Oner Taliha, Akgun Gokmen, Ergin Selma Oktay, Karadag Huseyin, Yucel İlker Kemal, Celebi Ahmet
Department of Pediatric Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Uskudar, Istanbul, Turkey.
Pediatr Cardiol. 2019 Jun;40(5):980-986. doi: 10.1007/s00246-019-02102-6. Epub 2019 Apr 23.
Aneurysms of the ascending aorta are frequently found in patients with a bicuspid aortic valve (BAV). This study assessed the risk factors of ascending aortic aneurysms and aortic elasticity in children with BAV. The study included 66 patients with no history of transcatheter intervention or surgical procedure who had been diagnosed with isolated BAV. Echocardiographic, blood pressure, and pulse measurements were obtained for all patients. The BAVs were classified as described by Sievers et al. (J Thorac Cardiovasc Surg 133:1226-1233, 2007), and aortic elasticity parameters were calculated using various formulas. The patients were divided into groups with and without cusp fusion, aortic stenosis (AS), aortic regurgitation (AR), or mixed lesions; the groups were then compared. The mean patient age was 10.43 ± 3.91 years; 15%he patients had no AS or AR, 33% had both AS and AR, 17% had AS alone, and 35% had AR alone. The most common type of BAV was type 5, and the ascending aorta z-scores were higher in children with mixed lesions and without a cusp fusion. Aortic distensibility (AD) was significantly higher, and the stiffness index was significantly lower, in patients with an ascending aorta z-score > 4. The ascending aortic z-scores were higher in the no-fusion and mixed lesion (AS + AR) groups, especially those originating from post-stenotic dilation due to AS. The AD was increased in patients with an ascending aorta z-score > 4. Patients should thus be monitored closely for dissection risk, and preventive medical treatment should be started early in those with AS without cusp fusion.
升主动脉瘤常见于二叶式主动脉瓣（BAV）患者。本研究评估了BAV患儿升主动脉瘤的危险因素及主动脉弹性。该研究纳入了66例无经导管干预或手术史且被诊断为孤立性BAV的患者。对所有患者进行了超声心动图、血压和脉搏测量。BAV按照Sievers等人（《胸心血管外科杂志》133:1226 - 1233, 2007年）的描述进行分类，并使用各种公式计算主动脉弹性参数。患者被分为有或无瓣叶融合、主动脉狭窄（AS）、主动脉反流（AR）或混合病变的组，然后对这些组进行比较。患者的平均年龄为10.43 ± 3.91岁；15%的患者无AS或AR，33%的患者同时有AS和AR，17%的患者仅有AS，35%的患者仅有AR。最常见的BAV类型是5型，混合病变且无瓣叶融合的儿童升主动脉z值更高。升主动脉z值>4的患者，主动脉扩张性（AD）显著更高，僵硬度指数显著更低。无融合和混合病变（AS + AR）组的升主动脉z值更高，尤其是那些因AS导致的狭窄后扩张引起的病变。升主动脉z值>4的患者AD增加。因此，应密切监测患者的夹层风险，对于无瓣叶融合的AS患者应尽早开始预防性药物治疗。
