Coronary orifice rotation for symmetric bicuspidization of a paediatric unicuspid aortic valve.

Bicuspidization is a valid option for unicuspid aortic valve repair, in which creating symmetrical commissural orientation is essential for improved outcomes. However, the right coronary orifice often interferes with symmetrical attachment of the neocommissure. In a paediatric patient without aortic root dilation, we rotated the right coronary orifice clockwise by cutting out a triangular piece of the non-coronary sinus wall and augmenting it between the left and right coronary sinuses. A neocommissure with patching was sewn to the left side of the right coronary orifice, and the symmetrical bicuspidized configuration was adjusted in a standardized fashion according to the cusp effective height measurement.

Aortic root replacement for bicuspid aortic valve dysfunction does not impair survival rates.

BACKGROUND: Patients with a bicuspid aortic valve carry an increased risk for developing an ascending aortic aneurysm due to intrinsic aortic wall alterations. A lower threshold for aortic surgery may therefore be considered in these patients, especially in those who require aortic valve surgery. This study aimed to compare the outcomes of an isolated aortic valve replacement with that of an aortic root replacement in bicuspid aortic valve patients with an indication for aortic valve surgery. METHODS: Patients were included in retrospect from a tertiary academic hospital. Included patients received an elective aortic valve (AVR) or a composite valve-graft conduit (both mechanical and biological) between 2006 and 2021 without any concomitant procedure. Mortality data were retrieved from a national database and comparisons, including survival analyses, were performed between both groups. RESULTS: A total of 132 isolated AVR and 149 aortic root replacements were included. Patients who received an isolated AVR were significantly older than the aortic root replacement group (62.9 vs. 57.7 year respectively, p < 0.001). Survival analyses showed a comparable long-term mortality between both groups (8.1% vs. 9.1%, p = 0.321). CONCLUSION: This study shows that performing an aortic root replacement with a composite valve-graft conduit in bicuspid aortic valve patients does not impair the survival outcomes. In the light of preventing potential future aortic complications within this patient group with a congenitally and structurally weakened aortic wall, a more aggressive approach towards the treatment of BAV aortopathy might be considered.

A Case Report of Modified Cabrol Technique in a 60-Year-Old Male with Coronary Origin Anatomical Variant.

Introduction: Reconstruction surgery of the proximal aorta in most cases involves the use of an aortic conduit, followed by reimplantation of the coronary ostia. Although uncommon, the origin of the coronary arteries in certain anatomical variants poses additional difficulties when performing surgery on the aortic root and requires a different treatment rationale. Case report: We hereby present the case of a 60-year-old patient with multiple cardiovascular risk factors (smoking, arterial hypertension and dyslipidemia), suffering from severe degenerative stenosis of a bicuspid aortic valve, associated with ascending aorta aneurysm and a significant extrinsic stenosis of the left coronary artery caused by the aneurysm. The patient presented with severe degenerative bicuspid aortic valve stenosis associated with ascending aorta aneurysm and a significant extrinsic stenosis of the left coronary artery caused by the aneurysm. Following the preoperative assessment, it was decided that the best course of action was to perform surgery on the aortic valve and ascending aorta. During the surgery, the origin of the right and left coronary ostia were found at the level of the left coronary cusp, both forming a common coronary button. Due to this particular anatomical variant, it was decided to reimplant them as a common button onto the main conduit by means of an interposed No.10 PTFE (Polytetrafluoroethylene) vascular prosthesis. Conclusion: A rare case of aortic root surgery associated with coronary ostia origin variant "shotgun barrel", which required a different method of reimplantation: modified Cabrol technique.

Case report: membranoproliferative glomerulonephritis associated with Q fever causing chronic endocarditis.

BACKGROUND: Membranoproliferative glomerulonephritis is a rare entity which can be a result from autoimmune diseases, caused by various medications and infections. CASE PRESENTATION: We herein present the case of a 62-year-old male patient who presented with fatigue and was found to have severe anemia, impaired renal function, and nephrotic syndrome. A renal biopsy revealed membranoproliferative glomerulonephritis (MPGN) of the immune complex type with activation of the classical complement pathway. Further investigations led to the diagnosis of a chronic Coxiella burnetii-infection (Q fever), likely acquired during cycling trips in a region known for intensive sheep farming. Additionally, the patient was found to have a post endocarditic destructive bicuspid aortic valve caused by this pathogen. Treatment with hydroxychloroquine and doxycycline was administered for a duration of 24 months. The aortic valve was replaced successfully and the patient recovered completely. CONCLUSIONS: Early detection and targeted treatment of this life-threatening disease is crucial for complete recovery of the patient.

