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      Valve disease and aortopathy associations of bicuspid aortic valve phenotypes differ between men and women

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          Abstract

          Objective

          Determine whether associations between bicuspid aortic valve (BAV) phenotypes, valve disease and aortopathy differ between sexes.

          Methods

          1045 patients with BAV (76.0% men, n=794) from two surgical centres were included in this cross-sectional study. Valve phenotype was classified intraoperatively as right–left (RL), right-non-coronary (RN), left-non-coronary (LN) or 2-sinus BAV. Echocardiography was used to determine type and degree of valve disease, and aortic dimensions. Aortic dilatation was defined as diameter ≥4.5 cm.

          Results

          RL was the most common phenotype (73.6%), followed by RN (16.2%), 2-sinus BAV (9.2%) and LN (1.1%), with no difference in phenotype distribution between men and women (p=0.634). Aortic valve insufficiency (AI) prevalence differed significantly with valve phenotype in men (p=0.047), with RL and LN having the highest prevalence (34.1% and 44.0%, respectively). In women, RN had a higher proportion of AI than RL (21.3% vs 7.3%, p=0.017). Men with RL had larger root dimensions, in particular at the sinus (mean difference 0.24 cm compared with RN, p=0.002). Men with 2-sinus BAV had the highest prevalence of root phenotype dilatation (7.0%, other phenotypes ≤2.3%, p=0.031), whereas women with 2-sinus BAV did not have root dilatation and smaller sinus dimensions (mean difference: 0.35 cm compared with RL, p=0.021). Aortic root segments were larger in men with AI compared with aortic stenosis (sinus mean difference: 0.40 cm, p<0.001). The difference was even larger in women (mean difference: 0.78 cm, p<0.001), and women with AI also had larger tubular segments (mean difference: 0.61 cm, p=0.001).

          Conclusions

          There are significant sex differences in clinical associations of BAV phenotypes, which should be considered in further studies on the role of phenotypes in individualised patient management.

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          Most cited references26

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          2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease

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            A classification system for the bicuspid aortic valve from 304 surgical specimens.

            In general, classification of a disease has proven to be advantageous for disease management. This may also be valid for the bicuspid aortic valve, because the term "bicuspid aortic valve" stands for a common congenital aortic valve malformation with heterogeneous morphologic phenotypes and function resulting in different treatment strategies. We attempted to establish a classification system based on a 5-year data collection of surgical specimens. Between 1999 and 2003 a precise description of valve pathology was obtained from operative reports of 304 patients with a diseased bicuspid aortic valve. Several different characteristics of bicuspid aortic valves were tested to generate a pithy and easily applicable classification system. Three characteristics for a systematic classification were found appropriate: (1) number of raphes, (2) spatial position of cusps or raphes, and (3) functional status of the valve. The first characteristic was found to be the most significant and therefore termed "type." Three major types were identified: type 0 (no raphe), type 1 (one raphe), and type 2 (two raphes), followed by two supplementary characteristics, spatial position and function. These characteristics served to classify and codify the bicuspid aortic valves into three categories. Most frequently, a bicuspid aortic valve with one raphe was identified (type 1, n = 269). This raphe was positioned between the left (L) and right (R) coronary sinuses in 216 (type 1, L/R) with a hemodynamic predominant stenosis (S) in 119 (type 1, L/R, S). Only 21 patients had a "purely" bicuspid aortic valve with no raphe (type 0). A classification system for the bicuspid aortic valve with one major category ("type") and two supplementary categories is presented. This classification, even if used in the major category (type) alone, might be advantageous to better define bicuspid aortic valve disease, facilitate scientific communication, and improve treatment.
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              Incidence of aortic complications in patients with bicuspid aortic valves.

