Research progress of application of transcatheter aortic valve replacement in the bicuspid aortic valve stenosis / 中国胸心血管外科临床杂志

@#Patients with bicuspid aortic valve (BAV) are characterized by asymmetric anatomy, severe calcification and combined aortic dilatation. Compared with tricuspid aortic valve stenosis patients, patients with BAV stenosis confront with greater surgical risks in transcatheter aortic valve replacement (TAVR), including paravalvular leak, aortic valve rupture, coronary artery obstruction, atrioventricular block and so on. However, with the advent of new generation of prosthetic valves and optimization of surgical strategies, several studies have shown that TAVR is safe and effective in the treatment of BAV stenosis. Therefore, we aim to provide an overview of the use of TAVR in patients with BAV stenosis.

Transapical transcatheter aortic valve replacement in bicuspid aortic valve patients: In-hospital outcomes / 中国胸心血管外科临床杂志

@#Objective    To compare the in-hospital outcomes of transapical transcatheter aortic valve replacement (TA-TAVR) for bicuspid aortic valve (BAV) patients and tricuspid aortic valve (TAV) patients. Methods    Patients (including BAV and TAV patients) who underwent TA-TAVR with the J-ValveTM in West China Hospital from July 2014 to July 2020 were included consecutively. The clinical outcomes of the patients were analyzed. Results    A total of 354 patients were included in the study, 75 in the BAV group and 279 in the TAV group. There were 229 males and 125 females with a mean age of 72.2±6.0 years. No death occurred during the procedure, and the overall technical success rate was 97.7%. The all-cause in-hospital mortality rate was 1.4%. Twenty (26.7%) patients with BAV and 46 (16.5%) patients with TAV had mild or higher perivalvular leaks immediately after the procedure. No patients with BAV required permanent pacemaker implantation postoperatively, while 13 (4.7%) TAV patients required permanent pacemaker implantation, with an overall pacemaker implantation rate of 3.7%. One (1.3%) BAV patient and 7 (2.5%) TAV patients developed acute kidney injury postoperatively. One (1.3%) BAV patient and 1 (0.4%) TAV patient developed peri-operative myocardial infarction. The average postoperative hospital stay was 7.6±3.6 d for BAV patients and 8.6±6.1 d for TAV patients. There was no statistical difference in primary or secondary in-hospital outcomes between BAV and TAV patients (P>0.05). Conclusion    Compared to TAV patients, BAV patients have similar in-hospital outcomes, with a low incidence of adverse clinical outcomes, which provides preliminary evidence for its implementation in Chinese patients with a high proportion of BAV.

Acute Aortic Dissection Masquerading a Rare Type of Congenital Bicuspid Aortic : A Case Report

Acute aortic dissection on bicuspid aortic valve (BAV) type 0 is a rare especially in young masculine. Risk factors like smoking and intensive sport activities can hasten their apparition especially in non-diagnosed population. Trans thoracic echocardiography is very important in the diagnosis of BAV whereas Angio CT scan helps to confirm acute aortic dissection. Treatment is based on surgery for type A Stanford classification for acute aortic dissection. We report a rare case of acute aortic dissection masquerading a rare type of BAV, type 0 in a young sportive male with no history of heart disease who presented for the first-time with acute chest pain after lifting a heavy weight metal at the complex sport center. Patient was initially placed on medical treatment before surgery for heart valve and root replacement (Bentall procedure).

Tratamiento en un tiempo quirúrgico de una coartación aortica asociada a valvulopatía aórtica bicúspide severa. Reporte de un caso

Percutaneous surgery is the treatment of choice of isolated aortic coarctation in adults However, when there are other heart problems related to aortic coarctation, its surgical management may vary. We report a 41-year-old male presenting with aortic coarctation associated with severe, symptomatic, bicuspid aortic valve lesions and significant left ventricular dysfunction. He underwent open heart surgery for the surgical resolution of these problems. One year after surgery the results are satisfactory with no evidence of postoperative complications and a significant improvement of patient symptoms and left ventricular function.

