Project description:BackgroundEdwards Intuity is recognized as a relatively contraindicated bioprosthesis for bicuspid aortic valve disease. This study compared the early echocardiographic and clinical outcomes of rapid-deployment aortic valve replacement for bicuspid versus tricuspid aortic valves.MethodsOf 278 patients who underwent rapid-deployment aortic valve replacement using Intuity at Seoul National University Hospital, 252 patients were enrolled after excluding those with pure aortic regurgitation, prosthetic valve failure, endocarditis, and quadricuspid valves. The bicuspid and tricuspid groups included 147 and 105 patients, respectively. Early outcomes and the incidence of paravalvular leak were compared between the groups. A subgroup analysis compared the outcomes for type 0 versus type 1 or 2 bicuspid valves.ResultsThe bicuspid group had more male and younger patients. Comorbidities, including diabetes mellitus, hypertension, chronic kidney disease, and coronary artery disease, were less prevalent in the bicuspid group. Early echocardiographic evaluations demonstrated that the incidence of ≥mild paravalvular leak did not differ significantly between the groups (5.5% vs. 1.0% in the bicuspid vs. tricuspid groups, p=0.09), and the early clinical outcomes were also comparable between the groups. In the subgroup analysis between type 0 and type 1 or 2 bicuspid valves, the incidence of mild or greater paravalvular leak (2.4% vs. 6.7% in type 0 vs. type 1 or 2, p=0.34) and clinical outcomes were comparable.ConclusionRapid-deployment aortic valve replacement for bicuspid aortic valves demonstrated comparable early echocardiographic and clinical outcomes to those for tricuspid aortic valves, and the outcomes were also satisfactory for type 0 bicuspid aortic valves.
Project description:BackgroundThere is little evidence on managing the proximal aorta of 4.0-4.5 cm during aortic valve replacement (AVR) in bicuspid aortic valve patients.MethodsA total of 431 patients between 1993 and 2019 underwent either an isolated AVR, AVR + concomitant ascending aorta replacement, or aortic root replacement. We divided patients into native root dilation (4.0-4.5 cm, n = 121) vs root control groups (<4.0 cm, n = 238), native ascending dilation (4.0-4.5 cm, n = 50) vs ascending control groups (<4.0 cm, n = 166), or proximal dilation (root or ascending aorta 4.0-4.5 cm, n = 160) and proximal control groups (both root and ascending aorta <4.0 cm, n = 272).ResultsGrowth rate was similar between the root dilation and control groups, (both were 0.1 mm/y, P = .56). The ascending dilation group had an aorta growth rate of 0.0 mm/y after AVR or root replacement, which was significantly different from the ascending control group (0.2 mm/y), P = .01. Furthermore, growth rate was similar between the proximal dilation (combined root or ascending dilation) and control group (both were 0.1 mm/y, P = .20). There were only 2 ascending aortic aneurysm repairs after AVR in the whole cohort. The long-term survival was similar between the root or ascending dilation groups vs root or ascending control groups, and between the proximal dilation and control groups. Multivariable Cox regression confirmed aortic root or ascending dilation was not a significant risk factor of long-term mortality.ConclusionsOur findings supported not replacing a 4.0-4.5 cm proximal thoracic aorta, including aortic root and ascending aorta, at the time of AVR for bicuspid aortic valve patients.
Project description:BackgroundBiological valve prostheses in rapid-deployment aortic valve replacement (RD AVR) procedures have demonstrated excellent outcomes. However, previous studies indicate a lack of specific surgical outcomes for the bicuspid aortic valve (BAV) or recommend RD AVR implementation. The existing literature comparing the clinical and hemodynamic outcomes of patients with BAV and those with tricuspid aortic valves (TAVs) after RD AVR is limited. Therefore, this study evaluated the safety and feasibility of RD AVR in BAV, including Sievers type 0, and compared the early clinical and hemodynamic outcomes of patients who underwent RD AVR at a single center based on aortic valve type.MethodsOur institution officially commenced performing RD AVR using Intuity valves in September 2022. The medical records of 80 patients who underwent aortic valve replacement (AVR) between September 1, 2022, and July 31, 2023 were retrospectively analyzed. In this set, 30 patients underwent RD AVR for aortic stenosis. Among the 30 RD AVR cases, groups A and B comprised 16 (53.3%) patients with TAV and 14 (46.7%) with BAV, respectively. The preoperative characteristics and postoperative echocardiographic data were compared between the two groups.ResultsNo statistically significant differences in preoperative characteristics, including mean age and sex distribution, were found between groups A and B. Notably, no patient in both groups exhibited mild- or higher-grade aortic regurgitation. The postoperative transvalvular mean pressure gradients showed significantly lower values in group B than in group A (12.20±4.64 vs. 16.26±5.49 mmHg, P=0.03). The necessity to insert a permanent pacemaker was not found in any of the patients (0%) in group A but was found in one (7.1%) patient in group B (P=0.46). Among the BAV cases, six (20%) were categorized as Sievers type 0. Of the 14 patients in group B, six with Sievers type 0 and the remaining eight with other bicuspid valve types were designated as groups B0 and B1, respectively. Similarly, no significant difference in postoperative transvalvular mean pressure gradient was observed between the two groups (11.33±4.49 vs. 12.86±4.94 mmHg, P=0.56). No in-hospital mortality was observed among all 30 patients.ConclusionsIn this study, RD AVR was considered feasible in a small, carefully selected cohort of patients with aortic stenosis, even in BAV, including Sievers type 0, as observed from the standpoint of postoperative hemodynamic outcomes and the incidence of aortic regurgitation.
