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Abstract：
Bicuspid aortic valve (BAV) is the most commonly encountered congenital malformation in the pediatric population, associated with aortic leaflet degeneration and aortopathy. However, studies on BAV and its complications in children are limited. We present the case of a 16-year-old with type 1B BAV with a raphe with fusion between the right and non-coronary cusps who exhibited severe aortic stenosis, regurgitation, and progressive dilatation of the ascending aorta. Surgical intervention, including aortic valve and aortic root replacement, was performed due to the patient\'s deteriorating condition. Histopathological examination revealed degenerative changes and calcifications in the aortic valve and mucoid fibrosis in the ascending aorta. The results are consistent with BAV patients being predisposed to aortic stenosis and regurgitation due to increased mechanical stress and hemodynamic abnormalities. Although more common in adults and a rare complication in pediatric patients, calcification was previously observed concurrently with rapid valve degeneration in our daily practice. Further studies are needed to improve our understanding of the mechanisms underlying BAV-related complications and refine treatment strategies for pediatric patients.
摘要：
双叶主动脉瓣(BAV)是儿科人群中最常见的先天性畸形,与主动脉小叶变性和主动脉病变有关。然而,对儿童BAV及其并发症的研究有限。我们介绍了一个16岁的1B型BAV患者，右和非冠状动脉尖之间融合的raphe表现出严重的主动脉瓣狭窄，返流,升主动脉进行性扩张.手术干预,包括主动脉瓣和主动脉根部置换,是由于患者病情恶化而进行的。组织病理学检查显示主动脉瓣的退行性改变和钙化以及升主动脉的粘液样纤维化。结果与BAV患者由于机械应力和血流动力学异常增加而倾向于主动脉瓣狭窄和反流的结果一致。尽管在成人中更常见，在儿科患者中也是罕见的并发症，在我们的日常实践中，先前观察到钙化与瓣膜快速变性同时发生。需要进一步的研究来提高我们对BAV相关并发症的机制的理解，并完善儿科患者的治疗策略。
