OBJECTIVE: To evaluate the diagnostic accuracy of the aortic dissection detection risk score (ADD-RS) used alone or in combination with D-dimer for detecting acute aortic syndrome (AAS) in patients presenting with symptoms suggestive of AAS.
METHODS: We searched MEDLINE, EMBASE, and the Cochrane Library from inception to February 2024. Additionally, the reference lists of included studies and other systematic reviews were thoroughly searched. All diagnostic accuracy studies that assessed the use of ADD-RS alone or with D-Dimer for diagnosing AAS compared with a reference standard test (e.g. computer tomographic angiography (CTA), ECG-gated CTA, echocardiography, magnetic resonance angiography, operation, or autopsy) were included. Two reviewers independently selected and extracted data. Risk of bias was appraised using QUADAS-2 tool. Data were synthesised using hierarchical meta-analysis models.
RESULTS: We selected 13 studies from the 2017 citations identified, including six studies evaluating combinations of ADD-RS alongside D-dimer>500ng/L. Summary sensitivities and specificities (95% credible interval) were: ADD-RS>0 94.6% (90%, 97.5%) and 34.7% (20.7%, 51.2%), ADD-RS>1 43.4% (31.2%, 57.1%) and 89.3% (80.4%, 94.8%); ADD RS>0 or D-Dimer>500ng/L 99.8% (98.7%, 100%) and 21.8% (12.1%, 32.6%); ADD RS>1 or D-Dimer>500ng/L 98.3% (94.9%, 99.5%) and 51.4% (38.7%, 64.1%); ADD RS>1 or ADD RS = 1 with D-dimer>500ng/L 93.1% (87.1%, 96.3%) and 67.1% (54.4%, 77.7%).
CONCLUSIONS: Combinations of ADD-RS and D-dimer can be used to select patients with suspected AAS for imaging with a range of trade-offs between sensitivity (93.1% to 99.8%) and specificity (21.8% to 67.1%).

UNASSIGNED: Tyrosine kinase inhibitors targeting the vascular endothelial growth factor (VEGF) inhibitor pathway with immune checkpoint blockade have shown promising outcomes in managing metastatic renal cancer. However, they increase the risk of a person developing high blood pressure and cardiovascular complications.
UNASSIGNED: In this study, we report the case of a 73-year-old woman on axitinib and pembrolizumab for her Stage 4 renal cell carcinoma. She presented with intractable chest pain and high systolic blood pressure, not responding to opiates. Her computed tomography angiography results showed an acute intra-mural haematoma with a rupture in the descending thoracic aorta. She underwent emergency thoracic endovascular aortic repair. Post-operatively, she recovered fully without any neurological or cardiovascular issues.
UNASSIGNED: The severity of cardiovascular haemodynamic complications arising from the consumption of VEGF inhibitors and from immunotherapy and the lack of anti-hypertensive strategies to adequately manage such events require an unequivocal and urgent assessment of their cardiovascular safety. This case highlights the crucial role of cardiovascular oncology in managing such acute aortic catastrophes.

This work aims to provide a comprehensive description of the characteristics of a group of acute aortic diseases that are all potentially life-threatening and are collectively referred to as acute aortic syndromes (AASs). There have been recent developments in the care and diagnostic plan for AAS. A substantial clinical index of suspicion is required to identify AASs before irreversible fatal consequences arise because of their indefinite symptoms and physical indicators. A methodical approach to the diagnosis of AAS is addressed. Timely and suitable therapy should be started immediately after diagnosis. Improving clinical outcomes requires centralising patients with AAS in high-volume centres with high-volume surgeons. Consequently, the management of these patients benefits from the increased use of aortic centres, multidisciplinary teams and an \"aorta code\". Each acute aortic entity requires a different patient treatment strategy; these are outlined below. Finally, numerous preventive strategies for AAS are discussed. The keys to good results are early diagnosis, understanding the natural history of these disorders and, where necessary, prompt surgical intervention. It is important to keep in mind that chest pain does not necessarily correspond with coronary heart disease and to be alert to the possible existence of aortic diseases because once antiplatelet drugs are administered, a blocked coagulation system can complicate aortic surgery and affect prognosis. The management of AAS in \"aortic centres\" improves long-term outcomes and decreases mortality rates.

