel
Department of Pediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands.
Am J Cardiol. 2017 Jul 1;120(1):131-136. doi: 10.1016/j.amjcard.2017.03.245. Epub 2017 Apr 12.
Knowledge of aortic growth in patients with bicuspid aortic valve (BAV) is essential to identify patients at risk for dissection, but data on children remain unclear. We retrospectively evaluated the aortic diameters of all pediatric BAV patients, identified through an echocardiographic database (2005 to 2013). Medical records were reviewed and aortic diameters re-measured on echocardiographic images at diagnosis and if available on variable mid- and endpoints follow-up. Dilatation (z-score >2) was based on 2 different z-score equation methods (Gautier/Campens). In 234 of the total 250 BAV patients, aortic diameters were analyzed; median age was 6.1 years (interquartile range 1.7 to 10), of which 63% were male. Aortic coarctation was present in 81 (36%) patients, 23% had a ventricular septal defect. BAV morphology according to Sievers was as follows: type 0 in 128 patients (55%), type 1 in 96 (41%), and type 2 in 10 (4%). Ascending aortic (AA) dilatation was present in 24% (Gautier) and 36% (Campens) at inclusion. Median follow-up was 4.7 years. The AA was the only location where mean z-scores progressed significantly with age: 0.06 (Gautier) and 0.09 (Campens) units per year between ages 5 and 15 years. Associations for higher AA z-scores at older age were an initial z-score >2 (p <0.001) and aortic valve stenosis (p <0.05). Neither dissection nor preventive aortic surgery occurred. In conclusion, only the AA seems at risk for complication, although no aortic complications occurred in this pediatric BAV cohort. BAV morphology seems associated with larger AA z-scores and valvular dysfunction.
了解二叶式主动脉瓣（BAV）患者的主动脉生长情况对于识别有夹层风险的患者至关重要，但关于儿童的数据仍不明确。我们回顾性评估了通过超声心动图数据库（2005年至2013年）识别出的所有儿科BAV患者的主动脉直径。查阅了病历，并在诊断时以及如有可用的中期和终点随访时，在超声心动图图像上重新测量主动脉直径。扩张（z评分>2）基于两种不同的z评分方程方法（Gautier/Campens）。在总共250例BAV患者中的234例中，分析了主动脉直径；中位年龄为6.1岁（四分位间距1.7至10岁），其中63%为男性。81例（36%）患者存在主动脉缩窄，23%有室间隔缺损。根据Sievers分类，BAV形态如下：128例患者（55%）为0型，96例（41%）为1型，10例（4%）为2型。纳入时升主动脉（AA）扩张在Gautier法中为24%，在Campens法中为36%。中位随访时间为4.7年。AA是唯一平均z评分随年龄显著进展的部位：5至15岁之间每年进展0.06（Gautier法）和0.09（Campens法）单位。年龄较大时AA较高z评分的相关因素为初始z评分>2（p<0.001）和主动脉瓣狭窄（p<0.05）。未发生夹层或预防性主动脉手术。总之，虽然该儿科BAV队列中未发生主动脉并发症，但似乎只有AA有并发症风险。BAV形态似乎与较大的AA z评分和瓣膜功能障碍有关。
