Marfan and Loeys-Dietz aortic phenotype: A potential tool for diagnosis and management.

Objective: In heritable aortic diseases, different vascular involvement may occur with potential variable implications in aortic dilation/dissection risk. This study aimed to analyze the aortic anatomy of individuals with Marfan syndrome and Loeys-Dietz syndrome to identify possible morphological differences. Methods: Computed tomography and magnetic resonance imaging of the thoracoabdominal aorta from the proximal supra-aortic vessels to the femoral bifurcation level of 114 patients with Marfan and Loeys-Dietz syndromes and 20 matched control subjects were examined. Aortic diameters, areas, length, and tortuosity were measured in different aortic segments using specific vessel analysis software. Results: Patients with Marfan syndrome showed a higher prevalence of ascending aorta and aortic root dilation (P = .011), larger and longer aortic roots (P = .013) with pear-shaped phenotype, larger isthmus/descending aorta diameter ratio (P = .015), and larger suprarenal aorta and iliac arteries. Patients with Loeys-Dietz syndrome showed longer indexed segments and a significantly longer arch (P = .006) with type 2/3 arch prevalence (P = .097). Measurement ratios analysis provided cut-off values (aortic root to ascending aorta length/aortic root diameter, aortic root/sinotubular junction, aortic root/ascending aorta diameter) differentiating patients with Marfan syndrome from patients with Loeys-Dietz syndrome, even in the early stage of the disease. Conclusions: Both syndromes show peculiar anatomic patterns at different aortic levels irrespective of aortic dilation and disease severity. These features may represent the expression of different genetic mutations on aortic development, with a potential impact on prognosis and possibly contributing to better management of the diseases. The systematic adoption of whole body imaging with magnetic resonance or computed tomography should always be considered, because they allow a complete vascular assessment with practical indicators of differential diagnosis.

Reoperative aortic root surgery: single-center long-term outcomes and literature review.

Background and Objective: Reoperative aortic root surgery has become more and more common over the years and is considered high-risk, with significantly worse outcomes compared to first-procedure root surgery. At our institution, this kind of surgery is frequently performed. The aim of the present review is to describe currently available literature on reoperative surgery on the aortic root in terms of patients' population, indications for surgery and outcomes and to present our center's experience on the matter. Methods: A literature review was performed in order to identify pertinent studies. They were then compared and described. We also described preoperative characteristics, operative strategies and outcomes of all the patients who underwent redo aortic root surgery from January 1986 to December 2022 at our center. Key Content and Findings: Our literature review identified 12 pertinent studies, with a total of 16,627 considered patients. The most frequent indications for redo surgery were endocarditis (35.5%), aneurysm, dissection and pseudoaneurysm. Mean cardiopulmonary bypass (CPB) and cross-clamp times were 218 and 152 minutes, respectively. In-hospital mortality was 12%. When analyzing our center's data, 344 procedures were identified. Aortic root dilation was the most frequent indication (36.9%). Mean CPB and cross-clamp times were 218.0±78.8 and 158.2±49.7 minutes, respectively. In-hospital mortality was 9.6%. Survival at 5 and 15 years was 76.1% and 51.4% respectively. Freedom from further aortic reintervention was 88.1% after 5 years and 64.9% after 15 years. Conclusions: Reoperative aortic root surgery is a difficult cardiac procedure which is linked to significantly higher mortality than first-time root replacement. If it is performed by experienced surgeons with a careful preoperative planning its result can still be satisfactory. Our results showed acceptable rates of mortality and reinterventions at follow-up. Endocarditis, however, was linked to worse outcomes.

Functional Foods and Adapted Physical Activity as New Adjuvant Therapy for Chronic Kidney Disease Patients.