Transcatheter Aortic Valve Implantation in Bicuspid Aortic Valve with Aortic Stenosis: a Meta-Analysis and Trial Sequential Analysis

Abstract Objectives: Bicuspid aortic valve (BAV) is an important aetiology of aortic stenosis and the use of transcatheter aortic valve implantation (TAVI) has not been fully explored in this cohort. This systematic review and meta-analysis compared the outcomes of TAVI in stenotic BAV against tricuspid aortic valve (TAV). Methods: An electronic literature search was performed in PubMed, MEDLINE, EMBASE, and Scopus to identify all studies comparing TAVI in stenotic BAV versus TAV. Only studies comparing TAVI in BAV versus TAV were included, without any limit on the study date. Primary endpoints were 30-day and 1-year mortality, while secondary endpoints were postoperative rates of stroke, acute kidney injury (AKI), and permanent pacemaker (PPM) requirement. A trial sequential analysis (TSA) was performed for all endpoints to understand their significance. Results: Thirteen studies met the inclusion criteria (917 BAV and 3079 TAV patients). The BAV cohort was younger (76.8±7.43 years vs. 78.5±7.12 years, P=0.02), had a higher trans-aortic valve gradient (P=0.02), and larger ascending aortic diameters (P<0.0001). No significant difference was shown for primary (30-day mortality [P=0.45] and 1-year mortality [P=0.41]) and secondary endpoints (postoperative stroke [P=0.49], AKI [P=0.14], and PPM requirement [P=0.86]). The BAV group had a higher rate of significant postoperative aortic regurgitation (P=0.002). TSA showed that there was sufficient evidence to conclude the lack of difference in PPM requirements, and 30-day and 1-year mortality between the two cohorts. Conclusion: TAVI gives satisfactory outcomes for treating stenotic BAV and should be considered clinically.

Remodeling+Ring (modified Yacoub) technique for the treatment of bicuspid aortic valve regurgitation with ascending aortic aneurysm: A case report / 中国胸心血管外科临床杂志

@#Patients with bicuspid aortic valve are often complicated with aortic dilatation. If the aortic valve is of good quality, aortic root replacement with aortic valve preservation is feasible. A 35-year-old male patient with bicuspid aortic regurgitation complicated with ascending aortic aneurysm underwent Remodeling+Ring (modified Yacoub) operation. Echocardiography showed that there was no aortic regurgitation on the 3rd day after operation, and the patient was discharged satisfactorily on the 6th day after operation. Remodeling+Ring surgery ensures the physiological movement of the aortic valve, solves the enlarged annulus, avoids the problems caused by valve replacement, and significantly improves the quality of life of patients, which is worth popularizing.

A Case Report on Localized Acute Aortic Dissection of the Sinus of Valsalva Associated with a Bicuspid Aortic Valve Diagnosed with the Onset of Acute Coronary Syndrome / 日本心臓血管外科学会雑誌

We experienced a rare case of a patient who had a bicuspid aortic valve associated with acute aortic dissection limited to the sinus of Valsalva involving the left main coronary artery and acute coronary syndrome. The patient was a 36-year-old male who was identified as having a congenital bicuspid aortic valve. He visited our emergency room with a chief complaint of acute chest/back pain. He was diagnosed with acute coronary syndrome based on ECG findings and underwent an emergency coronary angiography. This test revealed filling defects at the entrance of the left main coronary artery, with aortic dissection limited to the sinus of Valsalva suspected. Emergency chest contrast-enhanced CT (ECG gated) led to his being diagnosed as having an acute aortic dissection limited to the sinus of Valsalva. After an intra-aortic balloon pump was inserted in order to maintain the coronary blood flow by surgery, emergency coronary artery bypass surgery and a modified Bentall procedure (the Carrel patch method) were carried out. His postoperative course was good and he was discharged home on the 19th disease day.

Establishment of large animal model of bicuspid aortic valve / 中国胸心血管外科临床杂志

@#Objective    To discuss the feasibility of establishment of animal model of "functional" bicuspid aortic valve with swine and observe its effect on the wall shear stress inside the aorta. Methods    Four common Shanghai White Swine with body weight between 50 kg to 55 kg were selected. Under general anesthesia and cardiopulmonary bypass, the aortic transverse incision approach was used, continuous suture with 6-0 polypropylene to align the left and right coronary valve leaflets to create a bicuspid valve morphology. After the operation, echocardiography was used to observe the aortic valve morphology and the hemodynamic changes of the aortic valve orifice. The effect on the wall shear stress inside the aorta was studied with 4D-Flow magnetic resonance imaging (MRI). Results    A total of 4 swine "functional" bicuspid aortic valve models were established, with a success rate of 100.0%. Echocardiography showed that the blood flow velocity of the aortic valve orifice was faster than that before the operation (0.96 m/s vs. 1.80 m/s). 4D-Flow MRI showed abnormally increased wall shear stress and blood flow velocity in the aorta of the animal models. After the surgery, in model animals, the maximal wall shear stress inside the ascending aorta was greater than 1.36 Pa, and the maximum blood flow velocity was greater than 1.4 m/s. Conclusion    Establishment of the animal model of "functional" bicuspid aortic valve in swine is feasible, scientific and reliable. It can be used in researches on evaluating the pathophysiological changes.

