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A 38-year-old man was evaluated for heart palpitations and chest pain diagnosed with atrial fibrillation, left ventricular ejection fraction 30%, and moderate aortic insufficiency. On echocardiographic control, evidence of aortic bicuspid valve and aortic coarctation on the isthmus with dilated epiaortic vessels. Computed tomography angiography confirmed the presence of aortic coarctation of the descending portion of the arch with supply of the thoracic aorta by collateral vessels originating from the brachiocephalic vessels. The patient underwent two surgical procedures, the first to correct the aortic coarctation in left posterior lateral thoracotomy, the second to repair the aortic valve with valvuloplasty. At the remote re-evaluation, the patient presented in good general and compensatory conditions, in stable sinus rhythm and with a left ventricular systolic function at the lower limits. Aortic coarctation accounts for about 8% of all congenital heart diseases, and bicuspid aortic valve is associated with congenital heart disease in 85% of cases. The peculiarity of the case is the type of vessel malformation considered as an aortic \"atresia\". The late presentation in a patient previously in substantial well-being is also unusual. Finally, the near normalization of cardiac function after surgery is important.

Background Abnormal blood flow patterns are known to contribute to the ascending aortic dilation in patients with bicuspid aortic valve (BAV). The present study elucidated the blood flow characteristics in the dilated ascending aorta before and after transcatheter aortic valve replacement (TAVR) using computational fluid dynamics (CFD) analysis. Methods We performed CFD analysis in three BAV patients with ascending aortic dilation (maximum diameter ≥ 45 mm) who underwent TAVR. The blood flow streamline was visualized to evaluate the pre- and post-operative flow velocity, severity of vortex and helix, and wall shear stress (WSS) in the ascending aorta. Results Before the procedure, all three patients showed abnormal blood flow patterns, with vortex and helix in the ascending aorta. Regionally elevated WSS was also observed in the lateral or posterior ascending aortic wall (16.7 Pa, 12.2 Pa, and 14.5 Pa in patient 1, 2, and 3, respectively). After the procedure, the blood flow patterns significantly improved, and the maximum WSS also decreased (4.2 Pa, 1.1 Pa, and 3.2 Pa in patient 1, 2, and 3, respectively). Conclusion Abnormal blood flow patterns and WSS appeared to improve after TAVR in BAV patients with ascending aortic dilation. The impact on the long-term aortic growth rate and the incidence of aortic dissection requires further studies. Trial Registration Changes of Ascending Aortic Diameter in Patients Undergoing Transcatheter Aortic Valve Replacement. ClinicalTrial.gov number NCT05739253. Trial registration date 20,230,212.

BackgroundThe comparative outcomes of transcatheter aortic valve replacement (TAVR) in bicuspid versus tricuspid aortic stenosis (AS) with severe calcification remain unclear. This study aimed to compare the safety and efficacy of TAVR in these patient groups.MethodsUsing data from the Chinese Cardiovascular Association Database—National Transcatheter Valve Therapeutics Registry, we analysed 870 propensity score matched pairs of patients with severe calcification (≥470 mm³) undergoing TAVR between April 2014 and August 2023. Primary outcome was all-cause mortality at 1 year.ResultsNo significant differences were observed in technical success (95.1% vs 94.7%), procedural complications or in-hospital outcomes. All-cause mortality at 1 year was similar between bicuspid and tricuspid AS (4.3% vs 5.3%, HR 0.87; log-rank p=0.62). The incidence of stroke (1.5% vs 1.4%), cardiovascular hospitalisation (1.4% vs 1.7%) and moderate-to-severe paravalvular leak (3.5% vs 2.5%) was similar during follow-up. The rate of new permanent pacemaker implantation was higher in bicuspid AS (8.4% vs 5.6%; p=0.03).ConclusionsTAVR was observed to be equally safe and effective in bicuspid and tricuspid AS with severe calcification, though bicuspid AS was associated with a higher rate of permanent pacemaker implantation.

