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The European Heart Journal article sheds new light on sex-related risk factors and frailty in cardiovascular disease. It discusses the challenges in diagnosing and treating type 2 myocardial infarction, as well as the outcomes of percutaneous vs. surgical revascularization in patients with non-ST-segment elevation myocardial infarction. Additionally, the article explores sex-related differences in aortic aneurysms and the association between pregnancy and aortic events, highlighting the need for personalized care and inclusion of frail patients in cardiovascular trials. The research findings aim to inform clinical practice and future research directions in cardiovascular medicine. [Extracted from the article]

Background and Aims To explore male–female differences in aneurysm growth and clinical outcomes in a two-centre retrospective Dutch cohort study of adult patients with ascending aortic aneurysm (AscAA). Methods Adult patients in whom imaging of an AscAA (root and/or ascending: ≥40 mm) was performed between 2007 and 2022 were included. Aneurysm growth was analysed using repeated measurements at the sinuses of Valsalva (SoV) and tubular ascending aorta. Male–female differences were explored in presentation, aneurysm characteristics, treatment strategy, survival, and clinical outcomes. Results One thousand eight hundred and fifty-eight patients were included (31.6% female). Median age at diagnosis was 65.4 years (interquartile range: 53.4–71.7) for females and 59.0 years (interquartile range: 49.3–68.0) for males (P <.001). At diagnosis, females more often had tubular ascending aortic involvement (75.5% vs. 70.2%; P =.030) while males more often had SoV involvement (42.8% vs. 21.6%; P <.001). Maximum absolute aortic diameter, at any location, at diagnosis did not differ between females (45.0 mm) and males (46.5 mm; P =.388). In females, tubular ascending growth was faster (P <.001), whereas in males, SoV growth was faster (P =.005), corrected for covariates. Unadjusted 10-year survival was 72.5% [95% confidence interval (CI) 67.8%–77.6%] for females and 78.3% (95% CI 75.3%–81.3%) for males (P =.010). Twenty-three type A dissections occurred, with an incidence rate of 8.2/1000 patient-years (95% CI 4.4–14.1) in females and 2.4/1000 patient-years (95% CI 1.2–4.5) in males [incidence rate ratio females/males: 3.4 (95% CI 1.5–8.0; P =.004)]. Conclusions In patients having entered a diagnostic programme, involvement of aortic segments and age- and segment-related growth patterns differ between women and men with AscAA, particularly at an older age. Unravelling of these intertwined observations will provide a deeper understanding of AscAA progression and outcome in women and men and can be used as an evidence base for patient-tailored clinical guideline development. [ABSTRACT FROM AUTHOR]

Background: Patients with pulmonary atresia and ventricular septal defect (PA/VSD) are prone to progressive aortic dilation. However, there are relatively few reports of progressive development of aortic aneurysm or aortic dissection in adult patients who missed early corrective surgery. Presentation of cases: Case 1: A 38-year-old man with PA/VSD and a bicuspid aortic valve (BAV), underwent VSD repair, aortic valve replacement, and PA correction at age 21. Seventeen years after surgery, an aortic dissection occurred in the ascending aorta, which subsequently underwent the Bentall procedure. Case 2: A 33-year-old male with PA/VSD, and a Nakata index of 31.24 mm2/m2, underwent a central shunt surgery at age 17. Sixteen years after surgery, an aortic root aneurysm and ascending aortic dilatation (AAD) developed. Case 3: A 42-year-old female underwent corrective surgery for PA/VSD repair at age 14. Twenty-eight years after surgery, an AAD developed. Conclusions: Adult patients with PA/VSD who miss the optimal age for surgery are more likely to develop dilatation of the ascending aorta and are at risk for aortic dissection. Therefore, long-term follow-up and monitoring is needed in this patient population. [ABSTRACT FROM AUTHOR]

A study on video-assisted thoracoscopic surgery (VATS) for pleural effusions found a high diagnostic yield and efficacy rate, confirming the safety and effectiveness of VATS in managing pleural effusions. Another study investigated the role of KCa3.1 in preventing saphenous vein graft stenosis following coronary artery bypass grafting. Additionally, research on adjuvant chemotherapy in octogenarians undergoing lung cancer resection and the development of a cardiopulmonary simulator for heart transplant training were highlighted. The introduction of endoscopic vein harvesting for coronary artery bypass grafting at the Bristol Heart Institute showed positive patient outcomes and satisfaction rates. Lastly, a study on postoperative stroke in cardiac surgery patients identified risk factors and mitigation strategies to reduce stroke incidence. [Extracted from the article]

The article discusses the long-term outcomes of aortic valve-sparing root surgery (VSRS) with concomitant aortic valve repair (AVRep) for aortic root aneurysms. The study included 378 patients who underwent VSRS, with a follow-up period ranging from 1 to 18.25 years. Results showed high long-term survival rates and low occurrence of major adverse cardiac and cerebrovascular events (MACCE) for patients undergoing VSRS. However, patients who required concomitant AVRep had a higher risk of aortic valve reintervention compared to those who underwent isolated VSRS. Close monitoring is recommended for patients with concomitant AVRep to detect recurrent aortic regurgitation early. [Extracted from the article]