Short and mid-term outcomes of valve repair in patients with insufficient bicuspid aortic valves / 中国胸心血管外科临床杂志

@#Objective    To evaluate the short and mid-term outcomes of valve repair in patients with insufficient bicuspid aortic valves (BAV). Methods    The 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. Results    All 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). Conclusion    In patients with insufficient BAV, valve repair is safe and effective, and has excellent short and mid-term outcomes.

A Case of Giant Unruptured Left Coronary Sinus of Valsalva Aneurysm / 日本心臓血管外科学会雑誌

We herein report a rare case of unruptured, giant left coronary sinus of Valsalva aneurysm and discuss surgical pitfalls associated with sinus of Valsalva aneurysms. A 63-year-old man was referred to us for clinical diagnosis and surgical treatment of a huge mass in the mediastinum. Enhanced computed tomography (CT) imaging revealed that the mass was a left coronary sinus of Valsalva aneurysm with a diameter of 74×57 mm ; moreover, the left coronary artery originated from the aneurysmal wall. In addition, echocardiography showed moderate aortic regurgitation (AR) caused by dilatation of the aortic annulus. Based on these findings, the Bentall procedure was selected for the Valsalva aneurysm and significant AR. The orifice of the aneurysm was 15×15 mm in size, and the aortic wall of the left coronary sinus was relatively thin. The left main trunk was injured due to severe adhesion between the trunk and the aneurysm ; therefore, vein patch repair was performed with a saphenous vein graft. Since the aortic annulus of the left coronary cusp was fragile, proximal anastomosis of the composite graft to the lesion had to be placed in the fibrous continuity between the aortic and mitral valves. With respect to the proximal anastomosis at the aortic annulus of the left coronary cusp, the suture line was covered with a bovine pericardium patch as there were no remnants of the normal aortic wall. The postoperative course was uneventful, and postoperative CT revealed complete resection of the aneurysm with no evidence of stenosis of the left main trunk.

Comparison study of left ventricular reverse remodeling after transcatheter aortic valve replacement of bicuspid versus tricuspid aortic valve stenosis / 中华超声影像学杂志

Objective:To compare the left ventricular (LV) reverse remodeling after transcatheter aortic valve replacement (TAVR) between patients with bicuspid aortic valve (BAV) stenosis and tricuspid aortic valve (TAV) stenosis.Methods:The data of patients who underwent TAVR procedure from March 2013 to December 2018 in the Second Affiliated Hospital of Zhejiang University were retrospectively reviewed. The patients were divided into BAV group and TAV group according to cardiac computed tomography. Echocardiographic parameters, including aortic valve peak velocity (Vmax), mean gradient (PGmean), effective orifice area(EOA), interventricular septum diastolic thickness (IVSd), left ventricular posterior wall diastolic thickness (LVPWd), left ventricular end diastolic diameter( LVEDd), LV mass index (LVMI), ΔLVMI%, left ventricular ejection fraction( LVEF) of the two groups at baseline, 1 week, 1 month and 1 year post TAVR procedure were obtained and compared.Results:①Compared with preoperative measurements, both groups showed decreases in Vmax, PGmean and increase in EOA at 1 week, 1 month, 1 year follow-ups(all P<0.05). No significant differences were found in Vmax, PGmean, EOA, moderate/sever perivalvular leakage(PVL), moderate/sever prosthetic-patient mismatch(PPM) between BAV group and TAV group at 1 year. ②Both groups showed decreases in IVSd, LVPWd, LVEDd at 1 month, 1 year post TAVR compared with those before the procedure (all P<0.05), as well as increases in LVEF at 1 week, 1 month, 1 year (all P<0.05). Downward trends of LVMI were detected in both groups within 1 year follow-up( P<0.05). ③Compared to TAV group, BAV group showed smaller baseline LVMI( P<0.05), while there were no significant differences in ΔLVMI% post TAVR for all follow-up times of the two groups(all P>0.05). Repeated measures analysis of variance also showed no significant differences in downward trend of LVMI between the two groups after TAVR within 1 year( P>0.05). Conclusions:Left ventricular reverse remodeling can be detected in both BAV and TAV patients after TAVR, which starts from 1 week and can be lasted for 1 year post procedure. Patients with bicuspid morphology might experience similar reverse LV remodeling post TAVR versus patients with tricuspid morphology.

Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos / Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects

Resumen La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.

Abstract The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Cardiac disease and pregnancy: hyper vigilance and extreme caution for optimal outcome

Cardiac disease in pregnancy is a leading cause of maternal death in more so high-income countries. The armamentarium for winning this difficult battle involves shared decision-making with communication across the clinical team and the patient. There is limited clinical evidence concerning effective approaches to managing such complex care and moreover involvement of different specialists makes coordinated care challenging. Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1-2% of the population whereas a single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. We had two cases of pregnancy with bicuspid aortic valve in one case and the other with single ventricle. The involvement of multidisciplinary team involving cardiologist, cardiothoracic anaesthetist and fetal maternal medicine specialist resulted in good maternal and fetal outcome in both the cases.

A Case of Aortic Subannular Left Ventricular Aneurysm Appears to Be an Acute Aortic Root Dissection / 日本心臓血管外科学会雑誌

A 67-year-old man with dyspnea at rest was diagnosed with acute heart failure and admitted to our hospital. Echocardiogram showed severe AR, and CT implied an ascending aortic aneurysm and abnormal space in the aortic root. The patient underwent emergent surgery for suspected acute aortic dissection. Intraoperative findings showed the dehiscence of commissure of the aortic valve, and more, the abnormal space in the aortic root was not due to acute aortic dissection but an aortic subannular left ventricular aneurysm. The aneurysm was sutured and closed, and after that, aortic valve replacement and ascending aortic replacement were performed. Although subannular left ventricular aneurysm is a rare disease, it is important to carry out the preoperative evaluation considering the existence of such diseases.

A Case of Biventricular Repair with Damus-Kaye-Stansel (DKS) Take-Down after DKS Anastomosis for Interruption of the Aortic Arch and Ventricular Septal Defect with Subaortic Stenosis / 日本心臓血管外科学会雑誌

The patient in this case was a boy aged 2 years and 9 months. The patient was transferred to our hospital with ductal shock, and bilateral pulmonary artery banding was performed on the 9th day after the diagnosis of interruption of the aortic arch, ventricular septal defect, subaortic stenosis, and bicuspid aortic valve. Left ventricular outflow tract stenosis due to aortic annulus diameter and subaortic stenosis after repair was suspected. Damus-Kaye-Stansel (DKS) anastomosis, extended aortic arch anastomosis, and a right modified Blalock-Taussig operation were performed. Preoperative examination of the intracardiac repair showed growth of the aortic annulus and confirmed that biventricular repair was possible after DKS take-down. The patient's native aortic and pulmonary valves were preserved, and an intracardiac repair was performed without using an extracardiac conduit. The postoperative course was uneventful, and the patient is currently in a good condition at the age of 6 years. Three and a half years after surgical intervention, echocardiography and cardiac catheterization showed improvement of subaortic stenosis and enlargement of the aortic annulus. Our findings indicate that the most appropriate surgical procedure can be selected by detailed examination of the preoperative condition at each stage of the staged operation.

Echocardiographic diagnosis of bicuspid aortic valve combined with infective endocarditis / 中国医学影像技术

Objective: To explore the value of transthoracic echocardiography (TTE) and three-dimensional transesophageal echocardiography (3D-TEE) in diagnosis of bicuspid aortic valve (BAV) combined with infective endocarditis (IE). Methods: Ultrasonic data of 53 BAV combined with IE patients were retrospective reviewed. The ultrasonic findings of BAV combined with IE included vegetation, leaflet perforation, abscess and fistula. The ultrasonic features and diagnostic value of TTE and 3D-TEE were evaluated taken surgical findings as the gold standards. Results: The sensitivity and positive predictive value of 3D-TEE were both higher than those of TTE in detecting vegetation, leaflet perforation, abscess and fistula (100% vs.90.20%, 96.87% vs.90.62%, 92.86% vs.67.86%, 94.12% vs.70.59% and 100% vs.95.83%, 100% vs.93.55%, 96.30% vs.86.36%, 100% vs.85.71%, all P<0.05). Conclusion: The echocardiographic detection rate of BAV combined with IE is very high. 3D-TEE has better visibility and higher spatial resolution than TTE, therefore having higher sensitivity and positive predictive value in diagnosis of BAV combined with IE.

