A 78-year-old female patient was admitted to West China Hospital of Sichuan University because of “amaurosis and polypnea for 4+ years”. The examination results before admission showed that severe stenosis of aortic valve, bicuspid aortic valve malformation, severe horizontal heart with 61.54° aortic angle. The preoperative evaluation of the patient indicated that the Society of Thoracic Surgeons score was 17.9%, which was high risk for surgery. After discussion by the heart team, the aortic stenosis was finally relieved by transcatheter aortic valve replacement. The operation was successfully completed by using the method of pulling and releasing the device with the same side basket. One month follow-up showed that the patient’s condition was stable and no complications occurred. This case aims to provide a reference for the treatment of horizontal heart with aortic stenosis. In the future, it is hoped that through continuous clinical practice, such patients will be provided with a safer and more efficient treatment plan.
ObjectiveTo summarize the characteristics of bicuspid aortic valve (BAV) aortopathy and analyze the association between aortopathy and BAV phenotype and patterns of valvular dysfunction.MethodsClinical data of 191 patients who underwent the first aortic valve replacement in Fuwai Hospital from June 2017 to March 2018 were retrospectively analyzed, including 143 males and 48 females with an average age of 53.91±12.52 years. All patients underwent multidetector computed tomography (MDCT) and echocardiography before the operation, excluding patients with aortic coarctation. The BAV aortopathy phenotype was classified during operation. The characteristics of BAV aortopathy were analyzed by cluster and artificial analysis. BAV anatomic phenotype was divided into two types according to the direction of valve opening: BAV-AP and BAV-LR.ResultsFour distinct BAV aortopathy phenotypes were identified: a common type (n=70, 36.6%), with no dilation or mild dilation of aorta; a root type (n=24, 12.6%), with predominant dilatation of aortic sinus; an ascending aorta type (n=72, 37.7%), with predominant dilatation of ascending aorta; an arch type (n=25, 13.1%), with predominant dilatation of aortic arch dilatation. The root type was mainly in young patients, while the arch type was mainly in elderly patients (P<0.05). BAV-AP and aortic insufficiency were most prevalent in root type, while BAV-LR and aortic stenosis were most prevalent in arch type (P<0.05). There were 111 (58.1%) patients undergoing aortic surgery, and the coincidence rate of BAV aortopathy phenotype and aortic surgery was 80.6%.ConclusionAccording to the location of aortic dilation, BAV aortopathy can be divided into four types. There is an association between BAV aortopathy and valvular phenotype and dysfunction.
Transcatheter aortic valve replacement (TAVR) developed rapidly since firstly introduced to clinical practice in 2002. In 2015, Experts Consensus for Transeatheter Aortic Valve Replacement (abbreviated as the Consensus) helped TAVR develop normatively and safely in China. This article interpreted the Consensus in combination of new evolutions of TAVR field: first, the indications of TAVR expand from inoperative and high risk patients to the intermediate risk patients; second, although the Consensus recommended pre-dilation with balloon of modest size, the necessity of pre-dilation is under debate; third, the Consensus pointed out main complications of TAVR, and the main strategies to avoid complications are careful pre-procedural analysis and development of new device; fourth, our experts had made outstanding contribution to TAVR in the treatment of patients with bicuspid aortic valve, which still has many problems to be solved urgently.
ObjectiveTo find the relationship between bicuspid aortic valve (BAV) and the dilatation or aneurysm of the aorta using electrocardiogram-gated computed tomography angiography (CTA).
MethodsWe collected the clinical data of the BAV coexisting with suspected aortic dilatation or aneurysm from February 2012 through April 2015. A total of 124 patients were analyzed retrospectively. There were 97 males and 27 females at an anverage age of 50.35±16.26 years. According to the CTA, patients were classified into two groups: a pure BAV(without raphe) group and a BAV (with raphe) group. we recorded the aortic diameters, gender, age, and so on.
