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》

Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry.

This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P<0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P<0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P<0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P<0.001) than women. Endocarditis (4.5% versus 2.5%, P=0.037) and aortic dissections (0.5% versus 0%, P<0.001) occurred more frequently in men. Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women.

Kong WK ，Regeer MV ，Ng AC ，McCormack L ，Poh KK ，Yeo TC ，Shanks M ，Parent S ，Enache R ，Popescu BA ，Yip JW ，Ma L ，Kamperidis V ，van der Velde ET ，Mertens B ，Ajmone Marsan N ，Delgado V ，Bax JJ ... -  《-》

Long-Term Recovery of Reduced Left Ventricular Ejection Fraction after Aortic Valve Replacement in Patients with Bicuspid Aortic Valve Disease.

Background Long-term prognosis of patients with bicuspid aortic valve (BAV) disease and poor left ventricular ejection fraction (LVEF) who underwent aortic valve replacement (AVR) is unknown. We aimed to analyze the recovery of LVEF and incidence of adverse events after AVR in patients with BAV and poor LVEF. Materials and Methods A total of 90 consecutive BAV patients (mean age 57 ± 10 years, 89% male) with baseline LVEF ≤40% underwent an isolated AVR between January 1, 1995, and June 30, 2008, and served as our study population. Follow-up data (800 patient-years) were obtained for all 90 hospital survivors. A subgroup of patients who underwent AVR for BAV stenosis (Group aortic stenosis [AS], n = 70) was compared with those who underwent AVR for BAV regurgitation (Group aortic regurgitation [AR], n = 20). Primary end point was the recovery of LVEF in AS Group versus AR Group. Secondary end points were survival and freedom from adverse cardiac events (i.e., cardiac-related death and need for reinterventions due to persisting heart failure). Results There was a significant increase in LVEF (mean follow-up 9.0 ± 5 years) in AS versus AR Group (i.e., 32 ± 7% [baseline] and 53 ± 9% [follow-up], p < 0.001 in AS Group vs. 33 ± 7% [baseline] and 38 ± 13% [follow-up], p = 0.07 in AR Group). Recovery rate of LVEF was significantly higher in AS Group versus AR Group (i.e., 2.8 percentage points (pp)/year vs. 0.7 pp/year, respectively). In Group AS, 86% of patients were responders, whereas in Group AR, only 30% (p < 0.001). The subjects in Group AR did not show a difference between baseline and follow-up left ventricular end-diastolic diameter (LVEDD) (baseline 61 ± 12 vs. follow-up 58 ± 8, p = 0.813), whereas in Group AS, there was a significant difference of LVEDD (baseline 56 ± 7 vs. follow-up 54 ± 6 mm, p = 0.019). Ten-year survival was 76 ± 6.5% in AS Group versus 78 ± 11% in AR Group (p = 0.3). Prevalence of late adverse cardiac events was 7% in AS Group versus 40% in AR Group (p = 0.03). Conclusion The recovery of reduced LVEF after AVR surgery is significantly impaired in patients with BAV regurgitation as compared with BAV stenosis.

Disha K ，Espinoza A ，Rouman M ，Secknus MA ，Kuntze T ，Girdauskas E ... -  《-》

Aortic events after isolated aortic valve replacement for bicuspid aortic valve root phenotype: echocardiographic follow-up study.

Various forms of bicuspid aortic valve (BAV) aortopathy exist, and the optimal treatment for the different subgroups is insufficiently defined. We aimed to analyse the risk of adverse aortic events after aortic valve replacement (AVR) for BAV insufficiency and concomitant mild-to-moderate dilatation of the aortic root (i.e. BAV root phenotype). A total of 56 consecutive patients (mean age 47 ± 11 years, 95% men) with BAV insufficiency and a root diameter of 40-50 mm underwent AVR surgery from 1995 to 2008. All patients, as identified from our institutional BAV database, had a dilated aortic annulus (i.e. defined as valve prosthesis size ≥27 mm) without aortic stenosis (i.e. mean gradient ≤20 mmHg). Patients who underwent concomitant aortic surgery were excluded. Follow-up (622 patient-years) including echocardiography data was available for all patients. Aortic events were defined as the need for proximal aortic surgery, the occurrence of aortic dissection/rupture, echocardiographic evidence of increasing aortic root diameter/occurrence of late paravalvular leakage or sudden death during follow-up. Actuarial survival was 90% at 10 years and 78% at 15 years. Adverse aortic events occurred in 19 (34%) study patients. Redo aortic surgery was performed in 6 patients (11%), 2 of which were for aortic dissection. Four patients (7%) suffered sudden cardiac death. Moreover, follow-up echocardiography revealed a significant, progressive enlargement of the aortic root diameter in 7 (13%) patients and occurrence of late de novo paravalvular leakage in 2 (3%) patients. The resultant freedom from aortic events was 81% at 10 years and 51% at 15 years. Patients with a BAV root phenotype are at significant risk of aortic events after isolated AVR. Simultaneous root/ascending aortic surgery should be strongly considered in such patients.

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

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 ... -  《-》