Department of Cardiac Surgery, Central Hospital Bad Berka, Bad Berka, Germany.
Department of Cardiac Surgery, Heart Center Leipzig, Leipzig, Germany.
Semin Thorac Cardiovasc Surg. 2013 Winter;25(4):310-6. doi: 10.1053/j.semtcvs.2014.01.004. Epub 2014 Jan 24.
Bicuspid aortic valve (BAV) is the most common form of congenital heart disease and most such patients develop cardiovascular complications over time. Recent studies have shed light on one of the most common of these complications, BAV-associated aortopathy. Two distinct BAV phenotypes have been identified, which may have different causes of their associated aortopathy. Increasing evidence suggests that the BAV stenosis phenotype is predominantly secondary to hemodynamic perturbances in transvalvular flow and is associated with a more benign long-term prognosis once the stenotic BAV is replaced. In contrast, the root phenotype--which is associated with aortic insufficiency--appears to have a genetic origin and may be associated with a higher risk of adverse aortic complications, irrespective of the extent of valvular disease. Such observations may have implications for patient decision making. Future studies should be performed so as to better define phenotypes and risk factors for BAV-associated aortopathy.
二叶式主动脉瓣（BAV）是最常见的先天性心脏病形式，大多数此类患者随着时间的推移会发展出心血管并发症。最近的研究揭示了其中最常见的并发症之一，即与 BAV 相关的主动脉病变。已经确定了两种不同的 BAV 表型，它们的相关主动脉病变可能有不同的原因。越来越多的证据表明，BAV 狭窄表型主要是由于跨瓣血流的血流动力学紊乱引起的，并且一旦狭窄的 BAV 被替换，其预后就较为良性。相比之下，根部表型（与主动脉瓣关闭不全相关）似乎具有遗传起源，并且无论瓣膜疾病的严重程度如何，都可能与更高的不良主动脉并发症风险相关。这些观察结果可能对患者的决策产生影响。未来应开展更多研究，以更好地确定与 BAV 相关的主动脉病变的表型和危险因素。
