Prevalence of Bicuspid Aortic Valve and Associated Aortopathy in Newborns in Copenhagen, Denmark
Prevalence of Bicuspid Aortic Valve and Associated Aortopathy in Newborns in Copenhagen, Denmark
复制标题
DOI：
10.1001/jama.2020.27205
复制
发表时间：
2021-02-09
影响因子：
120.7
通讯作者：
Bundgaard, Henning
中科院分区：
文献类型：
--
作者：
Sillesen, Anne-Sophie;Vogg, Ottilia;Bundgaard, Henning
重要性 二叶式主动脉瓣 (BAV) 的患病率和特征主要来自选定的队列。 BAV 与主动脉病相关，但尚不清楚它是否代表胎儿发育缺陷或继发于瓣膜动力学异常。目的 确定 BAV 和 BAV 亚型的患病率，并描述大型新生儿队列中相关的主动脉病。设计、设置和参与者 哥本哈根婴儿心脏研究是一项基于人群的横断面研究，向 2016 年 4 月 1 日至 2018 年 10 月 31 日期间在哥本哈根出生的所有新生儿开放。患有 BAV 的新生儿与具有三尖瓣主动脉瓣的新生儿（非 BAV 组）在性别、单胎/双胎妊娠、胎龄、体重和检查时年龄以 1:2 进行匹配。出生后 60 天内接受经胸超声心动图检查。主要结果和措施 主要结果是 BAV 患病率和类型，即根据 Sievers 和 Schmidtke 分类系统（分为 0、1 或 2 型，数字表示中缝数），中缝数和中缝或尖点（无中缝）的空间方向。次要结果是瓣膜功能和 BAV 相关主动脉病，定义为主动脉直径 z 评分等于或大于 3 或缩窄。结果 总共有 25 556 名新生儿（51.7% 为男性；平均年龄为 12 [SD，8] 天）接受了超声心动图检查。 196 名新生儿被诊断为 BAV（患病率为 0.77% [95% CI, 0.67%-0.88%]），男女比例为 2.1:1。 17 名新生儿中 BAV 被分类为 0 型（8.7% [95% CI, 5.5%-13.5%]），178 名新生儿中 BAV 被分类为 1 型（90.8% [95% CI, 86.0%-94.1%]）（147 名[75.0% {95% CI, 68.5%-80.5%}] 左右冠状动脉中缝，27 [13.8% {95% CI, 9.6%-19.3%}] 右冠状动脉-非冠状动脉中缝，4 [2.0% {95% CI, 0.8%-5.1%}] 左冠状动脉-非冠状动脉中缝），以及 1 例 2 型（0.5% [95% CI, 0.1%-2.8%]）。主动脉瓣关闭不全在患有 BAV 的新生儿中 (n = 29 [14.7%]) 比没有 BAV 的新生儿 (1.3%) 更常见（绝对百分比差异，13.4% [95% CI, 7.8%-18.9%]；P < .001）。与未患有 BAV 的新生儿相比，患有 BAV 的新生儿跨瓣膜的流速较高（0.67 [95% CI, 0.65-0.69] m/s vs 0.61 [95% CI, 0.60-0.62] m/s；平均差为 0.06 m/s [95% CI, 0-0.1]），并且主动脉根部和管状升主动脉直径较大（10.7 [95% CI，10.7-10.9] mm vs 10.3 [95% CI，10.2-10.4] mm；平均差，0.43 mm [95% CI，0.2-0.6 mm] 和 9.8 [95% CI，9.6-10.0] mm vs 9.4 [95% CI，9.3-9.5] mm；平均差异，分别为 0.46 mm [95% CI，0.30-0.70]）（所有 P < .001）。 65 名 BAV 新生儿 (33.2%) 出现主动脉病变（62 名主动脉 z 评分 >= 3；3 名主动脉缩窄）。结论和相关性 在哥本哈根的新生儿中，BAV 的患病率为 0.77%。主动脉病变在患有 BAV 的新生儿中很常见，表明它也代表胎儿畸形。问题 新生儿中二叶式主动脉瓣的患病率是多少？主动脉相关疾病的程度如何？结果 这项横断面研究纳入了 2016 年至 2018 年间丹麦 25 556 名接受经胸超声心动图检查的新生儿。二叶式主动脉瓣患病率为0.77%；其中 33% 存在主动脉病。含义 在哥本哈根的新生儿中，二叶式主动脉瓣的患病率为 0.77%，相关主动脉疾病很常见，表明这是一种胎儿畸形。这项人群流行病学研究描述了 2016-2018 年在哥本哈根出生的丹麦新生儿中二叶式主动脉瓣 (BAV) 和 BAV 亚型以及伴随的主动脉直径扩大或缩窄的患病率。
Importance The prevalence and characteristics of bicuspid aortic valve (BAV) are mainly reported from selected cohorts. BAV is associated with aortopathy, but it is unclear if it represents a fetal developmental defect or is secondary to abnormal valve dynamics. Objective To determine the prevalence of BAV and BAV subtypes and to describe the associated aortopathy in a large, population-based cohort of newborns. Design, Setting, and Participants The Copenhagen Baby Heart Study was a cross-sectional, population-based study open to all newborns born in Copenhagen between April 1, 2016, and October 31, 2018. Newborns with BAV were matched 1:2 to newborns with a tricuspid aortic valve (non-BAV group) on sex, singleton/twin