Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación / Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

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Abstract This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Analysis of related factors of new-onset conduction disturbance after transcatheter aortic valve replacement with self-expanding valve / 中国介入心脏病学杂志

Objective To investigate the baseline clinical characteristics,ascending aortic root anatomical characteristics,and related factors of the surgical strategy of patients with new-onset conduction disturbance(NOCD)after transcatheter aortic valve replacement(TAVR)with self-expanding valve(SEV)implantation.Methods A retrospective study was conducted on 245 patients who underwent TAVR at the Xiamen Cardiovascular Hospital Xiamen University between December 2014 and November 2022.According to the inclusion and exclusion criteria,167 patients with SEV implantation during surgery were continuously included.They were divided into tricuspid aortic valve group(TAV group,113 cases)and bicuspid aortic valve group(BAV group,54 cases)according to aortic valve morphology.The TAV group was divided into NOCD group(43 cases)and non NOCD group(70 cases)according to postoperative electrocardiogram characteristics.The BAV group was divided into NOCD group(16 cases)and non NOCD group(38 cases).Collect clinical data such as preoperative electrocardiogram and ascending aortic root CT angiography from patients.Results The right-non valvular calcification quantification(P=0.005)in the non-NOCD group was significantly greater than that in the NOCD group,but the aortic angle(P=0.002)was smaller in TAV patients.Multivariate analysis suggested that the risk of NOCD after TAVR is reduced by 2.6％for every 10 mm3 increase in right-non valvular calcification in patients(OR 0.974,P=0.039),the risk of postoperative NOCD nearly 7.3％for every degree increase in aortic angulation(OR 1.073,P=0.003).In BAV patients the increase of the risk of NOCD after TAVR is nearly 3.3％for every l ms increase in preoperative PR interval(OR 1.033,P=0.041),the risk of NOCD is reduced by 6.6％for every 10 mm3 increase in calcification quantification in the right coronary valve area(OR 0.934,P=0.013).Conclusions In TAV patient,right-non valvular calcification may have a protective effect on the cardiac conduction system,but a larger aortic angle increases the risk of NOCD.In BAV patients,a longer preoperative PR interval is a risk factor for NOCD,and the right coronary valve area may protect the cardiac conduction system.

Advances in cardiovascular diseases in children with Turner syndrome / 国际儿科学杂志

Turner syndrome（TS），a condition among females with a karyotype containing one X chromosome and complete or partial absence of the second sex chromosome，affects 20～50 in 100 000 newborn girls. Congenital or acquired cardiovascular diseases are present in about half of patients with TS. The congenital defects occur in about 25%～50% of TS patients；aortic dilatation as an important risk factor for aortic dissection is common in adult patients with TS. Furthermore，arrhythmias are found more frequently in patients with TS. A higher prevalence of hypertension in children and adolescents with TS has also been reported，and the application of 24h ambulatory blood pressure monitoring may facilitates the diagnosis of hypertension among these children and adolescent.Thus，active health education，correct follow-up and timely intervention are crucially needed.

Outcome analysis of functional mitral regurgitation after transcatheter aortic valve replacement in patients with severe bicuspid aortic stenosis / 复旦学报（医学版）

Objective To explore the relationship between the types of bicuspid aortic valves(BAV)and the outcome of functional mitral regurgitation(FMR)and the affecting factors of FMR.Methods From Jun 2018 to Sep 2022,patients with severe BAV aortic valve stenosis(AS)complicated with FMR underwent post transcatheter aortic valve replacement(TAVR)in Zhongshan Hospital,Fudan University were retrospectively analyzed.The baseline information and imaging data of different BAV patients were collected.Logistic regression was used to analyze the factors affecting the outcome of FMR(improvement and non-improvement).Result A total of 100 patients with TAVR were included,including 49 patients with type 0 of BAV and 51 patients with type 1 of BAV.Compared with patients of type 1,patients of type 0 had younger age[(72.78±6.09)y vs.(77.00±8.35)y,P=0.050],lower male ratio(47%vs.73%,P= 0.009)higher BMI[(23.19±2.62)kg/m2 vs.(21.99±3.13)kg/m2,P=0.041],and lower incidence of aortic regurgitation(69%vs.92%,P=0.040).Compared with the non-improvement group,the improvement group had a lower incidence of coronary heart disease(5%vs.18%,P=0.042),higher incidence of pulmonary hypertension(20%vs.2%,P=0.007),larger left ventricular diastolic diameter[(51.98±6.74)mm vs.(48.04±7.72)mm,P=0.009]and higher maximum flow velocity[(4.86±0.95)cm/s vs.(4.47±0.75)cm/s,P= 0.023]of the aortic valve.The results of Logistic regression analysis showed that preoperative pulmonary hypertension,left ventricular end-diastolic diameter and maximum valvular flow velocity of BAV patients were the potential affecting factors of FMR improvement after TAVR.Conclusion No significant difference was found in FMR improvement between BAV patients of type 0 and type 1 after TAVR.For BAV patients with AS,preoperative pulmonary hypertension,larger left ventricular end-diastolic diameter,and faster aortic valve flow velocity were associated with higher FMR improvement rate.

A Successful Aortic Valve-Sparing Root Replacement Operation (Reimplantation) to Repair Root Dilatation and Aortic Valve Regurgitation after a Ross Procedure / 日本心臓血管外科学会雑誌

We report a successful case of aortic valve-sparing root replacement for dilated aortic root after a Ross procedure. A 29-year-old male underwent a Ross procedure when he was 11 years old for congenital aortic bicuspid valve. The right ventricular outflow tract was reconstructed using an autologous pericardium as a single leaflet valve. Aortic root dilatation and moderate aortic valve regurgitation were noted. Further investigation with enhanced computed tomography and ultrasonic cardiography revealed good quality of leaflets and sufficient geometric height, and aortic valve-sparing root replacement was performed. In addition, we performed pulmonary valve replacement with a biological valve. The post-operative course was uneventful and the patient was discharged after 8 days with a completely controlled aortic valve regurgitation. No recurrence of aortic valve regurgitation was observed 1 year later. Because surgical outcomes of congenital heart diseases have improved and more patients have an increasing life expectancy, several other problems were revealed, such as the occurrence of aortic root dilatation after a Ross procedure. Aortic roots may dilate due to arterial pressure; however, valve-sparing procedures may be performed if the volume of the leaflets is sufficient.

Un tipo de válvula aórtica bicúspide poco frecuente / A rare type of bicuspid aortic valve

The bicuspid aortic valve corresponds to the most common valve defect in the general population. This can present asymptomatically as an incidental finding or symptomatically due to valve disease (stenosis and regurgitation), aortopathy, and infective endocarditis. Imaging techniques such as transthoracic and transesophageal echocardiography are fundamental pillars in diagnosis and treatment, determining the need for surgical intervention and the type of surgery to be performed. The case of a young patient with a less frequent bicuspid aortic valve phenotype is presented.

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.

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.

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 / One stage surgical treatment of aortic coarctation associated with bicuspid aortic valve. Report of one case

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.

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.

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.

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 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.

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.

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.