ResultsOf the 124 patients, 91 (73.4%) had BAV with raphe, and 33 patients (26.6%) had pure BAV. The analysis revealed that the diameter of the annulus (23.90±3.34 mm vs. 21.74±3.46 mm, P=0.005), the sinuses of Valsalva (40.93±6.78 mm vs. 37.35±7.06 mm, P=0.022), the tubular portion of the ascending aorta (45.38±7.66 mm vs. 38.29±8.18 mm, P=0.0001), and the part of the aorta proximal to the innominate artery (34.19±4.98 mm vs. 30.23±6.62 mm, P=0.02) between patients with BAV with raphe and pure BAV had significant differences. And there was a significant difference in prevalence of dilatation of the aorta between patients with pure BAV and BAV with raphe [77/91 (84.6%) vs.18/31(58.1%), P=0.004]. Of the 91 BAV with raphe patients, we found 76 patients (83.5%) with right and left coronary cusps (R-L) fusion, 13 patients (14.3%) with right and non-coronary cusps (R-N) fusion, and 2 patients (1.2%) with left and non-coronary cusps (L-N) fusion. There was a statistical difference in the aortic root diameters between R-L fusion BAV and R-N fusion BAV. The diameter of the distal ascending aorta and proximal aortic arch between R-L and R-N fusion BAV had statistical differences.
ConclusionsBAV with raphe is more common than pure BAV and is more often associated with dilatation and aneurysm of the ascending aorta. Otherwise R-L fusion BAV is associated with increased diameters of the aortic root, while R-N fusion BAV is associated with increased diameters of the distal ascending aorta and proximal arch.
Transcatheter aortic valve replacement (TAVR) is a mature technology. Because of the complicated valvular anatomy and the severe non-symmetrical valve calcification, the patient with bicuspid aortic valve stenosis (BAVs) once having a lower procedure successful rate, was considered as the relative contradiction for TAVR. However, with the application of the skirted and retrievable next generation prosthesis system, the outcomes of the treatment have been greatly improved. In this article, we summarized the current situation of TAVR applied in BAVs, and the outcomes difference of the old and new generation prosthesis systems.
ObjectiveTo assess clinical results of aortic cusps replacement with bovine pericardium for bicuspid aortic valve (BAV) and severe aortic regurgitation (AR).
MethodsClinical data of 79 patients with BAV and severe AR who underwent aortic cusps replacement with bovine pericardium in Wuhan Asia Heart Hospital from June 2008 to December 2013 were retrospectively analyzed. There were 60 male and 19 female patients with their age of 38±14 years (ranged 12-78 years). All the patients were in NYHA class Ⅱ. There were 26 patients with ascending aorta and sinotubular expanding.
ResultsNo early death or major complication was recorded. Intraoperative transesophageal echocardiography showed successful repair with normal coaptation of the aortic leaflets in all the patients. AR grade was less than mild in all the patients with peak aortic valve pressure gradients of 14.2±2.8 mm Hg. All the patients were discharged from the hospital within 15 postoperative days without any adverse symptom, and were followed up for 50±16 months (ranged 9-64 months). During follow-up, all the patients were in NYHA classⅠ. There were 57 patients without AR, 16 patients with mild AR, 5 patients with moderate AR and 1 patients with severe AR. The peak of aortic valve pressure gradient was 12.4±3.2 mm Hg. The average diameter of ascending aorta was 2.7 cm in the patients with ascending aorta and sinotubular expanding. The shape of sinotubular kept normal. The height of coaptation of aortic valve was 0.58 cm by echocadiography. None of the patients died or required reoperation. The structural valve degeneration was not observed during the follow-up.
ConclusionThree aortic cusps replacement with bovine pericardium can produce good hemodynamics and midterm results for patients with BAV and severe AR. The ascending aorta and sinotubular should be reduced and fixed in the patients with ascending aorta and sinotubular expanding.
Due to the lack of calcification of the aortic valve, it is difficult to provide effective support for the fixation of artificial valve in aortic stenosis characterized by leaflet thickening. There is a risk of postoperative perivalvular leakage and secondary valve implantation, which is widely regarded as a relative contraindication for transcatheter aortic valve replacement (TAVR). But aortic valvuloplasty and self-expanding transcatheter aortic valve has showed its safety and efficacy. This paper reports a patient with bicuspid aortic valve characterized by leaflet thickening treated with TAVR. After the operation, the patient’s symptom of exertional dyspnea was significantly improved, New York Heart Association class increased to Ⅱ, no perivalvular leakage was found, and the mean transaortic pressure gradient and the maximum aortic valve velocity decreased significantly.