              Bicuspid aortic valve (BAV), the most common congenital heart defect, has been thought to cause frequent and severe aortic complications; however, long-term, population-based data are lacking. To determine the incidence of aortic complications in patients with BAV in a community cohort and in the general population. In this retrospective cohort study, we conducted comprehensive assessment of aortic complications of patients with BAV living in a population-based setting in Olmsted County, Minnesota. We analyzed long-term follow-up of a cohort of all Olmsted County residents diagnosed with definite BAV by echocardiography from 1980 to 1999 and searched for aortic complications of patients whose bicuspid valves had gone undiagnosed. The last year of follow-up was 2008-2009. Thoracic aortic dissection, ascending aortic aneurysm, and aortic surgery. The cohort included 416 consecutive patients with definite BAV diagnosed by echocardiography, mean (SD) follow-up of 16 (7) years (6530 patient-years). Aortic dissection occurred in 2 of 416 patients; incidence of 3.1 (95% CI, 0.5-9.5) cases per 10,000 patient-years, age-adjusted relative-risk 8.4 (95% CI, 2.1-33.5; P = .003) compared with the county's general population. Aortic dissection incidences for patients 50 years or older at baseline and bearers of aortic aneurysms at baseline were 17.4 (95% CI, 2.9-53.6) and 44.9 (95% CI, 7.5-138.5) cases per 10,000 patient-years, respectively. Comprehensive search for aortic dissections in undiagnosed bicuspid valves revealed 2 additional patients, allowing estimation of aortic dissection incidence in bicuspid valve patients irrespective of diagnosis status (1.5; 95% CI, 0.4-3.8 cases per 10,000 patient-years), which was similar to the diagnosed cohort. Of 384 patients without baseline aneurysms, 49 developed aneurysms at follow-up, incidence of 84.9 (95% CI, 63.3-110.9) cases per 10,000 patient-years and an age-adjusted relative risk 86.2 (95% CI, 65.1-114; P <.001 compared with the general population). The 25-year rate of aortic surgery was 25% (95% CI, 17.2%-32.8%). In the population of patients with BAV, the incidence of aortic dissection over a mean of 16 years of follow-up was low but significantly higher than in the general population.
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                Author and article information

                Journal
                Open Heart
                Open Heart
                openhrt
                openheart
                Open Heart
                BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                2053-3624
                2021
                20 October 2021
                : 8
                : 2
                : e001857
                Affiliations
                [1 ]departmentDepartment of Molecular Medicine and Surgery, Section of Cardiothoracic Surgery , Karolinska Institutet , Stockholm, Sweden
                [2 ]departmentDepartment of Cardiac and Thoracic Vascular Surgery , Universitätsklinikum Schleswig-Holstein Campus Lübeck , Lubeck, Germany
                [3 ]departmentDepartment of Thoracic and Cardiovascular Surgery , Karolinska University Hospital , Stockholm, Sweden
                [4 ]departmentDepartment of Paediatrics , LHSC Children's Hospital, University of Western Ontario , London, Ontario, Canada
                [5 ]departmentDepartment of Paediatrics, Division of Cardiology , The Hospital for Sick Children, University of Toronto , Toronto, Ontario, Canada
                [6 ]departmentDepartment of Medicine, Cardiovascular Medicine Unit, Center for Molecular Medicine , Karolinska Institutet, Karolinska University Hospital , Stockholm, Sweden
                Author notes
                [Correspondence to ] Dr Hanna M Björck; Hanna.Bjorck@ 123456ki.se
                Author information
                http://orcid.org/0000-0002-5383-1305
                http://orcid.org/0000-0002-5677-0747
                http://orcid.org/0000-0002-9155-3609
                Article
                openhrt-2021-001857
                10.1136/openhrt-2021-001857
                8529975
                34670833
                eef027ea-d194-4055-8e52-66745383fcde
                © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

                This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See:  http://creativecommons.org/licenses/by-nc/4.0/.

                History
                : 15 September 2021
                : 01 October 2021
                Funding
                Funded by: FundRef http://dx.doi.org/10.13039/501100004348, Stockholms Läns Landsting;
                Award ID: 20180072
                Funded by: FundRef http://dx.doi.org/10.13039/501100004359, Vetenskapsrådet;
                Award ID: 12660
                Funded by: FundRef http://dx.doi.org/10.13039/501100001674, Fondation Leducq;
                Award ID: MIBAVA, 12CVD03
                Funded by: FundRef http://dx.doi.org/10.13039/501100003793, Hjärt-Lungfonden;
                Award ID: 20180451
                Categories
                Aortic and Vascular Disease
                1506
                Original research
                Custom metadata
                unlocked

                aortic aneurysm,epidemiology,aortic valve insufficiency,aortic valve stenosis,heart defects,congenital

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