BACKGROUND: Chronic kidney disease (CKD) will become the fifth leading cause of death in the world by 2040. It is fundamental to prevent and treat this pathology to reduce its impact on national health costs. This trial's aim is to evaluate the effects induced by a combination of consumed functional foods (FFs) with adapted physical activity (APA) on the progression of CKD-related comorbidities. METHODS: The study lasted 12 weeks. We divided 40 CKD patients into four groups: mixed (FF + APA), APA, FF and control group (usual care). The FFs were characterized by their total antioxidant capacity and antiradical activity. The APA was performed though an online training protocol, three times per week, 1 h each session. RESULTS: At the end of the study, we observed, in the mixed group, a decrease in azotemia (p = 0.0272), diastolic blood pressure (p = 0.0169), and C-reactive protein (p = 0.0313), with increases in the FORD test (p = 0.0203) and fat free mass (p = 0.0258). The APA group showed a reduction in total cholesterol (p = 0.0039). CONCLUSIONS: The combination of FFs and APA can help counteract several CKD-related comorbidities, such as arterial hypertension, dyslipidemia and uremic sarcopenia, and improve the CKD patients' quality of life.

Innovative Treatments to Counteract Endothelial Dysfunction in Chronic Kidney Disease Patients.

In chronic kidney disease (CKD) patients, several risk factors contribute to the development of endothelial dysfunction (ED), which can be described as an alteration in the cell structure or in the function of the endothelium. Among the well-known CKD-related risk factors capable of altering the production of endothelium-derived relaxing factors, we include asymmetric dimethylarginine increase, reduced dimethylarginine dimethylamine hydrolase enzyme activity, low-grade chronic systemic inflammation, hyperhomocysteinemia, oxidative stress, insulin resistance, alteration of calcium phosphorus metabolism, and early aging. In this review, we also examined the most important techniques useful for studying ED in humans, which are divided into indirect and direct methods. The direct study of coronary endothelial function is considered the gold standard technique to evaluate if ED is present. In addition to the discussion of the main pharmacological treatments useful to counteract ED in CKD patients (namely sodium-glucose cotransporter 2 inhibitors and mineralocorticoid receptor antagonist), we elucidate innovative non-pharmacological treatments that are successful in accompanying the pharmacological ones. Among them, the most important are the consumption of extra virgin olive oil with high intake of minor polar compounds, adherence to a plant-dominant, low-protein diet (LPD), an adaptive physical activity program and, finally, ketoanalogue administration in combination with the LPD or the very low-protein diet.

[Stroke and stroke prevention in cardiac and thoracic aortic surgery]. / Ictus e prevenzione dell'ictus in cardiochirurgia e in chirurgia dell'aorta toracica.

Perioperative stroke and neurological injuries in general are complications that can occur during and after cardiac surgery, particularly in aortic surgery that involves the aortic arch. The overall incidence of early and delayed stroke is about 1% according to recent meta-analyses. This incidence depends on interindividual risk factor profile and type of surgery. In order to reduce cerebrovascular complications during cardiac surgery, a number of preventative measures can be taken, including the evaluation of atherosclerotic plaques, the site of cannulation and neuroprotection strategies. During aortic arch surgery, main strategies for cerebral protection are represented by deep hypothermic circulatory arrest, retrograde and antegrade cerebral perfusion.

Comparison of 4 mortality scores for surgical repair of type A aortic dissection: a multicentre external validation.

OBJECTIVES: In the last decades, 4 different scores for the prediction of mortality following surgery for type A acute aortic dissection (TAAD) were proposed. We aimed to validate these scores in a large external multicentre cohort. METHODS: We retrospectively analysed patients who underwent surgery for TAAD between 2000 and 2020. Patients were enrolled from 10 centres from 2 European countries. Outcomes were the early (30-day and/or in-hospital) and 1-year mortality. Discrimination, calibration and observed/expected (O/E) ratio were evaluated. RESULTS: A total of 1895 patients (31.7% females, mean age 63.72 ± 12.8 years) were included in the study. Thirty-day mortality and in-hospital mortality were 21.7% (n = 412) and 22.5% (n = 427) respectively. The German Registry of Acute Aortic Dissection Type A (GERAADA) score shows to have the best discrimination [area under the curve (AUC) 0.671 and 0.672] in predicting as well the early and the 1-year mortality, followed by the International Registry of Acute Aortic Dissection (IRAD) model 1 (AUC 0.658 and 0.672), the Centofanti (AUC 0.645 and 0.66) and the UK aortic score (AUC 0.549 and 0.563). According to Hosmer-Lemeshow and Brier tests, the IRAD model I and GERAADA, respectively, were well calibrated for the early mortality, while the GERAADA and Centofanti for the 1-year mortality. The O/E analysis showed a marked underestimation for patients labelled as low-risk for UK aortic score and IRAD model I for both outcomes. CONCLUSIONS: The GERAADA score showed the best performance in comparison with other scores. However, none of them achieved together a fair discrimination and a good calibration for predicting either the early or the 1-year mortality.