Objective To explore the key genes, pathways and immune cell infiltration of bicuspid aortic valve (BAV) with ascending aortic dilation by bioinformatics analysis. Methods The data set GSE83675 was downloaded from the Gene Expression Omnibus database (up to May 12th, 2022). Differentially expressed genes (DEGs) were analyzed and gene set enrichment analysis (GSEA) was conducted using R language. STRING database and Cytoscape software were used to construct protein-protein interaction (PPI) network and identify hub genes. The proportion of immune cells infiltration was calculated by CIBERSORT deconvolution algorithm. Results There were 199 DEGs identified, including 19 up-regulated DEGs and 180 down-regulated DEGs. GSEA showed that the main enrichment pathways were cytokine-cytokine receptor interaction, pathways in cancer, regulation of actin cytoskeleton, chemokine signaling pathway and mitogen-activated protein kinase signaling pathway. Ten hub genes (EGFR, RIMS3, DLGAP2, RAPH1, CCNB3, CD3E, PIK3R5, TP73, PAK3, and AGAP2) were identified in PPI network. CIBERSORT analysis showed that activated natural killer cells were significantly higher in dilated aorta with BAV. Conclusions These identified key genes and pathways provide new insights into BAV aortopathy. Activated natural killer cells may participate in the dilation of ascending aorta with BAV.
ObjectiveTo evaluate the short and mid-term outcomes of valve repair in patients with insufficient bicuspid aortic valves (BAV).MethodsThe clinical data of 27 consecutive patients with insufficient BAV undergoing valve repair in Shanghai Chest Hospital from September 2016 to January 2020 were retrospectively reviewed. There were 24 males and 3 females with a mean age of 38.5±14.6 years (range: 20-68 years). BAV of all patients was type 1 in Seviers' classification. There were 23 patients with left-right fusion and 4 patients with right-noncoronary fusion. There was aortic regurgitation in the patients measured by the echocardiogram, including moderate regurgitation in 3 patients, moderate-severe in 18 patients, and severe in 6 patients. The diameter of aortic annular base was 27.9±3.4 mm, and the largest diameter of aortic sinus was 39.9±7.6 mm. Left ventricular end diastolic diameter was 62.7±6.5 mm, and the volume was 197.9±53.6 mL.ResultsAll 27 patients completed the follow-up, and the mean time was 24.2±12.5 months (range: 12-51 months). No patient died or required aortic valve-related reoperation during the follow-up. The cardiac function of the patients significantly improved postoperatively (P<0.05). By echocardiography, 11 patients had no aortic regurgitation, 13 had mild aortic regurgitation, and 3 had moderate aortic regurgitation, and no patient had severe aortic regurgitation. Postoperative left ventricular end diastolic diameter and volume decreased, compared to preoperative ones (P<0.05).ConclusionIn patients with insufficient BAV, valve repair is safe and effective, and has excellent short and mid-term outcomes.
Objective
To compare the efficacy and safety of transcatheter aortic valve replacement (TAVR) in aortic stenosis patients with bicuspid aortic valve (BAV) versus tricuspid aortic valve (TAV).
Methods
This retrospective study included 252 consecutive patients with severe and symptomatic aortic valve stenosis undergoing TAVR in West China Hospital form April 2012 to September 2017, in whom 4 patients were excluded because of valve failure after surgical aortic valve replacement.
Results
Of the 248 patients, 131 had BAV and 117 had TAV. Baseline characteristics were similar between the two groups, including age [(73.56±6.35) vs. (74.31±6.35) years, P=0.354], male proportion (51.9% vs. 63.2%, P=0.072). Society of Thoracic Surgeons score for BAV patients was lower than that for TAV patients [(7.56±4.01)% vs. (8.78±5.14)%, P=0.037]. Procedural success rate was high in both BAV and TAV groups (95.4% vs. 98.3%, P=0.359). There was no difference in 30-day all-cause mortality (6.1% vs. 2.6%, P=0.176) between the two groups; the aortic valve maximum velocity of valve hemodynamics after TAVR was similiar [(2.39±0.53) vs. (2.31±0.45) m/s, P=0.262] between the two groups.
Conclusions
Patients with BAV show similar procedural and clinical outcomes to patients with TAV. Therefore, TAVR appears to be a safe and effective procedure for patients with BAV as well as those with TAV.