Indications for transcatheter aortic valve implantation (TAVI) have expanded in many countries to include patients with aortic stenosis who are at low surgical risk, and a similar expansion to this cohort is anticipated elsewhere in the world, together with an increase in the proportion of patients with bicuspid aortic valve (BAV) morphology as the age of the patients being treated decreases. To date, patients with BAV have been excluded from major randomized trials of TAVI owing to anatomical considerations. As a consequence, BAV has been a relative contraindication to the use of TAVI in international guidelines. Although clinical experience and observational data are accumulating, BAV presents numerous anatomical challenges for successful TAVI, despite advances in device design. Furthermore, in those with BAV, substantial geographical variation exists in patient characteristics, clinical approach and procedural strategy. Therefore, in this Roadmap article, we summarize the existing evidence and provide consensus recommendations from an international group of experts on the application of TAVI in patients with BAV in advance of the anticipated growth in the use of this procedure in this challenging cohort of patients.In this Roadmap article, international experts summarize the evidence and provide consensus recommendations on the application of transcatheter aortic valve implantation in patients with bicuspid aortic valve anatomy in advance of the anticipated growth in the use of this procedure in this challenging cohort of patients.

In an era of rapidly expanding use of transcatheter aortic valve implantation (TAVI), the management of patients with bicuspid aortic valve (BAV) disease is far less well established than in those with trileaflet anatomy. Results of isolated surgical aortic valve replacement are excellent in suitable patients, and surgery also allows treatment of concomitant pathology of the aortic root and ascending aorta that is frequently encountered in this cohort. Conversely, TAVI provides an excellent alternative in older patients who may be unsuitable for surgery, although outcomes in BAV disease have only been reported in relatively small non-randomised series. Here, we discuss the pertinent literature on this topic and outline contemporary interventional treatment options in this challenging setting.

•Risk of maternal complications during pregnancy with bicuspid aortic valve is incompletely understood.•In 20 years of pregnancies in women with bicuspid aortic valve, no major adverse events occurred.•Most patients had a single transthoracic echocardiogram to assess the aortic valve and thoracic aorta.•Single transthoracic echocardiogram is likely sufficient if no high-risk features are identified.

Background Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for high-risk patients with symptomatic severe aortic valve stenosis (AS). Recently, its use has also been extended to low-risk patients, resulting in its increasing utilization in patients with bicuspid aortic valve (BAV). But as a serious post-TAVR complication, ischemic stroke was associated with a nearly 6‐fold increased 30‐day mortality. BAV presents unique challenges for post-TAVR antithrombotic therapy due to its distinct valvular anatomy. Case presentation We present a case of a 72-year-old female who presented with angina pectoris symptoms and was found to have severe BAV stenosis (Type 0). According to the patient’s age, obvious symptom and willingness herself, TAVR was successful performed with deployment of a 23 mm Venus-A Plus valve (Venus Medtech, Hangzhou, China). A post-procedure echocardiogram confirmed the appropriate placement of the bioprosthetic valve with minor paravalvular regurgitation. Six months after TAVR, this patient experienced multiple strokes, presenting a significant challenge for clinicians. Conclusions This case underscores the serious complications that can occur post-TAVR and highlights the need for improved strategies to prevent early strokes.

There has not been a consensus on the prothesis sizing strategy in type 0 bicuspid aortic stenosis (AS) patients undergoing transcatheter aortic valve replacement (TAVR). Modifications to standard annular sizing strategies might be required due to the distinct anatomical characteristics. We have devised a downsizing strategy for TAVR using a self-expanding valve specifically for patients with type 0 bicuspid AS. The primary aim of this study is to compare the safety and efficacy of downsizing strategy with the Standard Annulus Sizing Strategy in TAVR for patients with type 0 bicuspid AS. It is a prospective, multi-center, superiority, single-blinded, randomized controlled trial comparing the Down Sizing and Standard Annulus Sizing Strategy in patients with type 0 bicuspid aortic stenosis undergoing transcatheter aortic valve replacement. Eligible participants will include patients with severe type 0 bicuspid AS, as defined by criteria such as mean gradient across aortic valve ≥40 mmHg, peak aortic jet velocity ≥4.0 m/s, aortic valve area (AVA) ≤1.0 cm², or AVA index ≤0.6 cm2/m2. These patients will be randomly assigned, in a 1:1 ratio, to either the Down Sizing Strategy group or the Standard Sizing Strategy group. In the Down Sizing Strategy group, a valve one size smaller will be implanted if the “waist sign” manifests along with less than mild regurgitation during balloon pre-dilatation. The primary end point of the study is a composite of VARC-3 defined device success, absence of both permanent pacemaker implantation due to high-degree atrioventricular block and new-onset complete left bundle branch block. This study will compare the safety and efficacy of Down Sizing Strategy with the Standard Annulus Sizing Strategy and provide valuable insights into the optimal approach for sizing in TAVR patients with type 0 bicuspid AS. We hypothesize that the Down Sizing Strategy will demonstrate superiority when compared to the Standard Annulus Sizing Strategy. (Down Sizing Strategy (HANGZHOU Solution) vs Standard Sizing Strategy TAVR in Bicuspid Aortic Stenosis (Type 0) (TAILOR-TAVR), NCT05511792).