Successful TAVR for a Patient with Bicuspid Aortic Valve Stenosis and Previous Type A Aortic Dissection.

Whether transfemoral transcatheter aortic valve replacement (TAVR) can be accomplished in patients with a bicuspid aortic valve (AV) and previous type A aortic dissection remains rarely addressed. We report such a case in whom transfemoral TAVR was smoothly performed employing an extraordinary long sheath to bypass the dissected aortic segment, yet at the cost of perioperative left temporoparietal infarction owing to no suitable cerebral embolic protection device available in the presence of a remaining dissection in the aortic arch. Thus, for such patients, transfemoral TAVR is still feasible but novel embolic protection devices of distinct designs should be developed to avoid perioperative cerebral embolism.

Prognostic value of the electrocardiogram in patients with bicuspid aortic valve disease.

BACKGROUND: Identifying bicuspid aortic valve (BAV) patients at risk for cardiac events remains challenging and the role of the electrocardiogram (ECG) has not yet been described. Therefore, this study aims to describe ECG parameters in BAV patients, and investigate their prognostic value. METHODS: In this single-center prospective study patients with BAV without a prior aortic valve replacement (AVR) were included. Transthoracic echocardiogram and 12­lead resting-ECG were obtained. Associations between ECG parameters and the composite endpoint of all-cause mortality and AVR were assessed using Cox-proportional hazard analysis. RESULTS: 120 patients with BAV were included (median age 30 years, 61% male). Median aortic jet velocity was 2.4 m/s [IQR: 1.7-3.4] and 5 patients (4%) had severe aortic regurgitation. All patients were in sinus rhythm. Any ECG abnormality was present in 57 patients (48%). Median PR-interval was 156 [IQR: 138-170] msec. A deviating QRS axis was found in 17 patients (14%) and Cornell criteria for LVH were fulfilled in 20 patients (17%). Repolarization abnormalities were present in 12 patients (10%). Median follow-up duration was 7.0 [6.3-9.8] years, during which 23 patients underwent AVR and 2 patients died. After adjusting for age, a longer PR-interval was associated with worse intervention-free survival (HR 1.02, 95% CI: 1.01-1.04). CONCLUSION: Almost half of the patients with BAV had abnormalities on their ECG. Moreover, the PR-interval may be an interesting prognostic marker for intervention-free survival in BAV patients.

Management of bicuspid aortic valve disease in the transcatheter aortic valve implantation era.

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.

Ascending Aortic Progression After Isolated Aortic Valve Replacement Among Patients with Bicuspid and Tricuspid Aortic Valves.

OBJECTIVES: The aims of the present study were to compare the long-term outcomes for ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement between patients with bicuspid aortic valve (BAV) and tricuspid aortic valve ( TAV). METHODS: This retrospective study included 310 patients who had undergone isolated aortic valve replacement with an ascending aorta diameter ≤ 45 mm between January 2010 and September 2021. The patients were divided into BAV group (n=90) and TAV group (n=220). The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. RESULTS: Overall survival was 89 ± 4% in the BAV group vs. 75 ± 6% in the TAV group at 10 years postoperatively (P=0.007), yet this difference disappeared after adjusting exclusively for age (P=0.343). The mean annual growth rate of the ascending aorta was similar between the two groups during follow-up (0.5 ± 0.6 mm/year vs. 0.4 ± 0.5 mm/year; P=0.498). Ten-year freedom from adverse aortic events was 98.1% in the BAV group vs. 95.0% in the TAV group (P=0.636). Multivariable analysis revealed preoperative ascending aorta diameter to be a significant predictor of adverse aortic events (hazard ratio: 1.76; 95% confidence interval: 1.33 to 2.38; P<0.001). CONCLUSION: Our study revealed that the long-term survival and the risks of adverse aortic events between BAV and TAV patients were similar after isolated aortic valve replacement. BAV was not a risk factor of adverse aortic events.