Long-Term Atrioventricular Block Following Valve Surgery: Electrocardiographic and Surgical Predictors.

BACKGROUND: Bradyarrhythmia requiring pacemaker implantation among patients undergoing valve surgery may occur even after several years, with unclear predictors. Our aim was to investigate the incidence of pacemaker implantation at different follow-up times and identify associated predictors. METHODS: We conducted a retrospective study evaluating 1046 consecutive patients who underwent valve surgery at the Cardiac Surgery Division of Bologna University Hospital from 2005 to 2010. RESULTS: During 10 ± 4 years of follow-up, 11.4% of these patients required pacemaker implantation. Interventions on both atrioventricular valves independently predicted long-term pacemaker implantation (SHR 2.1, 95% CI 1.2-3.8, p = 0.014). Preoperative atrioventricular conduction disease strongly predicted long-term atrioventricular block, with right bundle branch block as the major predictor (SHR 7.0, 95% CI 3.9-12.4, p < 0.001), followed by left bundle branch block (SHR 4.9, 95% CI 2.4-10.1, p < 0.001), and left anterior fascicular block (SHR 3.9, 95% CI 1.8-8.3, p < 0.001). CONCLUSION: Patients undergoing valvular surgery have a continuing risk of atrioventricular block late after surgery until the 12-month follow-up, which was clearly superior to the rate of atrioventricular block observed at long-term. Pre-operative atrioventricular conduction disease and combined surgery on both atrioventricular valves are strong predictors of atrioventricular block requiring pacemaker implantation.

Frozen elephant trunk technique using hybrid grafts: 15-year outcomes from a single-centre experience.

OBJECTIVES: The purpose of the study is to compare the short- and long-term outcomes of the frozen elephant trunk (FET) technique based on 2 different hybrid grafts implanted from January 2007 to July 2022. METHODS: The study includes patients who underwent an elective or emergency FET procedure. Short-term, long-term mortality and freedom from thoracic endovascular aortic repair (TEVAR) were the primary end points. Analyses were carried out separately for the periods 2007-2012 and 2013-2022. RESULTS: Of the 367 enrolled, 49.3% received E-Vita Open implantation and 50.7% received Thoraflex Hybrid implants. Overall mean age was 61 years [standard deviation (SD) = 11] and 80.7% were male. The average annual volume of FET procedures was 22.7 cases/year. Compared to E-Vita Open, patients implanted with Thoraflex Hybrid grafts were more likely to receive distal anastomosis in zone 2 (68.3% vs 11.6%, P < 0.001) with a shorter stent portion, mean = 103mm (SD = 11.3) vs mean = 149 mm (SD = 12.7; P < 0.001) and they underwent a reduced visceral ischaemia time, mean = 42.5 (SD = 14.2) vs mean= 61.0 (SD = 20.2) min, P < 0.001. In the period 2013-2022, overall survival at 1, 2 and 5 years was 74.8%, 72.5% and 63.2% for Thoraflex and 73.2%, 70.7% and 64.1% for E-Vita, without significant differences between groups (log-rank test = 0.01, P = 0.907). Overall freedom from TEVAR at 1, 2 and 5 years was 66.7%, 57.6% and 39.3% for Thoraflex and 79%, 69.7% and 66% for E-Vita, with significant differences between groups (log-rank test = 5.28, P = 0.029). In a competing risk analysis adjusted for chronic/residual aortic syndromes and stent diameter, the Thoraflex group was more likely to receive TEVAR during follow-up (subdistribution hazard ratio SHR = 2.12, 95% confidence interval 1.06-4.22). CONCLUSIONS: The FET technique addresses acute and chronic arch disease with acceptable morbidity and mortality. Downstream endovascular reinterventions are very common during follow-up.