Bicuspid aortic valve (BAV) anatomy has routinely been considered an exclusion in the setting of transcatheter aortic valve implantation (TAVI) because of the large dimension of the aortic annulus having a more calcified, bulky, and irregular shape. The study aims to develop a patient-specific computational framework to virtually simulate TAVI in stenotic BAV patients using the Edwards SAPIEN 3 valve (S3) and its improved version SAPIEN 3 Ultra and quantify stent frame deformity as well as the severity of paravalvular leakage (PVL). Specifically, the aortic root anatomy of n.9 BAV patients who underwent TAVI was reconstructed from pre-operative CT imaging. Crimping and deployment of S3 frame were performed and then followed by fluid-solid interaction analysis to simulate valve leaflet dynamics throughout the entire cardiac cycle. Modeling revealed that the S3 stent frame expanded well on BAV anatomy with an elliptical shape at the aortic annulus. Comparison of predicted S3 deformity as assessed by eccentricity and expansion indices demonstrated a good agreement with the measurement obtained from CT imaging. Blood particle flow analysis demonstrated a backward blood jet during diastole, whereas the predicted PVL flows corresponded well with those determined by transesophageal echocardiography. This study represents a further step towards the use of personalized simulations to virtually plan TAVI, aiming at improving not only the efficacy of the implantation but also the exploration of “off-label” applications as the TAVI in the setting of BAV patients.

Limited data are available about the impact of permanent pacemaker (PPM) implantation on long-term survival in patients with a bicuspid aortic valve (BAV) and severe aortic stenosis (AS) treated with transcatheter aortic valve replacement (TAVR). We aimed to evaluate the long-term clinical outcomes of patients with BAV with AS who underwent periprocedural PPM implantation after TAVR with a self-expandable prosthesis. Data from patients with BAV and severe AS who underwent TAVR between April 2009 and January 2022 and followed in the framework of the One Hospital ClinicalService–CoreValve Project were collected. Patients were categorized in 2 groups according to PPM implantation after TAVR (“PPM” group) or not (“no PPM” group). The coprimary end points were all-cause death and a composite of cardiac mortality, rehospitalization because of cardiac causes, stroke, and myocardial infarction. Overall, 106 patients were considered (74 in the “no PPM” group and 32 in the “PPM” group). No statistically significant difference was found between the groups in terms of follow-up and baseline characteristics. Patients in the PPM group were more likely to show baseline conduction abnormalities (p = 0.023). Patients in the PPM group were more often treated with older generation prosthesis than those in the no PPM group (28.1% vs 5.4%, respectively, p = 0.013). At 2 years of follow-up, all-cause death in the no PPM and PPM groups occurred in 20.0% and 10.0% of patients, respectively (hazard ratio 0.37, 95% confidence interval 0.08 to 1.67). Similarly, no difference was evident for the composite end point between the 2 groups (no PPM vs PPM: 8 [14.6%] vs 6 [19.3%], hazard ratio 1.67, 95% CI 0.58 to 4.81). In conclusion, patients with severe AS and BAV treated with TAVR complicated by PPM implantation are not exposed to an increased risk of major adverse events at 2 years of follow-up.