Marfan syndrome (MFS) is a systemic connective tissue disorder stemming from mutations in the gene encoding Fibrillin-1 (Fbn1), a key extracellular matrix glycoprotein. This condition manifests with various clinical features, the most critical of which is the formation of aortic root aneurysms. Reduced nitric oxide (NO) production due to diminished endothelial nitric oxide synthase (eNOS) activity has been linked to MFS aortic aneurysm pathology. Caveolin-1 (Cav1), a structural protein of plasma membrane caveolae, is known to inhibit eNOS activity, suggesting its involvement in MFS aneurysm progression by modulating NO levels. In this study, we examined the role of Cav1 in aortic smooth muscle and endothelial function, aortic wall elasticity, and wall strength in male and female MFS mice (FBN1+/Cys1041Gly) by generating developing Cav1-deficient MFS mice (MFS/Cav1KO). Our findings reveal that Cav1 ablation leads to a pronounced reduction in aortic smooth muscle contraction in response to phenylephrine, attributable to an increase in NO production in the aortic wall. Furthermore, we observed enhanced aortic relaxation responses to acetylcholine in MFS/Cav1KO mice, further underscoring Cav1's inhibitory impact on NO synthesis within the aorta. Notably, van Gieson staining and chamber myography analyses showed improved elastin fiber structure and wall strength in male MFS/Cav1KO mice, whereas these effects were absent in female counterparts. Cav1's regulatory influence on aortic root aneurysm development in MFS through NO-mediated modulation of smooth muscle and endothelial function, with notable sex-dependent variations. [ABSTRACT FROM AUTHOR]

Background and Objectives: Fibrillinopathies have different phenotypic expression: Marfan-like skeleton features, MLSF; mitral valve prolapse syndrome, MVPS; MASS phenotype, M = mitral valve prolapse, MVP, A = aortic root dilation, S = skeletal features, and the second S = (cutaneous) striae; Marfan Syndrome, MS. The study had the following main objectives: the correlation between disc displacement, DD (a major sign for temporomandibular joint dysfunction, TMDs) on magnetic resonance imaging, MRI, and aortic Z score (a major sign for aortic root dilation) on echocardiography; the predictive value of DD towards aortic Z score. DD had 2 types of severity: DD with reduction (the mild type, DDwR), and DD without reduction (the severe type, DDwoR). Materials and Methods: The type of fibrillinopathy was precised by clinical exam (systemic score), ophthalmic exam (for ectopia lentis), and echocardiography (aortic Z score, MVP). Medical treatment consisted of betablockers, BB (for patients with tachycardia), or angiotensin II receptor blockers, ARB. Surgical treatment was addressed to aortic root aneurysm, and severe mitral regurgitation, MR. DD benefited from dental conservatory treatment or surgical restoration. Results: DD-Z score had a powerful correlation in MASS (Rho = 0.787, p < 0.01), and in MS patients (Rho = 0.819, p < 0.01). For the entire sample, both DDwR-Z score and DDwoR-Z score had a moderate correlation (Rho = 0.469, and respectively 0.669). Furthermore, DD was an important predictor for Z score. DDwoR had a double power of prediction for the Z score compared with DDwR (B coefficient = 1.661 for DDwR and 3.281 for DDwoR). Conclusions: TMDs had a powerful correlation with aortic root dilation in MASS and MS patients from the sample. Likewise, TMDs was a major predictor for aortic root dilation, in the entire sample. In clinical practice, we can utilize an extracardiac finding, TMDs, obtained by a non-invasive technique, MRI, for cardiac severity stratification of fibrillinopathies. [ABSTRACT FROM AUTHOR]

Background Membranous interventricular septum aneurysm (MISA) is a rare abnormality occurring in 0.3% of patients with congenital heart disease, which thereby increases anatomical complexity. Case summary Transcatheter aortic valve replacement (TAVR) procedure was planned for a 71-year-old female patient from East Asia with a type 1 bicuspid aortic valve diagnosed with severe aortic stenosis by transthoracic echocardiography (TTE). Pre-procedural multidetector computed tomography (MDCT) clearly revealed an extremely horizontal aorta and a MISA originating from the sub-annulus with the upper edge extending 7.2 mm above the annulus. A probable communicating flow between the left ventricle and the aorta was confirmed by reviewing the TTE images. Moreover, there was a calcified raphe between the left- and right-coronary cusps. A downsized balloon-expandable valve (a 23 mm Sapien 3 valve with an additional 2 mL dilation) was therefore chosen and deployed with a 100/0 aortic/ventricular ratio position. The TTE post-implantation indicated a trace perivalvular leakage. The cardiac MDCT performed post-procedure, at the 6-month, and 12-month follow-ups demonstrated complete sealing and significant healing of the aneurysm. Discussion Transcatheter aortic valve replacement utilizing a balloon-expandable valve was successfully performed for a case with membranous interventricular septum aneurysm extending above the annulus. Comprehensive imaging analysis before the procedure is crucial for TAVR with challenging anatomical conditions. [ABSTRACT FROM AUTHOR]

Background A sinus of Valsalva aneurysm involving a single cusp is a rare condition, and coronary computed tomography angiography with fractional flow reserve-computed tomography helps evaluate not only the anatomical aspects of the aneurysm and coronary artery but also the physiological details of coronary artery disease. Case summary A 71-year-old woman presented with exertional chest pain and dyspnoea. Enhanced computed tomography revealed an aneurysmal change in the right sinus of Valsalva, and coronary computed tomography angiography revealed diffuse narrowing of the proximal segment of right coronary artery due to mechanical stretching by the large Valsalva aneurysm. Fractional flow reserve-computed tomography revealed a significantly low fractional flow reserve (0.50 in the distal right coronary artery). A modified Bentall procedure was performed with a 21 mm bioprosthetic valve and a 24 mm Valsalva graft conduit for the aortic root aneurysm; mitral valve annuloplasty was performed for mitral valve regurgitation. Post-operative coronary computed tomography angiography revealed no significant stenosis in the proximal segment of the right coronary artery. Furthermore, fractional flow reserve-computed tomography revealed a normalized fractional flow reserve in the distal right coronary artery. The patient experienced relief from chest pain and was discharged 19 days after the surgery. Discussion A right coronary sinus of Valsalva aneurysm, which caused right coronary artery ischaemia, was successfully treated using a modified Bentall procedure. Coronary computed tomography angiography and fractional flow reserve-computed tomography revealed anatomical and functional improvements in the right coronary artery ischaemia post-operatively. [ABSTRACT FROM AUTHOR]

