Eroğlu Ayşe Güler, Atik Sezen Ugan, Çinar Betül, Bakar Murat Tuğberk, Saltik İrfan Levent
Department of Pediatric Cardiology, İstanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey.
Department of Pediatrics, İstanbul University Cerrahpaşa Medical Faculty İstanbul, Istanbul, Turkey.
Pediatr Cardiol. 2018 Dec;39(8):1547-1553. doi: 10.1007/s00246-018-1928-2. Epub 2018 Jul 6.
We evaluated the natural course of congenital aortic valvular stenosis (AVS) and factors affecting AVS progression during long-term follow-up with echocardiography. Medical records of 388 patients with AVS were reviewed; patients with concomitant lesions other than aortic regurgitation (AR) were excluded. Trivial AVS was defined as a transvalvular Doppler peak systolic instantaneous gradient of < 25 mmHg; mild stenosis, 25-49 mmHg; moderate stenosis, 50-75 mmHg; and severe stenosis, > 75 mmHg. Median age of the patients was 3 years (range 0 day to 21 years), and 287 (74%) were male. A total of 355 patients were followed with medical treatment alone for a median of 4.6 years (range 1 month to 20.6 years), and the degree of AVS increased in 75 (21%) patients. The risk of AVS progression was higher when AVS was diagnosed in neonates (OR 4.29, CI 1.81-10.18, p = 0.001) and infants (OR 3.79, CI 2.21-6.49, p = 0.001). After the infancy period, bicuspid valve morphology increased AVS progression risk (OR 2.4, CI 1.2-4.6, p = 0.034). Patients with moderate AVS were more likely to have AVS progression (OR 2.59, CI 1.3-5.1, p = 0.006). Bicuspid valve morphology increased risk of AR development/progression (OR 1.77, CI 1.1-2.7, p = 0.017). The patients with mild and moderate AVS were more likely to have AR development/progression (p = 0.001). The risk of AR development/progression was higher in patients with AVS progression (OR 2.25, CI 1.33-3.81, p = 0.002). Newborn babies and infants should be followed more frequently than older patients according to disease severity. Bicuspid aortic valve morphology and moderate stenosis are risk factors for the progression of AVS and AR.
我们通过超声心动图对先天性主动脉瓣狭窄（AVS）的自然病程及影响AVS进展的因素进行了长期随访评估。回顾了388例AVS患者的病历；排除合并主动脉瓣反流（AR）以外其他病变的患者。轻度AVS定义为经瓣多普勒收缩期峰值瞬时压差<25 mmHg；轻度狭窄为25 - 49 mmHg；中度狭窄为50 - 75 mmHg；重度狭窄为>75 mmHg。患者的中位年龄为3岁（范围0天至21岁），其中287例（74%）为男性。共有355例患者仅接受药物治疗，中位随访时间为4.6年（范围1个月至20.6年），75例（21%）患者的AVS程度加重。新生儿期诊断为AVS时（OR 4.29，CI 1.81 - 10.18，p = 0.001）及婴儿期诊断为AVS时（OR 3.79，CI 2.21 - 6.49，p = 0.001），AVS进展风险更高。婴儿期过后，二叶式瓣膜形态增加了AVS进展风险（OR 2.4，CI 1.2 - 4.6，p = 0.034）。中度AVS患者更易出现AVS进展（OR 2.59，CI 1.3 - 5.1，p = 0.006）。二叶式瓣膜形态增加了AR发生/进展的风险（OR 1.77，CI 1.1 - 2.7，p = 0.017）。轻度和中度AVS患者更易出现AR发生/进展（p = 0.001）。AVS进展患者发生AR的风险更高（OR 2.25，CI 1.33 - 3.81，p = 0.002）。应根据疾病严重程度，对新生儿和婴儿比年长患者进行更频繁的随访。二叶式主动脉瓣形态和中度狭窄是AVS及AR进展的危险因素。
