. . . . . . . . . . . . . . . . Bicuspid aortic valve and aortopathy: novel prognostic predictors for the identification of high-risk patients Luca Longobardo 1 , Scipione Carerj 1 , Alessandra Bitto 2 , Maurizio Cusma`-Piccione 1 , Maria Ludovica Carerj 3 , Maria Pia Calabro` 4 , Gianluca Di Bella 1 , Roberto Licordari 1 , Francesco Squadrito 2 , Bijoy K. Khandheria 5 *, and Concetta Zito 1 1 Department of Clinical and Experimental Medicine - Section of Cardiology, University of Messina, Azienda Ospedaliera Universitaria ‘Policlinico G. Martino’ and Universita` degli Studi di Messina, Via Consolare Valeria n.12, 98100 Messina, Italy; 2 Department of Clinical and Experimental Medicine - Section of Pharmacology, University of Messina, Azienda Ospedaliera Universitaria ‘Policlinico G. Martino’ and Universita` degli Studi di Messina, Via Consolare Valeria n.12, 98100 Messina, Italy; 3 Department of Biomedical Sciences and Morphological and Functional Imaging - Section of Radiological Sciences, Azienda Ospedaliera Universitaria ‘Policlinico G. Martino’ and Universita` degli Studi di Messina, Via Consolare Valeria n.12, 98100 Messina, Italy; 4 Department of Human Pathology of Adulthood and Childhood - Section of Pediatric Cardiology, Azienda Ospedaliera Universitaria ‘Policlinico G. Martino’ and Universita` degli Studi di Messina, Via Consolare Valeria n.12, 98100 Messina, Italy; and 5 Aurora Cardiovascular and Thoracic Services, Aurora Sinai/ Aurora St. Luke’s Medical Centers, University of Wisconsin School of Medicine and Public Health, Marcus Family Fund for Echocardiography (ECHO) Research and Education, 2801 W. Kinnickinnic River Parkway, #880, Milwaukee, WI 53215, USA Received 30 June 2020; editorial decision 10 August 2020; accepted 11 August 2020; online publish-ahead-of-print 7 October 2020 Aims Bicuspid aortic valve (BAV) may be complicated by aortic aneurysms and dissection. This study aimed to evaluate the prognostic efficacy of markers from cardiac imaging, as well as genetic and new biomarkers, to early predict aortic complications. ................................................................................................................................................................................................... Methods and results We re-evaluated after a mean time of 48 ± 11 months 47 BAV patients who had undergone previous echocardiog- raphy for evaluation of aortic stiffness and 2D aortic longitudinal strain (LS) (by speckle-tracking analysis), and who had given a blood sample for the assessment of a single-nucleotide polymorphism of elastin gene (ELN rs2 071307) and quantification of elastin soluble fragments (ESF). Surgical treatment of aortic aneurysm/dissection was the pri- mary endpoint, and an aortic dimension increase (of one or more aortic segments) >_1 mm/year was the secondary endpoint. Nine patients underwent surgical treatment of ascending aorta (AA) aneurysms. Out of the 38 patients who did not need surgical intervention, 16 showed an increase of aortic root and/or AA dimension >_1 mm/year. At multivariate Cox regression analysis, an impaired AA LS was an independent predictor of aortic surgery [P = 0.04; hazard ratio (HR) 0.961; 95% confidence interval (CI) 0.924–0.984] and aortic dilatation (P = 0.007; HR 0.960; 95% CI 0.932–0.989). An increased quantity of ESF was correlated (P = 0.015) with the primary endpoint at univariate Cox regression analysis but it did not keep statistical significance at multivariate analysis. ................................................................................................................................................................................................... Conclusion In BAV patients, impairment of elastic properties of the AA, as assessed by 2D LS, is an effective predictor of aortic complications.                                                                                                                                                                                                                     Keywords aortic aneurysm • aortic dilatation • longitudinal strain • elastin • bicuspid aortic valve • aortic stiffness Introduction Aortic aneurysm and dissection are the most dangerous complica- tions in patients with bicuspid aortic valve (BAV). Indeed, aortic dis- section is 5–10 times more common in patients with BAV than in those with trileaflet aortic valve, and BAV is reported in 9–28% of cases among young patients (<40 years old) with aortic dissection. 1 Currently, elective aortic surgery for asymptomatic BAV patients is indicated when the ascending aorta (AA) diameter is >55 mm or when it is >50mm in the presence of additional risk factors for *Corresponding author. Tel: þ1 (414) 649 3909; Fax: þ1 (414) 649 3578. E-mail: publishing22@aurora.org Published on behalf of the European Society of Cardiology. All rights reserved. VC The Author(s) 2020. For permissions, please email: journals.permissions@oup.com. European Heart Journal - Cardiovascular Imaging (2021) 22, 808–816 doi:10.1093/ehjci/jeaa250 Downloaded from https://academic.oup.com/ehjcimaging/article/22/7/808/5918834 by guest on 27 August 2022