The article "Thoracic aortic disease: why sex matters" published in the European Heart Journal discusses the impact of sex-related differences on the natural course of ascending aortic aneurysms. The study by Notenboom et al. analyzed a cohort of 1858 patients with ascending or root diameter ≥40 mm, revealing sex-specific differences in disease location, growth rate, and aortic events during follow-up. Women showed higher ascending aortic diameter evolution and a higher rate of acute type A aortic dissection compared to men, highlighting the need for tailored follow-up strategies and conservative treatment approaches for women at higher risk. The study provides valuable insights into the anatomical disease pattern and aggressiveness of ascending aortic aneurysms in women, contributing to the advancement of personalized aortic care and precision medicine. [Extracted from the article]

OBJECTIVES Both aortic root remodelling and aortic valve (AV) reimplantation have been used for valve-sparing root replacement in patients with aortic root aneurysm with or without aortic regurgitation. There is no clear evidence to support one technique over the another. This study aimed to compare remodelling with basal ring annuloplasty versus reimplantation on a multicentre level with the use of propensity-score matching. METHODS This was a retrospective international multicentre study of patients undergoing remodelling or reimplantation between 2010 and 2021. Twenty-three preoperative covariates (including root dimensions and valve characteristics) were used for propensity-score matching. Perioperative outcomes were analysed along with longer-term freedom from AV reoperation/reintervention and other major valve-related events. RESULTS Throughout the study period, 297 patients underwent remodelling and 281 had reimplantation. Using propensity-score matching, 112 pairs were selected and further compared. We did not find a statistically significant difference in perioperative outcomes between the matched groups. Patients after remodelling had significantly higher reintervention risk than after reimplantation over the median follow-up of 6 years (P  = 0.016). The remodelling technique (P  = 0.02), need for decalcification (P  = 0.03) and degree of immediate postoperative AV regurgitation (P  < 0.001) were defined as independent risk factors for later AV reintervention. After exclusion of patients with worse than mild AV regurgitation immediately after repair, both techniques functioned comparably (P  = 0.089). CONCLUSIONS AV reimplantation was associated with better valve function in longer-term postoperatively than remodelling. If optimal immediate repair outcome was achieved, both techniques provided comparable AV function. [ABSTRACT FROM AUTHOR]

Aortic root pseudoaneurysm is a devastating complication post aortic valve replacement with a high mortality rate. And dissecting aneurysm into the interventricular septum is a rare variant of aortic root pseudoaneurysm, which is scarcely reported. Multimodal imaging is of great value in its diagnosis and differential diagnosis. [ABSTRACT FROM AUTHOR]

Background. Aortic root replacement with a composite mechanical valve graft (Bentall procedure) has been a recommended strategy in patients with aortic root aneurysm with or without aortic regurgitation. Aortic root remodeling (Yacoub procedure) has emerged as a valve‐sparing alternative although there is only scarce multicenter evidence. The aim of our study was to provide nationwide comparison of these two strategies. Methods. This was a retrospective study of data from national registry of cardiac surgery. Using propensity‐score matching, we compared all the patients undergoing the Bentall procedure in the Czech Republic between 2010 and 2021 with patients after the Yacoub procedure from four experienced centers. Results. During the study period, 199 patients underwent Yacoub and 526 had Bentall procedure. Of those, 166 pairs were selected and compared. There was no significant difference in perioperative outcomes and in mortality (p = 0.96) over the follow‐up of 5.7 vs. 6.4 years. The Bentall procedure was associated with a higher risk of major bleeding or thromboembolism (p < 0.001), and the Yacoub procedure led to a higher risk of rehospitalizations for valve failure (p = 0.01). Conclusions. In a nationwide propensity‐matched study, Bentall and Yacoub procedures yield similar longer‐term survival. Yacoub offers better freedom from thromboembolism or bleeding at the cost of higher risk of valve failure. [ABSTRACT FROM AUTHOR]

This article, published in the Journal of Cardiovascular Imaging, presents a case report of a 48-year-old woman who developed mucoid vasculopathy and multiple giant pseudoaneurysms following a Bentall procedure. The patient had previously undergone surgery for an ascending aortic aneurysm and severe aortic regurgitation. The article describes the diagnostic process, including echocardiogram and computed tomography (CT) scans, and the subsequent surgical repair of the pseudoaneurysms. The authors note that mucoid vasculopathy is a rare form of vasculopathy and that this case is the first reported instance of multiple huge pseudoaneurysms associated with this condition. [Extracted from the article]

Aortic root aneurysm is a potentially life‐threatening condition that may lead to aortic rupture and is often associated with genetic syndromes, such as Marfan syndrome (MFS). Although studies with MFS animal models have provided valuable insights into the pathogenesis of aortic root aneurysms, this understanding of the transcriptomic and epigenomic landscape in human aortic root tissue remains incomplete. This knowledge gap has impeded the development of effective targeted therapies. Here, this study performs the first integrative analysis of single‐nucleus multiomic (gene expression and chromatin accessibility) and spatial transcriptomic sequencing data of human aortic root tissue under healthy and MFS conditions. Cell‐type‐specific transcriptomic and cis‐regulatory profiles in the human aortic root are identified. Regulatory and spatial dynamics during phenotypic modulation of vascular smooth muscle cells (VSMCs), the cardinal cell type, are delineated. Moreover, candidate key regulators driving the phenotypic modulation of VSMC, such as FOXN3, TEAD1, BACH2, and BACH1, are identified. In vitro experiments demonstrate that FOXN3 functions as a novel key regulator for maintaining the contractile phenotype of human aortic VSMCs through targeting ACTA2. These findings provide novel insights into the regulatory and spatial dynamics during phenotypic modulation in the aneurysmal aortic root of humans. [ABSTRACT FROM AUTHOR]

