Coexistent bicuspid aortic valve and mitral valve prolapse: epidemiology, phenotypic spectrum, and clinical implications.

Bicuspid aortic valve (BAV) and mitral valve prolapse (MVP) are common but the prevalence and significance of coexistent conditions are unknown. This study investigated the prevalence, phenotypic expression, and clinical significance of coexistent MVP-BAV. Retrospective comparison of MVP-BAV and MVP-tricuspid aortic valve (TAV) prevalence including de novo echocardiographic analysis of all MVP-BAV patients between 2005 and 2015 was performed. The community prevalence of MVP-BAV was 2.7% vs. 3.4% for MVP-TAV (P = 0.45). Posterior mitral leaflet (PML)-MVP was the most common phenotype in both BAV and TAV (P = 0.38), but anterior mitral leaflet (AML)-MVP was twice more prevalent in BAV (31% vs. 15%, P < 0.0001). Among 130 subjects with coexistent MVP-BAV (81% men, 51 ± 16 years old), 31 (24%) exhibited AML:PML length ratio ≥3:1, termed large-AML prolapse (LAP-BAV), who had predominant BAV regurgitation when compared with those with non-LAP-BAV (P ≤ 0.001). An extreme phenotype of LAP-BAV with giant-AML prolapse and diminutive PML (GAP-BAV) was identified in 18/130 (14%) subjects. Compared with posterior-MVP-BAV, GAP-BAV patients were younger (42 ± 15 vs. 64 ± 12 years, P < 0.0001), had larger aortic annulus (28 ± 3 vs. 26 ± 2 mm, P = 0.01), and 61% had ≥ moderate BAV regurgitation (vs. 16%, P = 0.0007). Mitral repair occurred in 37/130 (28%) subjects. After median follow-up 5.5 months (4-83), 4/5 (80%) GAP-BAV patients required redo surgery for recurrent mitral regurgitation vs. 2/31 (6%) for non-LAP-BAV (P = 0.001). The community prevalence of coexistent MVP-BAV is comparable to MVP-TAV and their most common phenotype is posterior-MVP. However, anterior-MVP is twice as prevalent in MVP-BAV. A large-AML phenotype (LAP-BAV) with predominant BAV regurgitation affects 24% of MVP-BAV patients. An extreme phenotype of anterior-MVP (GAP-BAV) affects 14% of BAV patients; characterized by exceptionally large AML, diminutive PML, high mitral and aortic regurgitation prevalence, and high mitral repair failure rate.

Padang R ，Enriquez-Sarano M ，Pislaru SV ，Maalouf JF ，Nkomo VT ，Mankad SV ，Maltais S ，Suri RM ，Schaff HV ，Michelena HI ... -  《-》

Mitral regurgitation after previous aortic valve surgery for bicuspid aortic valve insufficiency.

Girdauskas E ，Disha K ，Espinoza A ，Misfeld M ，Reichenspurner H ，Borger MA ，Kuntze T ... -  《-》

New insights into bicuspid aortic valve disease: the elongated anterior mitral leaflet.

Charitos EI ，Hanke T ，Karluss A ，Hilker L ，Stierle U ，Sievers HH ... -  《-》

Sex Differences and Survival in Adults With Bicuspid Aortic Valves: Verification in 3 Contemporary Echocardiographic Cohorts.