Midterm outcome comparison between patients with bicuspid or tricuspid aortic stenosis undergoing transcatheter aortic valve replacement / 中华心血管病杂志

Objective: To compare the prognosis of transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV) stenosis. Methods: This was a retrospective study. Patients with symptomatic severe aortic stenosis, who underwent TAVR with follow-up time more than one year in Guangdong Provincial People's Hospital from April 2016 to August 2018, were included. According to aortic CT angiography, the patients were divided into BAV group and TAV group. The primary endpoint was the composite event of all-cause death and stroke, and the secondary endpoints were TAVR-related complications. Incidence of clinical endpoints and parameters derived from echocardiography were compared between the groups, and Kaplan-Meier survival analysis was used to compare the composite event between the two groups. Results: A total of 49 patients were included. The age was (73.6±6.3) years, and 25(51.0%) were male. There were 32 patients in BAV group and 17 in TAV group, the follow-up time was 466 (390, 664) days. The incidence of composite endpoint of death and stroke at one year were comparable in BAV and TAV groups (6.3% (2/32) vs. 5.9% (1/17), P=1.00). Kaplan-Meier curves also showed a similar risk of the composite endpoint(HR=1.03，95%CI 0.09-11.24，Log-rank P=0.98) between two groups. The incidence of all-cause death, stroke, myocardial infarction, severe bleeding, major vascular complications, new-onset atrial fibrillation or atrial flutter, and permanent pacemaker implantation were all similar between the two groups(all P>0.05), and there was no acute kidney injury (stage 2 or 3) in both groups. Echocardiographic parameters at one year were similar between the two groups (all P>0.05). Conclusions: The midterm prognosis of TAVR in patients with BAV and TAV stenosis is similar. Clinical trials of large sample size with long-term follow-up are warranted to verify our findings.

Comparing clinical outcomes between bicuspid and tricuspid aortic valve stenosis after transcatheter aortic valve replacement: A systematic review and meta-analysis / 中国胸心血管外科临床杂志

@#Objective    To compare the clinical outcomes of bicuspid aortic valve stenosis (BAV) and tricuspid aortic valve stenosis (TAV) after transcatheter aortic valve replacement (TAVR). Methods    A computer-based search in PubMed, EMbase, The Cochrane Library, CBM, CNKI and Wanfang databases from their date of inception to March 2019 was conducted. Two reviewers independently screened the articles, extracted data and evaluated their quality. RevMan 5.3 and Stata 15.0 softwares were used for meta-analysis. Results    The selected 15 cohort studies contained 45 770 patients, including 1 500 of BAV patients and 44 270 of TAV patients. The results showed that no statistical difference was found in postoperative in-hospital mortality [OR=1.29, 95%CI (0.91, 1.83), P=0.15], 30-day mortality [OR=1.23, 95%CI (0.86, 1.77), P=0.25], and 1-year mortality [OR=1.14, 95%CI (0.87, 1.50), P=0.33] between the two groups. The surgical success rate [OR=0.57, 95%CI (0.44, 0.74), P<0.01], postoperative bleeding events [OR=0.72, 95%CI (0.58, 0.91), P<0.01] and postoperative vascular injury [OR=0.68, 95%CI (0.47, 0.97), P=0.03] of patients in the BAV group were lower than those in the TAV group. The incidence of paravalvular leak in the BAV group was higher than that in the TAV group [OR=1.47, 95%CI (1.13, 1.90), P<0.01]. No significant difference was found between the two groups in terms of conversion to surgery, postoperative myocardial infarction, postoperative renal failure, postoperative neurologic events, postoperative valve gradient difference and pacemaker implantation. Conclusion    Compared with TAV, the application of TAVR in BAV patients is feasible and effective.

Patrón de flujo en "4D Flow" y su vínculo con la aortopatía bicúspide / 4D Flow pattern and its role in bicuspid aortic valve aortopathy

ABSTRACT: Bicuspid aortic valve (BAV) disease is generally associated with thoracic aortic dilatation (TAD). Related factors include; genetical, morphological (valvular phenotype) and most recently, hemodynamic profiles associated with flow pattern and wall shear stress. Cardiac magnetic resonance 4D Flow (4DF) can give an integral evaluation of these later flow variables. Remarkable, different spectrums of flow and vortex direction exist in BAV that are related to the site of TAD (proximal or distal). Therefore, we present a 57 years old patient with BAV (Sievers 0) with anteroposterior leaflets distribution in which 4DF depicted an anteriorly and righthand oriented jet that correlated with the zone of grater AD; also, vortex rotation was counterclockwise, corresponding to the most frequent vortex type in BAV. In conclusion, 4DF is a powerful and ground-breaking tool that enhances our knowledge of BAV related aortopathy.