This article presents a case study of a patient who has both multiple self-healing squamous epithelioma (MSSE) and features of Loeys-Dietz syndrome (LDS). MSSE is a rare condition characterized by the development of keratinizing tumors that regress on their own, while LDS is a connective tissue disorder that affects the aortic root. The patient in the study had recurrent keratoacanthomas and squamous cell carcinomas, and genetic testing revealed a pathogenic variant in the TGFBR1 gene. The article highlights the need for further research on the relationship between genotype and phenotype in disorders caused by TGFBR1 pathogenic variants, and emphasizes the importance of dermatologists being aware of potential aortic root dilatation in patients with MSSE. [Extracted from the article]

Marfan syndrome is a connective tissue disorder that affects multiple organs, with the most serious complications occurring in the cardiovascular system. Aortic root dilatation and dissection are particularly concerning. While the prevalence of Marfan syndrome is equal between genders, male patients have a higher risk of developing thoracic aortic aneurysm and dissection compared to females. A recent study using a mouse model of severe Marfan syndrome found that male mice had poorer survival rates and exhibited lower elastin content and more fragmented elastic fibers compared to female mice. The study suggests that material properties, aortic size, and wall integrity may be connected to dissection risk. Further research is needed to understand how age and sex contribute to dissection risk in Marfan syndrome. [Extracted from the article]

Coronary artery fistulas draining into the left ventricle is a rare finding. They can be associated with other congenital cardiac anomalies like ventricular septal defect or tetralogy of Fallot. While most of them are asymptomatic, they can lead to severe cardiac complications like infective endocarditis, heart failure, or myocardial ischemia. Symptomatic coronary artery fistulas can be managed surgically or percutaneously. We present a case of a 61-year-old male patient with both left anterior descending artery and right coronary artery fistulas draining into the left ventricle associated with ascending aorta and root aneurysm. Preoperative assessment for myocardial ischemia and the size and location of the fistulas was performed. The echocardiography stress test was negative. Surgery consisted of replacement of the ascending aorta and reconstruction of the noncoronary sinus with a Dacron patch with aortic valve preservation and no intervention for the coronary artery fistulas. The surgical strategy was adapted for cardioplegia administration to compensate for the volume of coronary blood drained into the left ventricle and for better protection of the distal myocardium. [ABSTRACT FROM AUTHOR]

Recently, an autosomal recessive subtype of connective tissue disorder within the spectrum of Ehlers–Danlos syndrome (EDS), named classical-like EDS type 2 (clEDS2), was identified. clEDS2 is associated with biallelic variants in the adipocyte enhancer binding protein 1 (AEBP1) gene, specifically, affecting its aortic carboxypeptidase-like protein (ACLP) isoform. We described the 15th patient (13th family) diagnosed with clEDS2. This patient presented with notable similarities in phenotype to the documented cases, along with additional characteristics such as significant prematurity and short stature. An EDS sequencing panel-based analysis revealed homozygous AEBP1: NM_001129.5:c.2923del, p.Ala975Profs*22 likely pathogenic variants, and maternally inherited heterozygous COL11A1: NM_001854.4:c.1160A>G, p.Lys387Arg variant of uncertain significance in our patient. Upon comprehensive review of all previously reported clEDS2 patients, our patient exhibited the following overlapping phenotypes, including cutaneous features: hyperextensibility, atrophic scars/delayed wound healing (100%), easy bruising (100%), excessive skin (93%); skeletal features: generalized joint hypermobility (93%), pes planus (93%), dislocation/subluxation (93%); and cardiovascular features (86%). Our patient did not display symptoms of the critical complications reported in a few individuals, including superior mesenteric artery aneurysms and ruptures, aortic root aneurysm/dissection, spontaneous pneumothoraxes, and bowel ruptures. Together, this case expands the genetic and clinical phenotypic spectrum of AEBP1-related clEDS2. [ABSTRACT FROM AUTHOR]

TRAF7-related disorders represent some of the rarest inherited disorders, exhibiting clinical features that overlap with cardiac, facial, and digital anomalies with developmental delay (CAFDADD) syndrome, as well as blepharophimosis-mental retardation syndrome (BMRS). A 36-year-old male, presenting with total blindness, blepharophimosis, and intellectual disability, was admitted for the assessment of resting dyspnea several months previously. He had a history of being diagnosed with obstructive sleep apnea (OSA). Transesophageal and transthoracic echocardiography unveiled right ventricular dilatation without significant pulmonary hypertension, bicuspid aortic valve with aortic root aneurysm, and aortic regurgitation in the proband. Sanger sequencing identified a de novo TRAF7 variant (c.1964G>A; p.Arg655Gln). Subsequently, aortic root replacement using the Bentall procedure was performed. However, despite the surgery, he continued to experience dyspnea. Upon re-evaluating OSA with polysomnography, it was discovered that continuous positive airway pressure support alleviated his symptoms. The underlying cause of his symptoms was attributed to OSA, likely exacerbated by the vertebral anomaly and short neck associated with CAFDADD syndrome. Clinicians should be attentive to the symptoms associated with OSA as it is a potentially serious medical condition in patients with TRAF7 variants. [ABSTRACT FROM AUTHOR]