Project description:BackgroundAlthough a bicuspid aortic valve (BAV) is known to be associated with progressive ascending aortic dilatation, the fate of the residual aorta after aortic valve and ascending aorta surgery is unknown. We reviewed surgical outcomes and explored serial changes in the size of the sinus of Valsalva (SOV) and distal ascending aorta (DAAo) in 89 patients with a BAV undergoing aortic valve replacement (AVR) and graft replacement (GR) of the ascending aorta.MethodsWe retrospectively examined patients who underwent AVR and GR of the ascending aorta for BAV-and related disease and thoracic aortic dilatation at our institution between January 2009 and December 2018. Patients who underwent AVR alone or required intervention for the aortic root and aortic arch and patients with connective tissue diseases were excluded. Aortic diameters were examined using computed tomography (CT). Late CT more than 1 year after surgery was performed in 69 patients (78%) with a mean follow-up of 4.9±2.8 years.ResultsThe surgical indication for aortic valve etiology was stenosis in 61 patients (69%), regurgitation in 10 (11%), and mixed in 18 (20%). Preoperative maximum short diameters of the ascending aorta, SOV, and DAAo were 47.3±4.7, 36.0±5.2, and 37.2±3.6 mm, respectively. The diameter of the SOV increased non-significantly by 0.08±0.45 mm per year [95% confidence interval (CI): -0.12 to 0.11, P=0.150], while that of the DAAo increased significantly by 0.11±0.40 mm per year (95% CI: 0.02-0.21, P=0.005). One patient required reoperation 6 years postoperatively due to a pseudo-aneurysm at the proximal anastomotic site. No patient required reoperation due to progressive dilatation of the residual aorta. According to the Kaplan-Meier analysis, the long-term survival rates were 98.9%, 98.9%, and 92.7% at 1, 5, and 10 years postoperatively, respectively.ConclusionsRapid dilatation of the residual aorta rarely occurred in patients with a BAV who underwent AVR and GR of the ascending aorta in the mid-term follow-up. For selected patients with a surgical indication for ascending aortic dilatation, simple AVR and GR of the ascending aorta may be sufficient surgical options.
Project description:BackgroundThere is a lack of available data on specific prognostic comparisons between transcatheter aortic valve replacement (TAVR) using self-expandable valves (SEV) in patients with stenotic Type 0, Type 1 bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV).ObjectivesTo evaluate the association between aortic valve morphology and outcomes following self-expandable TAVR.MethodsConsecutive patients with aortic stenosis(AS) undergoing self-expandable TAVR were enrolled and categorized into three groups (Type 0/Type 1 BAV or TAV) according to the Sievers classification. The primary endpoint was a composite of all-cause mortality and rehospitalization for heart failure (HF) within 2 years. Secondary outcomes included procedural complications and major cardiovascular events observed in clinical follow-ups. Clinical outcomes at 2 years following TAVR were compared among three groups using Kaplan-Meier curve and multivariable Cox proportional hazards regression models.ResultsA total of 344 AS patients (Type 0: 86; Type 1: 109; TAV: 149) were enrolled. The presence of moderate or severe paravalvular leak (PVL) was significantly higher in patients with Type 0 and Type 1 BAV versus TAV (10.47% vs. 16.51% vs. 6.71%, p = 0.043). All-cause 30-day mortality (2.33% vs. 0.92% vs. 2.68%, p = 0.626) and 2-year mortality (3.49% vs. 5.50% vs. 6.71%, p = 0.657) was comparable among the three groups. However, rehospitalization for HF within 2 years was significantly higher in Type 1 BAV (11.63% vs. 20.18% vs. 8.72%, p = 0.020). Multivariate Cox analysis showed that a higher STS score, Type 1 BAV morphology and excess leaflet calcification (≥ median calcium volume (CV) of the entire population) were independent predictors for HF rehospitalization. Additional intragroup Kaplan‒Meier analysis showed that excess leaflet calcification could predict higher long-term mortality and rehospitalization risk for HF(HR (95% CI): 3.430 (1.166-10.090), log rank p = 0.017) in Type 1 BAV patients.ConclusionOutcomes of self-expandable TAVR in BAV-AS patients might vary depending on valve subtypes. BAV patients with excess leaflet calcification and a raphe, especially calcified, had an increased risk of moderate PVL and HF readmission in mid-to-long term follow-ups.
