与二叶式主动脉瓣相关的主动脉扩张：与性别、血流动力学及瓣膜形态的关系（美国国立心肺血液研究所资助的遗传性胸主动脉瘤和心血管疾病国家注册研究）
Aortic Dilatation Associated With Bicuspid Aortic Valve: Relation to Sex, Hemodynamics, and Valve Morphology (the National Heart Lung and Blood Institute-Sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions).
作者信息
Roman Mary J, Pugh Norma L, Devereux Richard B, Eagle Kim A, Holmes Kathryn, LeMaire Scott A, Milewski Rita K, Morris Shaine A, Prakash Siddharth K, Pyeritz Reed E, Ravekes William J, Shohet Ralph V, Song Howard K, Asch Federico M
机构信息
Division of Cardiology, Weill Cornell Medicine, New York, New York.
Biostatistics and Epidemiology Division, RTI International, Rockville, Maryland.
出版信息
Am J Cardiol. 2017 Oct 1;120(7):1171-1175. doi: 10.1016/j.amjcard.2017.06.061. Epub 2017 Jul 14.
This study analyzed the impact of sex, hemodynamic profile, and valve fusion pattern on aortopathy associated with bicuspid aortic valve (BAV). The National Heart Lung and Blood Institute-sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) provided comprehensive information on a large population of well-characterized patients with BAV. Of 969 enrolled patients with BAV, 551 (57%, 77% male) had already undergone valvular and/or aortic surgery. Echocardiographic imaging data were available on 339 unoperated or preoperative participants who formed the basis of this study. BAV function was normal in 45 (14%), with a predominant aortic regurgitation (AR) in 127 (41%) and a predominant aortic stenosis (AS) in 76 (22%). Moderate-severe AR was associated with larger sinus of Valsalva (SOV) diameters compared with normal function and AS (all p <0.01). Moderate-severe AS was associated with a larger ascending aortic (AscAo) diameter compared with normal function (p = 0.003) but not with AR. The SOV diameter was larger in men than in women (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001), whereas AscAo diameters were comparable (3.9 ± 0.9 vs 3.7 ± 0.9 cm, p = 0.08). Right-left commissural fusion was associated with a larger SOV diameter (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001) compared with a right-noncoronary fusion pattern. Predominant AR was more common in men (45% vs 27%, p = 0.004), whereas AS was more common in women (29% vs 18%, p = 0.04). In conclusion, in the GenTAC Registry, AR was associated with diffuse (annular, SOV, and AscAo) enlargement, whereas moderate-severe AS was only associated with AscAo enlargement. Male sex and right-left cusp pattern of cusp fusion were associated with larger SOV diameters and a greater likelihood of AR, whereas women had a higher prevalence of AS.
本研究分析了性别、血流动力学特征和瓣膜融合模式对与二叶式主动脉瓣（BAV）相关的主动脉病变的影响。美国国立心肺血液研究所资助的遗传性触发胸主动脉瘤和心血管疾病国家注册库（GenTAC）提供了大量特征明确的BAV患者的全面信息。在969例入选的BAV患者中，551例（57%，男性占77%）已经接受了瓣膜和/或主动脉手术。339例未手术或术前参与者有超声心动图成像数据，这些数据构成了本研究的基础。45例（14%）BAV功能正常，127例（41%）以主动脉瓣反流（AR）为主，76例（22%）以主动脉瓣狭窄（AS）为主。与功能正常和AS相比，中重度AR与较大的主动脉瓣窦（SOV）直径相关（所有p<0.01）。与功能正常相比，中重度AS与升主动脉（AscAo）直径较大相关（p=0.003），但与AR无关。男性的SOV直径大于女性（3.7±0.7 vs 3.3±0.6 cm，p<0.0001），而AscAo直径相当（3.9±0.9 vs 3.7±0.9 cm，p=0.08）。与右-无冠状动脉融合模式相比，左右连合融合与较大的SOV直径相关（3.7±0.7 vs 3.3±0.6 cm，p<0.0001）。以AR为主在男性中更常见（45%对27%，p=0.004），而AS在女性中更常见（29%对18%，p=0.04）。总之，在GenTAC注册库中，AR与弥漫性（瓣环、SOV和AscAo）扩大相关，而中重度AS仅与AscAo扩大相关。男性和左右瓣叶融合模式与较大的SOV直径及更高的AR可能性相关，而女性AS的患病率更高。
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