Background Aortic regurgitation (AR) associated with detachment of the aortic valve commissure is extremely rare. We present a case of progressively worsening severe chronic AR due to detachment of the aortic valve commissure during hospitalization that was confirmed with multimodality imaging. Case summary A 50-year-old male with Marfan syndrome visited our hospital to receive treatment for cholelithiasis. Pre-operative examination revealed severe AR and aortic root aneurysm. Because the patient was asymptomatic, it was decided that cholecystectomy should be performed first. However, the patient's heart failure worsened acutely when his blood pressure increased just before induction of anaesthesia. The patient required intubation and management of heart failure. Five days later, the patient underwent cholecystectomy. He was treated for heart failure and underwent open heart surgery on the 35th hospital day. Intraoperative transoesophageal echocardiography revealed that his AR was caused by both enlargement of the aortic root and localized dissection of the aortic valve commissure, which was supported by intraoperative findings and histopathological evaluation. Aortic regurgitation was exacerbated by a new localized dissection, resulting in acute worsening of heart failure. Discussion Aortic valve commissure detachment can easily lead to sudden onset of severe AR, deteriorating haemodynamics, and acute pulmonary oedema. Since delayed medical treatment leads to poor clinical outcomes, prompt and accurate diagnosis and appropriately timed surgical intervention are essential. This very rare case of severe AR worsening due to spontaneous aortic valve commissure dissection was evaluated with multiple modalities during hospitalization. Understanding this clinical condition will help cardiologists provide better medical care. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 64-year-old patient with a rare combination of conditions, including a partial atrioventricular septal defect (PAVSD), bicuspid aortic valve (BAV), and idiopathic thrombocytopenic purpura (ITP). Topics discussed include the unique surgical approach involving a Bentall procedure, hemiarch replacement, and PAVSD repair, as well as a review of existing literature on adult PAVSD repair, highlighting the complexity and challenges of such cases.

Mitral valve aneurysm (MVA) is characterized by a saccular outpouching of the mitral leaflet, and it represents a rare condition typically associated with aortic valve endocarditis. Three-Dimensional Transesophageal Echocardiography (3D-TEE) serves as an effective tool for detecting the presence of MVA and its potential complications. In this report, we present a case involving a young man with striking images of bicuspid aortic valve endocarditis complicated by an aortic root abscess and multiple perforated mitral valve aneurysms, diagnosed using 3D TEE. This case suggests the uncommon coexistence of Marfan like morphotype, bicuspid aortic valve, and infective endocarditis as a triple mechanism in the occurrence of MVA. It underscores the significance of early and accurate imaging diagnosis for facilitating prompt surgical intervention. [ABSTRACT FROM AUTHOR]

Purpose: Aortic root and ascending aortic aneurysms are rarely encountered in pediatric patients. Our aim in this study was to compare our surgical approach and results in this patient group with similar studies in the literature. Materials and Methods: This study retrospective in design and its cohort consists of pediatric patients who were operated on due to the diagnosis of aortic root and/or ascending aortic aneurysm. The etiologies of the patients that caused the aneurysm were investigated preoperatively, and the severity of the disease in the aortic valve and ascending aorta and surgical indications were revealed with detailed imaging methods. Operative data was then retrieved and early postoperative morbidity and mortality, as well as post-discharge aortic valve functions were evaluated. The obtained data was compared with those of similar articles in the literature. Results: A total of eight pediatric patients were operated on with the diagnosis of aortic root and ascending aortic aneurysm. The average age of the patients was eleven (±4.03) years. The underlying cause of three (37.5%) patients was Marfan syndrome, two (25%) had bicuspid aortic valve and one (12.5%) had Wiscott-Aldrich Syndrome, previously operated sinus valsalva aneurysm and operated truncus arteriosus. There was 3rd degree aortic valve insufficiency in six patients (75%). The average aortic annulus diameter, mean sinus of valsalva diameter and ascending aorta diameter of the patients were 26.7 mm (±5.3), 40.9 mm (±9.7) and 37.8 mm (±9.2), respectively. The David I procedure was performed in three (37.5%) patients, while the Bentall procedure or aortic valve and ascending aorta replacement was performed on the other five patients. Non-cardiac mortality was observed in one (12.5%) patient, whereas native and mechanical valve dysfunction was not observed in any patient after discharge. Conclusion: Both valve-sparing surgical procedures and other replacement techniques can be used safely in pediatric patients with aortic root and ascending aortic aneurysms. [ABSTRACT FROM AUTHOR]

We reported a 54-year-old patient with a ruptured sinus of Valsalva aneurysm who successfully underwent aortic root replacement with coronary artery bypass grafting. The images of the perioperative findings were impactful. Although previous studies reported a high mortality rate in cases of ruptured pericardium, we rescued the patient with no postoperative adverse events. [ABSTRACT FROM AUTHOR]