Marfan syndrome (MFS) is a systemic connective tissue disease that commonly and most severely affects the ocular, skeletal, and cardiovascular systems. The aim of the manuscript is to review the aortic involvement and complications in MFS, including aortal dissection, thoracic aortic aneurysm, abdominal aortic aneurysm, and acute aortic syndrome. Dissecting thoracic aortic aneurysm and progressing aortic root enlargement are the major causes of MFS morbidity and mortality. Guidelines on aortic disease endorsed by the American College of Cardiology, and the American Heart Association recommend the measurement of the external and internal aortic diameters perpendicular to the axis of blood flow when Computed Tomography, or Magnetic Resonance Imaging, or Cardiac Echography are performed. The pathophysiology, diagnosis, prevention, and medical and surgical treatments of MFS associated with aortic complications are reported in this narrative review. Development and strengthening of centers specialized in cardiovascular diseases and MFS, together with an improvement in the knowledge of its pathogenesis through genetics and proteomics investigations, can ameliorate the prognosis of this disease.

Aortic intramural hematoma (AIH) is a life-threatening emergency that involves aortic wall integrity and is characterized by either a direct rupture of the vasa vasorum or spontaneous bleeding of an arterial plaque located in the tunica media of the aortic wall. A notable difference between AIH and acute aortic dissection is the absence of an intimal flap, a finding discernable on computed tomography angiography (CTA). Follow-up imaging allows for the monitoring of disease progression or early findings of impending complications. While some patients may require surgical intervention, medical management with blood pressure control remains the mainstay in treatment. Our case describes a patient who was found to be in cardiac arrest secondary to ventricular fibrillation and was then found to have presumed Stanford Type A aortic dissection on CTA. After reviewing the scans, the diagnosis was reclassified to AIH due to the absence of an intimal flap, the patient was then managed medically for AIH with antihypertensive medications.

In acute aortic dissection (AAD) sex heterogeneity reports are not exhaustive and in part even conflicting.
To explore sex differences in clinical features, management, and outcomes among patients with type A AAD.
A systematic review and meta-analysis of the literature were conducted for studies (2004-2022) reporting type A AAD sex differences. Among the 1938 studies retrieved, 16 (16 069 patients, 7142 women, and 8927 men) fulfilled all eligibility criteria. Data were aggregated used the random-effects model as pooled risk ratio and mean difference. Due to information reported by considered manuscripts, analysis were performed only among surgically treated type A AAD patients. At the time of hospital presentation type A AAD women were older than men but had lower body mass index (BMI), body surface area (BSA), and creatinine plasma levels. Active smoking, bicuspid aortic valve, and previous cardiac surgery were less common in women while diabetes mellitus was more frequent. Furthermore, women experienced more frequently pericardial effusion/cardiac tamponade than men. Interestingly, in-hospital surgical mortality did not differ between sexes [risk ratio (RR), 1.02; 95% confidence interval (CI), 0.53-1.99; P = 0.95], whereas 5 (RR 0.94; 95% CI: 0.92-0.97; P < 0.001) and 10-year survival (RR 0.82; 95% CI: 0.74-0.92; P = 0.004) was higher among men. A descriptive analysis of in-hospital outcomes among medically treated type A AAD patients confirmed prohibitive high mortality for both sexes (men 58.6% vs. women 53.8%, P = 0.59).
A female sex phenotype appears to be evident in type A AAD implying the need for a personalized management patient approach along with tailored preventive strategies.
CRD42022359072.
Reports regarding sex heterogenicity in acute aortic dissection (AAD) are not exhaustive and in part even conflicting. Sex differences in clinical features, management, and outcomes were investigated among patients with type A AAD through a systematic review and meta-analysis of literature. A female sex phenotype appears to be evident in type A AAD implying the need for a personalized management patient approach along with tailored preventive strategies. Interestingly, while in-hospital surgical mortality did not differ between sexes, 5- and 10-year survival was higher among men. In the near future, definitive sex-specific data from international clinical registries and trials are expected.