pregnancy, gestational age, weight, and age at time of examination. Exposures Transthoracic echocardiography within 60 days after birth. Main Outcomes and Measures Primary outcome was BAV prevalence and types, ie, number of raphes and spatial orientation of raphes or cusps (no raphes), according to the classification system of Sievers and Schmidtke (classified as type 0, 1, or 2, with numbers indicating the number of raphes). Secondary outcome was valve function and BAV-associated aortopathy, defined as aortic diameter z score of 3 or greater or coarctation. Results In total, 25 556 newborns (51.7% male; mean age, 12 [SD, 8] days) underwent echocardiography. BAV was diagnosed in 196 newborns (prevalence, 0.77% [95% CI, 0.67%-0.88%]), with male-female ratio 2.1:1. BAV was classified as type 0 in 17 newborns (8.7% [95% CI, 5.5%-13.5%]), type 1 in 178 (90.8% [95% CI, 86.0%-94.1%]) (147 [75.0% {95% CI, 68.5%-80.5%}] right-left coronary raphe, 27 [13.8% {95% CI, 9.6%-19.3%}] right coronary-noncoronary raphe, 4 [2.0% {95% CI, 0.8%-5.1%}] left coronary-noncoronary raphe), and type 2 in 1 (0.5% [95% CI, 0.1%-2.8%]). Aortic regurgitation was more prevalent in newborns with BAV (n = 29 [14.7%]) than in those without BAV (1.3%) (absolute % difference, 13.4% [95% CI, 7.8%-18.9%]; P < .001). Newborns with BAV had higher flow velocities across the valve (0.67 [95% CI, 0.65-0.69] m/s vs 0.61 [95% CI, 0.60-0.62] m/s; mean difference, 0.06 m/s [95% CI, 0-0.1]) and larger aortic root and tubular ascending aortic diameters than those without BAV (10.7 [95% CI, 10.7-10.9] mm vs 10.3 [95% CI, 10.2-10.4] mm; mean difference, 0.43 mm [95% CI, 0.2-0.6 mm] and 9.8 [95% CI, 9.6-10.0] mm vs 9.4 [95% CI, 9.3-9.5] mm; mean difference, 0.46 mm [95% CI, 0.30-0.70], respectively) (P < .001 for all). Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score >= 3; 3 with coarctation). Conclusions and Relevance Among newborns in Copenhagen, the prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.Question What is the prevalence of bicuspid aortic valve among newborns and what is the extent of associated disease of the aorta? Findings This cross-sectional study included 25 556 newborns in Denmark between 2016 and 2018 who underwent transthoracic echocardiography. The prevalence of bicuspid aortic valve was 0.77%; of these, aortopathy was present in 33%. Meaning Among newborns in Copenhagen, the prevalence of bicuspid aortic valve was 0.77% and associated aortic disease was common, suggesting that it was a fetal malformation.This population epidemiology study describes the prevalence of bicuspid aortic valve (BAV) and BAV subtypes, and of accompanying enlarged aortic diameter or coarctation, in Danish newborns born in Copenhagen 2016-2018.