Background: Valve-sparing aortic root replacement (VSARR) is a safe and effective surgical procedure to treat aortic root aneurysm. This meta-analysis aimed to investigate how this procedure might differ in patients with bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV). Design: Meta-analysis with meta-regression and systematic review. Setting: Systematic search in the following databases: PubMed, Cochrane Central Register of Controlled Trials, and Embase. Interventions: All observational studies of VSARR in patients with BAV or TAV were included in our study. Studies were included without any restrictions on language or publication date. A trial sequential analysis and a post-hoc meta-regression was performed on the main outcomes. Result: Eleven articles met the inclusion criteria. A total of 1138 patients in BAV group, and 2125 patients in TAV group. No significant differences in gender and age were observed between BAV and TAV patients. BAV and TAV patients showed no differences in in-hospital mortality rate [0.00% vs. 1.93%; RR (95% CI) 0.33 (0.09, 1.26), I2 = 0%, P = 0.11] and the rate of in-hospital reoperation [5.64% vs. 5.99%; RR (95% CI) 1.01(0.59, 1.73), I2 = 33%, P = 0.98]. The overall long-term mortality rate of BAV patients was better than that of TAV patients [1.63% vs. 8.15%; RR (95% CI) 0.34 (0.13, 0.86), I2 = 0%, P = 0.02]. During the follow-up observation period, patients in TAV group showed small but no statistic advantage in 3-year, 5-year, and over 10-year incidences of reintervention. Regarding the secondary endpoints, the two groups showed similar aortic cross-clamping time and total cardiopulmonary bypass time. Conclusion: The VSARR techniques yielded similar clinical outcomes in both BAV and TAV patients. Although patients with BAV might have a higher incidence of reinterventions after initial VSARR, it is still a safe and effective approach to treat aortic root dilation with or without aortic valve insufficiency. TAV patients showed small but no statistic advantage in long-term (over 10 years) reintervention rate, which means, patients with BAV may face a higher risk of reintervention in the clinic. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The aim of this retrospective study was to assess the long-term results of root remodelling with tricuspid aortic valves and the effects of concomitant cusp repair and annuloplasty. METHODS Between October 1995 and December 2021, 684 patients with root aneurysm and regurgitant tricuspid valves were treated by root remodelling. The mean age was 56.5 [standard deviation (SD): 14] years, and 538 (77.6%) were male. Relevant aortic regurgitation was present in 68.3%. Concomitant procedures were performed in 374 patients. The long-term results were analysed. The mean follow-up of 7.2 (SD: 5.3) years (median 6.6 years); it was 95% complete (4934.4 patient-years). RESULTS Cusp prolapse was repaired in 83%, and an annuloplasty was added in 353 instances (51.6%). Hospital mortality was 2.3%, and survival was 81.7% (SD: 1.2) and 55.7% (SD: 5.8) at 10 and 20 years; age and measurement of effective height were independent predictors for death. Freedom from Aortic insufficiency (AI) II was 90.5 (SD: 1.9) at 10 years and 76.7 (SD: 4.5) at 20 years. Cusp repair of all cusps showed a lower freedom from recurrent AI ≥II at 10 years (P < 0.001). Suture annuloplasty showed a lower freedom from recurrent AI II at 10 years (P  = 0.07). Freedom from reoperation was 95.5 (SD: 1.1) and 92.8 (SD: 2.8) at 10 and 20 years. The addition of an annuloplasty showed no difference (P  = 0.236). Cusp repair had no effect on valve durability (P  = 0.390). CONCLUSIONS Root remodelling leads to good long-term stability. The addition of cusp repair improves the valve stability over time. The addition of suture annuloplasty improves early valve competency; it showed no effect on freedom from reoperation up to 10 years. [ABSTRACT FROM AUTHOR]

We present a case of a 37-year-old woman diagnosed with Parkes-Weber syndrome characterized by high-flow arterio-venous malformations of the pelvic region with an estimated cardiac output of 7.2–8.5 l/min. Due to concomitant Marfan syndrome, the patient also presented an aortic root aneurysm needing surgical treatment under cardiopulmonary bypass. Concerns arose regarding the ability to maintain sufficient perfusion pressure during cardiopulmonary bypass due to the arterio-venous malformations. To address this, an intravascular balloon occlusion at the level of the aortic bifurcation was placed in order to limit arterial inflow into the arterio-venous malformations and achieve optimal perfusion pressure during cardiopulmonary bypass. The patient did not experience any complications and recovered completely after surgery. [ABSTRACT FROM AUTHOR]

Background: To evaluate different aortic root surgery techniques and their contemporary clinical outcomes in patients with regurgitant aortic valve and aortic root aneurysm. Methods: The study consisted of 141 adult patients who underwent aortic valve reimplantation (David group = 73) or aortic valve replacement surgery (Bentall group = 68) for aortic valve regurgitation (AR) and dilatation of the aortic root at our institution within the same period (April 2004–October 2016). Kaplan–Meier method was used to estimate survival and other clinically relevant outcomes between the groups. Results: The completeness of clinical follow-up was 100%, with a mean time of 8.0 ± 3.8 years. Thirty-day (in-hospital) mortality rates were equivalent between groups (1.3 and 1.5%, p = 1.0). The overall survival rates at 10 years were significantly better for the David group patients comparing to Bentall group patients (95.3 ± 2.6% vs 79.7 ± 6.8%; p = 0.04) with similar freedom from AV related reoperation (94.4 ± 2.7% vs 98.5 ± 1.5%; p = 0.2). Freedom from bleeding events at 10 years was 90.7 ± 3.6% for Bentall group patients and none were observed among David group patients (p = 0.01). Conclusions: Aortic valve and root surgery can be performed with equivalent safety and efficacy using either valve-sparing (David procedure) or valve-replacing (Bentall procedure) techniques in selected patients. Furthermore, patients after the David procedure demonstrated significantly improved survival and low risk of bleeding in comparison to the Bentall procedure with an acceptable risk of reoperation at 10 years follow-up. [ABSTRACT FROM AUTHOR]