Project description:BackgroundBicuspid aortic valve (BAV), the most common congenital heart defect affecting 1% to 2% of the population, is a major risk factor for premature aortic valve disease and accounts for the majority of valve replacement. The genetic basis and mechanisms of BAV etiology and pathogenesis remain largely undefined.MethodsCardiac structure and function was assessed in mice lacking a Gata6 allele. Human GATA6 gene variants were analyzed in 452 BAV cases from the BAV consortium and 1849 controls from the Framingham GWAS (Genome Wide Association Study). GATA6 expression was determined in mice and human tissues using quantitative real-time polymerase chain reaction and immunohistochemistry. Mechanistic studies were carried out in cultured cells.ResultsGata6 heterozygous mice have highly penetrant right-left (RL)-type BAV, the most frequent type in humans. GATA6 transcript levels are lower in human BAV compared with normal tricuspid valves. Mechanistically, Gata6 haploinsufficiency disrupts valve remodeling and extracellular matrix composition through dysregulation of important signaling molecules, including matrix metalloproteinase 9. Cell-specific inactivation of Gata6 reveals an essential role for GATA6 in secondary heart field myocytes because loss of 1 Gata6 allele from Isl- 1-positive cells-but not from endothelial or neural crest cells-recapitulates the phenotype of Gata6 heterozygous mice.ConclusionsThe data identify a new cellular and molecular mechanism underlying BAV. The availability of an animal model for the most frequent human BAV opens the way for the elucidation of BAV pathogenesis and the development of much needed therapies.
Project description:The purpose of this study was to evaluate whether bicuspid anatomy affects the discrepancy between CT-derived annular size and intraoperative size. We retrospectively analyzed annular measurements in 667 patients who underwent surgical aortic valve replacement (AVR). Preoperative CT measurements of the aortic annulus were compared to surgically implanted valve sizes. To evaluate whether the bicuspid valve affects the differences between CT annulus diameter and surgical AVR size, patients with diameter larger by > 10% (CT-Lg group) on CT, compared to surgical AVR size, were compared with those having size difference < 10% (CT-Sim group). Propensity score matching yielded 183 matched patients from each group. Bicuspid aortic valve annulus parameters significantly correlated with surgical aortic valve size (r = 0.52-0.71; for all, p < 0.01). The most representative measurements corresponded to surgical aortic valve size were area-derived diameters in tricuspid aortic valve (r = 0.69, p < 0.001) and bicuspid without raphe (r = 0.71, p < 0.001), and perimeter-derived diameter in bicuspid with raphe (r = 0.63, p < 0.001). After propensity score matching, native valve type was not different between CT-Sim and CT-Lg groups. In multivariable analysis, the difference between CT-derived diameter and surgical AVR size was affected by the operator factor and types of prosthesis. Bicuspid aortic annulus diameters measured on CT showed a significant correlation with surgical aortic valve size. The difference between CT-derived diameter and surgical AVR size is affected by operator factor and the types of prosthesis but not affected by the bicuspid valve.
Project description:PurposeThis study aimed to show the outcomes of isolated tricuspid valve replacement (ITVR) after left-sided valve surgery (LSVS).MethodsPatients who underwent ITVR after LSVS were divided into bioprosthetic tricuspid valve (BTV) and mechanical tricuspid valve (MTV) groups. Clinical data were collected and analyzed between groups.Results101 patients were divided into BTV (n = 46) and MTV (n = 55) groups. The mean ages of the BTV and MTV groups were 63.4 ± 8.9 and 52.4 ± 7.6 years, respectively (P <0.01). There was no significant difference in 30-day mortality (BTV 10.9% vs. MTV 5.5%), early postoperative complications, and long-term tricuspid valve (TV)-related adverse events between these two groups. New-onset renal insufficiency was an independent risk factor for early mortality. Survival rates in the BTV group were 94.8% ± 3.6%, 86.5% ± 6.5%, and 54.2% ± 17.6% and in the MTV group were 96.0% ± 2.8%, 79.0% ± 7.4%, and 59.4% ± 14.8% at 1, 5, and 10 years, respectively (P = 0.826).ConclusionThe TV prosthesis selection in ITVR after LSVS seems to not affect 30-day mortality and early postoperative complications. Long-term survival and the occurrence of TV-related events were also comparable between these two groups.
Project description:Bicuspid aortic valve (BAV), the most common congenital cardiac anomaly, predisposes individuals to aortic stenosis and regurgitation due to valve degeneration. Abnormal hemodynamics, arterial wall characteristics, and genetic factors contribute to ascending aorta dilatation, potentially leading to severe complications like aortic dissection. Presently, the most recent guidelines propose that individuals with BAV requiring valve replacement due to valve dysfunction should undergo simultaneous replacement of the ascending aorta when the diameter of aortic dilatation exceeds 4.5 cm. A 60-year-old female patient previously underwent mechanical aortic valve replacement and ascending aortoplasty at our center due to aortic stenosis and a 4.3 cm diameter ascending aorta. In the sixth postoperative year, she was readmitted due to ascending aorta rupture, resulting in blood entering the right atrium and causing acute right heart failure. We performed repair of the rupture and concomitant ascending aorta replacement to prevent further exacerbation of the patient's condition.