In this issue of The Annals, Wang and colleagues [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] reported follow-up data of a large cohort of consecutively treated patients who underwent isolated aortic valve replacement (AVR) surgery for bicuspid aortic valve (BAV) disease. The authors should be congratulated for their efforts to determine the fate of proximal aortopathy in BAV patients stratified on the pattern of aortic valve dysfunction, namely, BAV–aortic stenosis versus BAV–aortic insufficiency (AI). Indeed, there is an emerging evidence of the coexistence of distinct clinical entities of BAV disease, namely, those presenting with predominant aortic valve stenosis (ie, BAV stenosis phenotype) and those presenting with predominant aortic insufficiency and dilation of the aortic root (ie, BAV root phenotype). Given the evident paucity of homogeneous phenotype-specific reporting of outcomes in BAV disease, the current report [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] is of great clinical value and should be a good example for future BAV studies. The major focus of this paper is on a cohort of BAV patients presenting with aortic valve insufficiency and aortic root involvement (ie, root phenotype). Wang and colleagues [1Wang Y. Wu B. Li J. Dong L. Wang C. Shu X. Impact of aortic insufficiency on ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement in patients with a bicuspid aortic valve.Ann Thorac Surg. 2016; 101: 1707-1715Abstract Full Text Full Text PDF Scopus (36) Google Scholar] found a significantly increased ascending aortic growth rate and higher prevalence of aortic events in BAV-AI patients, which is in line with our previous data [2Girdauskas E. Disha K. Rouman M. Espinoza A. Borger M.A. Kuntze T. Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: echocardiographic follow-up study.Eur J Cardiothorac Surg. 2015; 48: e71-e76Crossref PubMed Scopus (41) Google Scholar, 3Girdauskas E. Rouman M. Disha K. et al.Aortic dissection after previous aortic valve replacement for bicuspid aortic valve disease.J Am Coll Cardiol. 2015; 66: 1409-1411Crossref PubMed Scopus (47) Google Scholar] and therefore indicate the genetic origin of BAV-AI associated aortopathy. In retrospect, these data show convincingly that the historically reported increased risk of aortic events in BAV disease is mainly due to the mixing of such root phenotype patients in the analyzed heterogeneous BAV cohorts. The congenital nature of aortopathy in root phenotype patients is further supported by common phenotypic features (ie, young male BAV patients) and involvement of adjacent cardiac structures—marked enlargement of aortoventricular junction, involvement of anterior mitral leaflet (ie, WAMBIRE complex [4Sievers H.H. Invited commentary.Ann Thorac Surg. 2009; 87: 82Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar]), defects/aneurysms of membranous interventricular septum, and coexistent coronary anomalies. Similar to other well-known connective tissue disorders, several noncardiac manifestations (eg, skeletal deformities, recurrent groin hernia, and cerebral arterial aneurysms) have been previously reported in BAV insufficiency cohorts. Prospective trials aiming to clarify the underlying gene expression and biomarker patterns in BAV root phenotype patients are under way, and their results are awaited eagerly. The proof of congenital background of BAV-AI associated aortopathy will undoubtedly change our current understanding of BAV disease complex and make this distinct entity to become a firm part in the novel BAV nomenclature. Impact of Aortic Insufficiency on Ascending Aortic Dilatation and Adverse Aortic Events After Isolated Aortic Valve Replacement in Patients With a Bicuspid Aortic ValveThe Annals of Thoracic SurgeryVol. 101Issue 5PreviewAberrant flow pattern and congenital fragility bestows bicuspid aortic valve (BAV) with a propensity toward ascending aorta dilatation, aneurysm, and dissection. Whether isolated aortic valve replacement (AVR) can prevent further dilatation in BAV ascending aorta and what indicates concurrent aortic intervention in the case of valve operation remain controversial. Full-Text PDF
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