Introduction: AEBP1-related classical-like EDS (clEDS type 2) is a rare type of Ehlers-Danlos syndrome (EDS) that was first reported in 2016. There are overlapping clinical features with TNXB-related classical-like EDS (or clEDS type 1), including skin hyperextensibility, joint hypermobility, and easy bruising. There are currently nine reported individuals with AEBP1-related clEDS type 2. This report confirms previous findings and provides additional clinical and molecular data on this group of individuals. Materials and methods: Two individuals (P1 and P2), with features of a rare type of EDS, were clinically assessed in the London national EDS service and underwent genetic testing. Results: Genetic testing in P1 revealed likely pathogenic AEBP1 variants: c.821del: p. (Pro274Leufs*18) and c.2248T>C:p. (Trp750Arg). In P2 pathogenic AEBP1 variants, c.1012G>T:p. (Glu338*) and c.1930C>T:p. (Arg644*) were identified. Discussion: These two individuals increased the reported number of individuals with AEBP1-related clEDS to 11 (six females and five males). There are shared features with previously reported individuals, including hypermobility (11/11), skin hyperextensibility (11/11), presence of atrophic scarring (9/11), and easy bruising (10/11). In P1, a chronic right vertebral artery dissection, mild dilatation of the splenic artery, aberrant subclavian artery, and tortuous iliac arteries were observed at the age of 63 years. Cardiovascular disease has been reported, including mitral valve prolapse (4/11), peripheral arterial disease (1/11), and aortic root aneurysm requiring surgical intervention (1/11). Hair loss has been reported in 6/11 individuals (five females and one male), only one of which was documented to have a formal diagnosis of androgenetic alopecia, while other individuals were described as having thinning of hair, male pattern hair loss, or unspecified alopecia. Conclusion: The clinical features of individuals with AEBP1-related EDS have not been fully elucidated yet. Hair loss is present in 6/11 individuals with AEBP1-related clEDS and appears to be a feature of this condition. This is the first time hair loss has been formally reported as a characteristic feature in a rare type of EDS. Cardiovascular surveillance seems warranted in this condition because 2/ 11 individuals have evidence of arterial aneurysm and/or dissection. Further descriptions of affected individuals are necessary to update diagnostic criteria and management guidelines. [ABSTRACT FROM AUTHOR]

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Open in new tab Download slide OBJECTIVES Over the past 20 years, valve-sparing aortic root replacement has aroused increasing interest because of a progressive attitude towards the preservation of natural tissue. Aortic reimplantation is the most used technique to spare the valve, allowing simultaneously aortic root replacement and aortic annular stabilization. The reimplantation into a graft with sinuses guarantees an optimal anatomic and functional reconstruction with established good results at 15 years. The aim of this study is to report the world longest follow-up (up to 20 years) of aortic valve reimplantation using the Valsalva graft. METHODS From February 2000 to December 2021, 265 consecutive patients with aortic root aneurysm received aortic valve reimplantation using the Valsalva graft. From 2018, leaflet plication with the routine use of calliper was performed. For each patient, we performed both intraoperative and post-procedural transoesophageal echocardiography. All patients were followed with clinical assessment and echocardiography. The mean duration of follow-up was 85 ± 63 months. RESULTS The study cohort had a median age of 55 ± 18 and 87.2% were male. The aortic root aneurysm was associated to bicuspid aortic valve in 18.9% of patients and to Marfan syndrome in 10.6% of cases. 55.9% had an aortic regurgitation ≥ 2+. Overall survival at 15 was 87.6 ± 3.4. Freedom from cardiac death was stable at 99.6 ± 0.4 at 5, 10 and 15 years. Freedom from recurrent AR ≥3+ and freedom from reoperation remained stable at 10 and 15 years at 92.2 ± 2.1 and 95.9 ± 1.6, respectively. There was a minimal incidence of infective endocarditis (0.8%), thromboembolism (2.2%) and haemorrhage (2.0%). Six out of 7 patients requiring reoperation had surgery in the first period of our experience (last in 2004). Early suboptimal results had a negative effect on residual aortic regurgitation. Moreover, we hypothesized that the routine use of calliper may have contributed to a further improvement of the outcome, even if these data need to be confirmed by a longer follow-up. CONCLUSIONS The first long-term follow-up after aortic valve reimplantation using the Valsalva graft demonstrated excellent results. These long-term results gradually improved with learning curve, remaining stable during the second decade of observation. The systematic use of calliper may have contributed to a further improvement of the outcome. [ABSTRACT FROM AUTHOR]

A 49-year-old man with Marfan syndrome and a history of a Bentall procedure presented with severe mitral regurgitation. Due to the high risk of traditional surgery, a minimally invasive thoracoscopic hypothermic fibrillatory arrest technique was used for mitral valve replacement. The procedure was successful, highlighting a simplified and reliable approach for managing recurrent mitral regurgitation in Marfan syndrome patients post-Bentall procedure. The study was supported by various research grants and no conflicts of interest were reported by the authors. [Extracted from the article]

This article discusses the need for new thresholds for surgical repair in patients with ascending thoracic aortic aneurysm disease (ATAA). The current general recommendation is a 55 mm aortic diameter threshold, but the cumulative evidence for this treatment strategy is not robust. The article presents a 30-year experience of patients with ATAA from the Aortic Institute at Yale University, providing important insights into the true risk for adverse aortic events and the natural history of the disease. The study highlights the need to re-evaluate treatment thresholds for ATAA and suggests that specific risk groups may benefit from a lower threshold. However, there are still knowledge gaps in the treatment of genetic subgroups, women, and the morphological categorizations of aortic root aneurysms. Overall, this study contributes to the understanding of ATAA and supports the need for individualized treatment thresholds. [Extracted from the article]