Frozen elephant trunk in acute aortic dissection: a literature review.

Background: Acute aortic dissection is a life-threatening condition that requires urgent surgical treatment. The frozen elephant trunk (FET) technique, including the Thoraflex hybrid prosthesis, has emerged as an effective strategy for treating complex aortic pathologies. With the widespread application of the FET technique, it continues to evolve, aiming to simplify procedures and reduce complications. These advancements provide improved outcomes and help save lives in patients with acute aortic dissection. Methods: For this review, PubMed databases were utilized from inception to March 2023. A descriptive approach was employed to identify and present the evidence regarding the application of the FET technique in acute settings and its clinical implications on the postoperative course. Results: In the reviewed studies, FET was a commonly used treatment approach for acute type A aortic dissection. A comprehensive analysis of 12 studies, comprising over 4056 FET procedures, revealed varying rates of early mortality (up to 21.1%), perioperative stroke (ranging from 2.7 to 18.0%), and spinal cord ischemia (ranging from 0 to 8.2%). During the follow-up period, which ranged from 6 to 108 months, the mortality rate was reported to be as high as 38%. Conclusions: The surgical management of acute aortic dissection remains challenging, but FET has shown promising results. Experienced teams have achieved acceptable in-hospital mortality and stroke rates, along with a lower risk of spinal cord injury compared to conventional repair. Furthermore, the FET technique has demonstrated positive alterations in the structure of the distal aorta, potentially improving long-term survival and reducing the necessity for future procedures.

Staging TEVAR after FET - an exception or the rule?

Purpose: Frozen elephant trunk (FET) was born as an ideal one-step procedure to treat complex arch and descending thoracic aorta pathology. It was then proved that it frequently needs reintervention, which can often be performed by thoracic endovascular aortic repair (TEVAR) extension since FET provides a safe proximal landing zone. We hereby describe our experience in TEVAR extension after FET, its main indications, technique, and outcomes. Methods: Between 2007 and 2022, 371 patients underwent FET at our center. Of these, 119 needed TEVAR extension. Some required more than one TEVAR, with a total of 154 procedures. The preoperative characteristics, indications, and outcomes were analyzed retrospectively. Results: Of 154 TEVAR procedures, 15 were performed in an urgent setting. Mean time from FET to TEVAR was 22,2 ± 28,73 months. Two patients died in the operating room; no others died during the hospital stay. Survival after 1, 2, 5, and 10 years was 96.2%, 93.9%, 90.1%, and 70.5% respectively. There was no statistically significant difference in the rates of TEVAR extension for patients in which a Thoraflex™ vs E-vita™ graft was used, nor for zone 2 vs zone 3 anastomosis and stent length. Conclusion: Though TEVAR extension is often required after FET, it is a safe and effective procedure with excellent post-operative outcomes in the short-, mid-, and long-term and allows successful treatment of complex aortic pathologies. Rigorous and specialized follow-up after FET is central to identify the right moment to intervene.

Odynophagia in individuals with neck pain: the importance of differential diagnosis in physiotherapy practice.

Odynophagia refers to painful swallowing caused by various underlying factors that must be excluded to determine the best treatment approach. Neck pain is a debilitating condition requiring treatment in rehabilitative settings. There are several circumstances in which odynophagia and neck pain coexist, such as tendinitis of the longus colli muscle and paravertebral calcification, prevertebral and retropharyngeal abscess, esophageal perforation, aortic dissection, thyroid cartilage fracture, thyrohyoid ligament syndrome, pneumomediastinum and subcutaneous emphysema, and after physical exercise. Physiotherapists are professionals most likely to encounter individuals with neck pain and provide interventions such as massage, manual therapy, exercise, and electrotherapy. Therefore, it is important to recognize that neck pain can stem from different clinical conditions that require interventions other than physiotherapy. A differential diagnosis is crucial to ensure appropriate referrals for therapeutic interventions.