Rationale and design of the SPREAD study: Sport Practice and its Effects on Aortic Size and Valve Function in Bicuspid Aortic Valve Disease.

The bicuspid aortic valve (BAV) is the most common congenital heart defect among adults, often leading to severe valve dysfunction and aortic complications. Despite its clinical significance, uncertainties persist regarding the impact of sports participation on the natural course of BAV disease. The SPREAD (Sport PRactice and its Effects on Bicuspid Aortic valve Disease) study is a multicenter and multinational project designed to investigate this relationship. This paper outlines the study's design, and objectives. The study is divided into two phases; phase one involves a cross-sectional analysis comparing aortic dimensions and valve function among competitive athletes with BAV, athletes with tricuspid aortic valves (TAV), and sedentary individuals with BAV. The second phase is a prospective, longitudinal follow-up aiming to evaluate the impact of regular sports training on disease progression. The SPREAD study seeks to provide evidence-based insights into the effects of sports participation on BAV disease progression, guiding clinical decision-making regarding sports eligibility and risk stratification for individuals with BAV.

A 25-Year-Old With an Incidentally Detected Ascending Thoracic Aortic Aneurysm and Bicuspid Aortic Valve.

A woman had shortness of breath and a first-degree relative with a history of ascending aorta dissection. Imaging showed an isolated dilatation in the mid ascending aorta and a bicuspid aortic valve but no pulmonary infiltrates, effusion, or embolism and no aortic stenosis or regurgitation. What would you do next?

Long-term outcomes in patients with bicuspid valve stenosis and aortic dilation undergoing transcatheter valve implantation.

BACKGROUND: To date, whether ascending aorta dilation (AAD) should be considered a contraindication for transcatheter aortic valve replacement (TAVR) remains a topic of debate.. OBJECTIVE: The study investigated the clinical outcome of TAVR in patients with bicuspid aortic valve stenosis (BAV-AS) complicated by AAD. METHODS: We included patients with BAV-AS who underwent TAVR between 2012 and 2019. We collected patient perioperative clinical data., tracked clinical outcomes for over four years post-TAVR, and obtained echocardiography images one year postoperatively. The Kaplan-Meier method was employed for analyzing both unadjusted and adjusted survival data, which was compared using the log-rank test. COX regression and nomograms were used to assess the impact of AAD on post-TAVR clinical outcomes in patients with aortic stenosis (AS), with all-cause mortality as the primary clinical endpoint. RESULTS: A total of 111 BAV patients were included in this study. Long-term follow-up showed an increased mortality risk in patients with BAV-AAD (adjusted Kaplan-Meier analysis: P = .02/0.001). Cox correlation analysis indicated that age (OR = 1.137; P = .034), AAD (OR = 3.51; P = .038), and postoperative left ventricular pressure (LVSP) (OR: 0.959; P = .044) were predictive factors for mortality more than four years after TAVR in patients with BAV. The area under the curve of the Nomogram predicting long-term survival for the training set of patients based on the above metrics was 0.845 (95% CI: 0.696-0.994). Short-term cardiac ultrasound follow-up showed a more rapid rate of AA expansion (0.29 [0-0.34] vs. -1 [-3.3-1] mm/month, P = .001) and a smaller proportion of AA diameter reduction (7.1% vs. 53.7%, P = .001) in patients who died. CONCLUSIONS: Patients with BAV-AAD-AS treated with TAVR have an increased risk of long-term mortality, and clinical prediction models, including AAD age and postoperative LVSP, may predict long-term patient survival. CONDENSED ABSTRACT: The study investigated the clinical outcome of transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve stenosis (BAV-AS) complicated by ascending aorta dilation (AAD). Patients with BAV-AAD-AS treated with TAVR have an increased risk of long-term mortality. AAD, age and postoperative LVSP, may predict long-term patient survival. Short-term cardiac ultrasound follow-up showed a more rapid rate of AA expansion and a smaller proportion of AA diameter reduction in patients who died. A high postoperative AAD expansion rate may indicate an adverse clinical outcome. Surgery regimens for tolerable BAV-AADs and can be considered as a treatment option.