Open in new tab Download slide OBJECTIVES The Bentall procedure is the gold standard for patients with combined aortic root dilation and valve dysfunction. Over the past decade, fast-track (FT) perioperative anaesthetic management protocols have progressively evolved. We reviewed our results for selected patients undergoing Bentall surgery under an FT protocol. METHODS We retrospectively analysed a consecutive cohort of patients who underwent elective Bentall procedures at our institution between 2000 and 2018. Complex aortic root repair (i.e. David and Ross procedure, redo surgery, major concomitant procedures, emergency repair for acute dissections) was excluded. Patients who underwent conventional perioperative treatment and those treated according to our institutional FT concept were compared following 1:1 propensity score matching. RESULTS Of 772 patients who fit the in- and exclusion criteria, 565 were treated conventionally post-surgery, while 207 were treated using the FT protocol. Propensity score matching resulted in 197 pairs, with no differences in baseline characteristics after matching. In-house mortality, 30-day mortality and overall all-cause long-term mortality were comparable between the FT and the conventionally treated cohort. Postoperative anaesthetic care unit/intensive care unit length-of-stay (6.2 vs 20.6 h, P  = 0.03) and postoperative ventilation times (158.9 vs 465.5 min, P  < 0.001) were significantly shorter in the FT cohort. There were no differences in rates of postoperative adverse events. CONCLUSIONS In centres with experienced anaesthesiologists, perioperative FT management is non-inferior to conventionally treated patients undergoing elective Bentall procedures without compromising patient safety. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Our goal was to evaluate the outcome of valve-sparing root replacement (VSRR) and to compare the outcomes to those of patients having composite valve–graft conduit aortic root replacement (CVG-ARR) in a cohort of patients with aortic root aneurysm ± valve insufficiency, without valvular stenosis. Although valve-sparing procedures are preferable in young patients, there is a lack of comparative data in comparable patients. METHODS The VSRR procedures were performed in 2005 patients, and 218 patients underwent a CVG-ARR procedure. Exclusion criteria included aortic dissection, endocarditis and valvular stenosis. Propensity score matching (3:1 ratio) was applied to compare VSRR (reimplantation 33% and remodelling 67%) and CVG-ARR. RESULTS We matched 218 patients with CVG-ARR to 654 patients with VSRR (median age, 56.0; median follow-up was 4 years in both groups; interquartile range 1–5 years). Early mortality was 1.1% of those who had VSRR versus 2.3% in those who had CVG-ARR. Survival was 95.4% [95% confidence interval (CI) 94–97%] at 5 years in patients who had VSRR versus 85.4% (95% CI 82–92%) in those who had CVG-ARR; P  = 0.002. Freedom from reintervention at 5 years was 96.8% (95% CI 95–98%) with VSRR and 95.4% (95% CI 91–99%) with CVG-ARR (P  = 0.98). Additionally, there were more thromboembolic, endocarditis and bleeding events in the patients who had CVG-ARR (P  = 0.02). CONCLUSIONS This multicentre study shows excellent results after valve-sparing root replacement in patients with an ascending aortic aneurysm with or without valve insufficiency. Compared to composite valve-graft aortic root replacement, survival is better and valve-related events are fewer. Consequently, valve-sparing procedures should be considered whenever a durable repair is feasible. We advocate a valve-sparing strategy even in more complex cases when performed in experienced centres. [ABSTRACT FROM AUTHOR]

Introduction: Full sternotomy is the gold standard for aortic root surgery. However, some individuals may benefit from an upper mini-sternotomy. Objective: The aim of the present study was to match results between cases who received aortic root replacement by an upper mini-sternotomy (mini-Bentall) and those who underwent the treatment through a median sternotomy (full-sternotomy Bentall). Patients and Methods: A total of 20 patients got the full-sternotomy Bentall operation and 9 patients underwent the mini-Bentall treatment between November 2016 and November 2020. Mini-Bentall surgery was accomplished by an incision beginning at the right upper sternal border and finishing at the fourth right intercostal gap. Concurrent surgeries, such as mitral valve intervention or aortic dissection, and profound hypothermic circulatory arrest, disqualified patients from participation. Major surgical complications and operative mortality were measured, as were open chest and reexploration for bleeding, hospital length of stay, blood transfusion rates, and duration of mechanical ventilation. Results: The rate of serious postoperative problems, including the need for a blood transfusion, was not significantly different between the two groups. There were no cases of stroke in either group, and the mini-Bentall group did not have any conversions to median sternotomies. Conclusion: In certain individuals undergoing elective aortic root replacement surgery, a partial upper J-shaped sternotomy can be a secure and alternative approach. Unfortunately, further research is needed before this method can replace current best practises. [ABSTRACT FROM AUTHOR]

This article discusses a case of Marfan syndrome (MFS) caused by a novel FBN1 gene mutation. The patient, a 31-year-old man, presented with symptoms of fatigue and shortness of breath. Upon examination, he exhibited physical signs of Marfan syndrome, including arachnodactyly. Diagnostic tests revealed a giant aortic root aneurysm and severe aortic regurgitation. Whole exome sequencing confirmed the presence of a heterozygous deleterious variant in the FBN1 gene. This discovery expands our understanding of FBN1 variants and provides valuable insights for genetic counseling. [Extracted from the article]

The main goal of minimally invasive surgery is to reduce the perioperative trauma, accelerate patient mobilization and reduce the length of hospital stay. Due to the development of modern technology, these treatments can be offered to a wider group of patients. For many years, aortic root surgery consisted of mechanical conduit implantation and, therefore, necessitated lifelong anticoagulation. At present, in patients with aortic root aneurysm and significant aortic valve regurgitation, it is possible to perform minimal-access valve sparing surgical procedures. The current paper is a brief description of the surgical technique for aortic root aneurysm surgery with preservation of the patient's own valve using the David procedure. [ABSTRACT FROM AUTHOR]

This article reports on a case of a 69-year-old patient who developed unilateral chylothorax, a rare complication of COVID-19 infection, six months after the initial infection. The patient had a history of seminoma and radiation therapy, as well as tricuspid regurgitation. He experienced symptoms such as a persistent cough and shortness of breath, and a chest x-ray revealed a significant pleural effusion. The patient underwent various treatments, including thoracic duct embolization and surgery, and ultimately recovered from chylothorax. The authors hypothesize that the patient's tricuspid regurgitation may have contributed to the development of chylothorax during the COVID-19 infection. [Extracted from the article]