Distal stent graft-induced new entry (dSINE) after frozen elephant trunk: a scoping review.

Background: The introduction of hybrid total arch replacement with the frozen elephant trunk (FET) technique has improved the field of aortic surgery by allowing single-stage treatment of complex aortic pathologies. Although FET has been associated with favorable aortic remodeling, it is also associated with the potential development of distal stent graft-induced new entries (dSINEs). The aim of our review is to collect data about the incidence and the supposed conditions for the occurrence of dSINE after total hybrid arch replacement with FET technique. Methods: The literature review was performed using PubMed databases from inception to January 2022. A descriptive approach to detect and display supposed risk factors and predictors for dSINE occurrence has been adopted. Results: Eight studies summarized the state-of-the-art of dSINE in a total number of 544 FET procedures performed to treat acute and chronic aortic dissections. The scoping review showed dSINEs occurrence in 69 patients (12.7%). The mean time between surgery and the diagnosis ranged from 12.6 to 30.6 months. Most patients that developed dSINE received endovascular treatment, whereas a couple of them needed open surgery. According to our experience, from January 2007 to December 2021, in 225 FET procedures a total of 54 cases of dSINE, both with Thoraflex and E-vita grafts have been detected. The mean time between the surgical procedure and the diagnosis was 27.2±33.6 months. Conclusions: dSINEs are frequent complications after FET. Although not emergent, they require proper treatment. Due to dSINE's asymptomatic nature and potential harm, a rigorous follow-up including angio-computed tomography (CT) should be planned.

[Management of acute type B aortic dissection: treatment and follow-up]. / Gestione della dissezione aortica acuta di tipo B: trattamento e follow-up.

Aortic dissection is a life-threatening condition caused by a tear in the tunica intima which creates a false lumen into the aortic wall. Acute type B aortic dissection (TBAD) is defined by the presence of the entry tear in the aorta distal to the left subclavian artery, without ascending aorta and arch involvement, and accounts for 25-40% of all aortic dissections. Optimal medical therapy (OMT), focused on blood pressure and heart rate control, remains the gold standard treatment, especially for patients with uncomplicated TBAD, while complicated dissections require surgical therapy. Recent studies have shown that a considerable number of patients treated only with OMT develop late aorta-related complications that increase morbidity and mortality, as well as the need for surgical intervention. During the last decades, emerging evidence indicates that thoracic endovascular aortic repair (TEVAR) is safe and effective in the treatment of TBAD, both complicated and uncomplicated, with improved long-term survival outcomes and aortic remodeling in combination with OMT compared to OMT alone. However, in cases of acute uncomplicated TBAD the optimal timing for TEVAR is not entirely clarified and there is lack of long-term evidence. Therefore, the role of pre-emptive TEVAR for these patients is still uncertain and the management of acute uncomplicated TBAD remains challenging.

Impact of Previous Cardiac Operations in Patients Undergoing Surgery for Type A Acute Aortic Dissection. Long-Term Follow Up.

Aim of this multicenter study was to evaluate the impact of reoperative cardiac surgery for type A acute aortic dissection (TAAAD) on early and long-term outcomes. Patients with history of previous cardiac surgery were included in group R while those undergoing first operation where included in group F. Kaplan-Meier analysis was used to evaluate long-term survival in the 2 groups. A total of 1472 patients were included in the analysis. Of these, 85 (5.8%) and 1387 (94.2%) were included in group R and F, respectively. Thirty-day mortality was 24% (20 patients) and 18% (249 patients) in groups R and F, respectively(P = 0.8). Kaplan-Meier survival at 10 and at 20-year was 51.5% and 30.2% in group R and 48% and 32% in group F (P = 0.368). Patients with a history of previous cardiac operations who develop TAAAD can undergo surgery with similar early and long-term outcomes compared to those at their first operation.