Assessment of aortic dilatation in Chinese children and adolescents with Turner syndrome: a single center experience.

BACKGROUND: Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS. METHODS: This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method). RESULTS: A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m2, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score. CONCLUSIONS: The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.

Transcatheter aortic valve implantation in low-risk tricuspid or bicuspid aortic stenosis: the NOTION-2 trial.

BACKGROUND AND AIMS: Transcatheter aortic valve implantation (TAVI) has become the first choice to treat older patients with severe symptomatic aortic stenosis (AS). This study aimed to compare TAVI with surgery in low-risk patients ≤75 years of age, including both tricuspid and bicuspid AS. METHODS: The Nordic Aortic Valve Intervention (NOTION)-2 trial enrolled and 1:1 randomized low-risk patients aged ≤75 years with severe symptomatic AS to TAVI or surgery. The primary endpoint was a composite of all-cause mortality, stroke, or rehospitalization (related to the procedure, valve, or heart failure) at 12 months. RESULTS: A total of 370 patients were enrolled with a mean age of 71.1 years and a median Society of Thoracic Surgeons risk score of 1.1%. A total of 100 patients had bicuspid AS. The 1-year incidence of the primary endpoint was 10.2% in the TAVI group and 7.1% in the surgery group [absolute risk difference 3.1%; 95% confidence interval (CI), -2.7% to 8.8%; hazard ratio (HR) 1.4; 95% CI, 0.7-2.9; P = .3]. Patients with TAVI, when compared to surgery, had lower risk of major bleeding and new-onset atrial fibrillation and higher risk of non-disabling stroke, permanent pacemaker implantation, and moderate or greater paravalvular regurgitation. The risk of the primary composite endpoint was 8.7% and 8.3% in patients with tricuspid AS (HR 1.0; 95% CI, 0.5-2.3) and 14.3% and 3.9% in patients with bicuspid AS (HR 3.8; 95% CI, 0.8-18.5) treated with TAVI or surgery, respectively (P for interaction = .1). CONCLUSIONS: Among low-risk patients aged ≤75 years with severe symptomatic AS, the rate of the composite of death, stroke, or rehospitalization at 1 year was similar between TAVI and surgery. Transcatheter aortic valve implantation outcomes in young bicuspid AS patients warrant caution and should be further investigated. (NOTION-2, ClinicalTrials.gov, NCT02825134). TRIAL REGISTRATION NUMBER: ClinicalTrials.gov NCT02825134.

Sex differences in transcatheter aortic valve replacement outcomes among patients with bicuspid aortic stenosis.

BACKGROUND: Despite comprising almost half of all patients undergoing valvular repair, data on transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic stenosis (BAS) are limited. OBJECTIVE: We aimed to evaluate whether there are any sex differences in trends and outcomes of TAVR in this population. METHODS: We utilized the National Inpatient Sample from 2012 to 2020 to identify admissions with BAS who underwent TAVR and analyzed trends and outcomes. Our primary outcome was in-hospital mortality and secondary outcomes were in-hospital complications. We used two models to adjust for demographics (A) and interventions (B). RESULTS: Between 2012 to 2020, there were 76,540 hospitalizations for BAS patients who underwent AVR, among which 6,010 (7.9 %) underwent TAVR. There was an overall increasing trend in number of TAVR cases with a decreasing trend in mortality (2013: 8.7 %, 2020: 1.3 %). TAVR was performed more in males (61.1% vs 38.9 %). Despite the worse baseline characteristics in males, in-hospital mortality (2.4% vs. 1.5 %; OR: 1.584; 95 % CI: 0.621-4.038; p = 0.335) and secondary outcomes were similar across both sexes, even after adjusting for demographics and interventions. CONCLUSION: TAVR in BAS has grown rapidly in the last decade. Males comprised the majority and had more comorbidities, but mortality and complications were similar in both sexes. Despite the increasing number of cases, a decreasing trend in mortality was observed for both sexes ultimately approaching that of SAVR, suggesting that TAVR may be a safe alternative among eligible males and females with bicuspid AS.