Aortic dilatation patterns and rates in adults with bicuspid aortic valves: a comparative study with Marfan syndrome and degenerative aortopathy
Aortic dilatation patterns and rates in adults with bicuspid aortic valves: a comparative study with Marfan syndrome and degenerative aortopathy
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DOI：
10.1136/heartjnl-2013-304920
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发表时间：
2014-01-15
期刊：
影响因子：
5.7
通讯作者：
Sarano, Maurice Enriquez
中科院分区：
文献类型：
--
作者：
Detaint, Delphine;Michelena, Hector I.;Sarano, Maurice Enriquez
背景二叶式主动脉瓣（BAV）与主动脉扩张相关，但不同病因的动脉瘤之间的模式/比率存在冲突，无法进行比较。我们试图确定BAV与其他血管病（马凡综合征（MFS），退行性血管病（DA））的升主动脉扩张模式/进展率。设计和设置回顾性观察性研究。在两个三级护理中心评价了主动脉扩张进展（美国和欧洲）通过重复超声心动图在BAV成人患者（n=353）中间隔≥ 2年，与MFS（n=50）和DA（n=51）的性别、血压和最短随访时间相匹配。管状升主动脉占60%（与BAV形态无关），主动脉窦扩张占27%（与典型BAV形态和男性性别独立相关（p=0.0001））。3.6 +/- 1.2年后，BAV中所有主动脉节段的主动脉扩张率均高于预期（p=0.005），BAV在管状升主动脉处最大MFS在主动脉窦最大（0.49 ± 0.5 mm/年）。BAV和MFS的最大主动脉扩张率相似（p>0.40），DA较低（p=0.02），但BAV不均匀，43%的BAV未进展（vs 20%的MFS，p=0.01）。主动脉扩张率与基线主动脉大小或BAV类型无关（所有模型p>0.40）。结论在BAV患者中，管状升主动脉扩张是最常见的模式，并且表现出最快的增长率，与瓣膜形态和功能无关。主动脉窦扩张不太常见，与典型的BAV形态和男性相关。BAV（管状段）和MFS（主动脉窦）的主动脉扩张进展同样快，但无论BAV类型如何，BAV患者中完全不进展的比例明显较高。基线主动脉直径不能成比例地预测进展率;因此需要对BAV患者进行系统随访。
Background Bicuspid aortic valve (BAV) is related to aortic dilatation, but patterns/rates are conflicting with no comparison among aneurysms of different aetiology. We sought to define ascending aorta dilatation patterns/progression rates in BAV versus other aortopathies (Marfan syndrome (MFS), degenerative aortopathy (DA)).Design and setting Retrospective, observational study. Aortic dilatation progression was evaluated in two tertiary care centres (US and European) by repeated echocardiography >= 2 years apart in adults with BAV (n=353), matched to MFS (n=50) and DA (n=51) for gender, blood pressure, and minimum follow-up time.Results At baseline, ascending aortic dilatation was present in 87% of BAV cases: tubular ascending aorta in 60% (irrespective of BAV morphology), and Valsalva sinuses dilatation in 27% (independently linked to typical BAV morphology and male gender (p=0.0001)). After 3.6 +/- 1.2 years, the aortic dilatation rate in BAV was higher than expected for the population for all aortic levels (p=0.005) and was maximal at the tubular ascending aorta for BAV (0.42 +/- 0.6 mm/year) and DA (0.20 +/- 0.3 mm/year), and was maximal at the Valsalva sinuses for MFS (0.49 +/- 0.5 mm/year). Maximal aortic dilatation rate was similar between BAV and MFS (p>0.40) and lower in DA (p=0.02) but was heterogeneous in BAV, with 43% of BAV not progressing (vs 20% of MFS, p=0.01). Aortic dilatation rate was not proportionally related to baseline aortic size or BAV type (all models p>0.40).Conclusions In patients with BAV, tubular ascending aorta dilatation is the most common pattern and exhibits the fastest growing rate, irrespective of valve morphology and function. Dilatation of the Valsalva sinuses is less common and associated with typical BAV morphology and male gender. Aortic dilatation progresses equally fast in BAV (tubular segment) and MFS (Valsalva sinuses), but a significantly higher proportion of BAV patients does not progress at all, irrespective of BAV type. Baseline aortic diameter does not proportionally predict progression rate; systematic follow-up is therefore warranted in patients with BAV.