The purpose of this paper is to describe all available evidence on the distinctive features of a group of 4 life-threatening acute aortic pathologies gathered under the name of acute aortic syndrome (AAS). The epidemiology, diagnostic strategy, and management of these patients has been updated. The authors propose a new and simple diagnostic algorithm to support clinical decision making in cases of suspected AAS, thereby minimizing diagnostic delays, misdiagnoses, and unnecessary advanced imaging. AAS-related entities are reviewed, and a guideline to avoid imaging misinterpretation is provided. Centralization of patients with AAS in high-volume centers with high-volume surgeons is key to improving clinical outcomes. Thus, the role of multidisciplinary teams, an \"aorta code\" (streamlined emergent care pathway), and aortic centers in the management of these patients is boosted. A tailored patient treatment approach for each of these acute aortic entities is needed, and as such has been summarized. Finally, a set of prevention measures against AAS is discussed.

Aortoenteric fistula is a life-threatening emergency and is associated with high morbidity and mortality. Prompt surgical intervention before the aneurysm ruptures lowers the mortality rate to about 50%. Potential imaging mimics for aortoenteric fistula include retroperitoneal fibrosis, mycotic aortic aneurysm, and infectious aortitis. Secondary aortoenteric fistula has relative higher incidence compared to primary and is more common with open aortic repair versus endovascular stent graft repair. Ectopic gas in the aneurysm sac and extravasation of enteric contrast into the aneurysm sac is diagnostic for aortoenteric fistula. However, enteric contrast is not recommended for routine evaluation of aortoenteric because the aforementioned finding is extremely rare. More common imaging findings include bowel loop appearing adherent to aneurysm sac with associated inflammatory stranding and foci or ectopic gas within the aneurysm sac or interposed between the bowel and aneurysm sac. Here we present a case of 52-year-old male who presents with incidental primary aortoenteric fistula.

Acute aortic syndromes comprise a heterogeneous group of life-threatening disorders, characterized by acute-onset chest or back pain with or without cardiovascular collapse. The major entities include acute aortic dissection, penetrating atherosclerotic ulcer, and intramural hematoma. Spontaneous rupture of the aorta is an additional, rare cause of this syndrome, which frequently leads to sudden cardiac death. This is a report of two cases of this uncommon entity with detailed pathological analysis.

Aortic dissection is an emergent medical condition, generally affecting the elderly, characterized by a separation of the aortic wall layers and subsequent creation of a pseudolumen that may compress the true aortic lumen. Predisposing factors mediate their risk by either increasing tension on the wall or by causing structural degeneration. They include hypertension, atherosclerosis, and a number of connective tissue diseases. If it goes undetected, aortic dissection carries a significant mortality risk; therefore, a high degree of clinical suspicion and a prompt diagnosis are required to maximize survival chances. Imaging methods, most commonly a CT scan, are essential for diagnosis; however, several studies have also investigated the effect of several biomarkers to aid in the detection of the condition. The choice of intervention varies depending on the type of dissection, with open surgical repair remaining of choice in those with type. In dissections, however, the role of conventional open surgery has considerably diminished in complicated type B dissections, with endovascular repair, a much less invasive technique, proving to be more effective. In uncomplicated type B dissections, where medical choice reigned supreme as the optimal intervention, endovascular repair is being explored as a viable option which may reduce long- term mortality outcomes, although the ideal intervention in this situation is far from settled.