Sex-related differences in morbidity and survival in bicuspid aortic valve (BAV) adults are fundamentally unknown. Contemporary studies portend excellent survival for BAV patients identified at early echocardiographic-clinical stages. Whether BAV adults incur a survival disadvantage throughout subsequent echocardiographic-clinical stages remains undetermined. Analysis was done of 3 different cohorts of consecutive patients with echocardiographic diagnosis of BAV identified retrospectively: (1) a community cohort of 416 patients with first BAV diagnosis (age 35±21 years, follow-up 16±7 years), (2) a tertiary clinical referral cohort of 2824 BAV adults (age 51±16 years, follow-up 9±6 years), and (3) a surgical referral cohort of 2242 BAV adults referred for aortic valve replacement (AVR) (age 62±14 years, follow-up 6±5 years). For the community cohort, 20-year risks of aortic regurgitation (AR), AVR, and infective endocarditis were higher in men (all P≤0.04); for a total BAV-related morbidity risk of 52±4% vs 35±6% in women (P=0.01). The cohort's 25-year survival was identical to that in the general population (P=0.98). AR independently predicted mortality in women (P=0.001). Baseline AR was more common in men (P≤0.02) in the tertiary cohort, with 20-year survival lower than that in the general population (P<0.0001); age-adjusted relative death risk was 1.16 (95% confidence interval [CI] 1.05-1.29) for men versus 1.67 (95% CI 1.38-2.03) for women (P=0.001). AR independently predicted mortality in women (P=0.01). Baseline AR and infective endocarditis were higher in men (both ≤0.001) for the surgical referral cohort, with 15-year survival lower than that in the general population (P<0.0001); age-adjusted relative death risk was 1.34 (95% CI 1.22-1.47) for men versus 1.63 (95% CI 1.40-1.89) for women (P=0.026). AR and NYHA class independently predicted mortality in women (both P≤0.04). Within evolving echocardiographic-clinical stages, the long-term survival of adults with BAV is not benign, as both men and women incur excess mortality. Although BAV-related morbidity is higher in men in the community, and AR and infective endocarditis are more prevalent in men, women exhibit a significantly higher relative risk of death in tertiary and surgical referral cohorts, which is independently associated with AR.

Michelena HI ，Suri RM ，Katan O ，Eleid MF ，Clavel MA ，Maurer MJ ，Pellikka PA ，Mahoney D ，Enriquez-Sarano M ... -  《Journal of the American Heart Association》

Comparative study of bicuspid vs. tricuspid aortic valve stenosis.

To examine differences and similarities in bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) stenosis occurring during the same age and whether any differences impact outcomes following aortic valve replacement (AVR). An age-matched cohort of 198 BAV stenosis and 198 TAV stenosis patients was identified from 888 consecutive patients undergoing AVR for severe AS. Mean age 68 ± 6 years; 68% male. Patients with BAV were less likely to have multiple comorbidities, as measured by the Charlson Comorbidity Index (CCI) >2 (3 vs. 10%, P = 0.007). Indexed aortic valve area (0.44 ± 0.09 vs. 0.42 ± 0.08 cm2/m2, P = 0.17) and left ventricular ejection fraction (LVEF) were similar (62 ± 11 vs. 61 ± 12%, P = 0.12), but E/e' ≥15 (46 vs. 66%, P = 0.0002) was less common in BAV stenosis. A tissue aortic valve prosthesis was more commonly utilized in both groups (81 vs. 78%, P = 0.54). Overall indexed effective orifice area was larger in BAV compared with TAV (1.08 ± 0.33 vs. 0.96 ± 0.25 cm2/m2, P = 0.0008). Five-year survival following AVR was lower in TAV compared with BAV stenosis (61 vs. 79%, P = 0.02). Independent predictors of survival following AVR were LVEF < 50% [hazard ratio (HR): 4.8, P = 0.0005], CCI > 2 (HR: 3.1, P = 0.015), effective orifice area index ≤0.85 cm2/m2 (HR: 2.5, P = 0.004), and bioprosthesis (HR: 3.7, P = 0.02). In an age-matched cohort, TAV compared with BAV stenosis is associated with greater prevalence of cardiovascular risk factors and cardiac impairment and worse survival after AVR.

Huntley GD ，Thaden JJ ，Alsidawi S ，Michelena HI ，Maleszewski JJ ，Edwards WD ，Scott CG ，Pislaru SV ，Pellikka PA ，Greason KL ，Ammash NM ，Malouf JF ，Enriquez-Sarano M ，Nkomo VT ... -  《-》