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AbstractThe role of inflammation in the development of aortic aneurysms is emerging, along with the potential diagnostic and therapeutical potential of this correlation. Abdominal aorta aneurysms have a strong inflammatory substrate since atherosclerosis, which is undoubtedly linked to inflammation, is also a predisposing factor to their formation. Yet, data have emerged that the development of thoracic aorta aneurysms involves several inflammatory pathways, although they were previously referred to as a non-inflammatory disease. Since aortic aneurysms are mainly asymptomatic during their clinical course until their complications—which may be lethal—serum biomarkers for their early diagnosis are a necessity. Studies highlight that inflammation molecules may have a critical role in that direction. In addition, imaging techniques that trace aortic wall inflammation are developed in order to predict aneurysm growth rates and sites vulnerable of rupture. Several anti-inflammatory agents have been also studied in animal models and clinical trials for the treatment of aortic aneurysms. This review highlights the role of inflammation in pathogenesis, diagnosis and treatment of aortic aneurysms.

Background Although an association between elevated blood pressure and risk of aortic aneurysm is established, few studies have investigated the association with aortic aneurysm subtypes. We investigated the association between systolic and diastolic blood pressure and hypertension status with the risk of aortic aneurysm in the UK Biobank. Methods The analysis included 495,542 men and women aged 37–73 years at recruitment between 2006 and 2010. Aortic aneurysm cases were identified by linkage to hospitalization and mortality records. Multivariable Cox proportional hazards regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for the association between blood pressure and hypertension and risk of aortic aneurysm overall and for subtypes. Results During a mean follow-up of 12.3 years, 3,346 incident aortic aneurysm cases were identified. Hypertension vs. no hypertension was associated with increased risk (HR, 95% CI) of aortic aneurysm (1.17, 1.08–1.27), and for thoracic (1.23, 1.04–1.46), abdominal (1.16, 1.04–1.30), and non-ruptured (1.18, 1.08–1.28) aortic aneurysm, and suggestively with unspecified site aortic aneurysm (1.18, 0.96–1.46) and aortic aneurysm mortality (1.26, 0.87–1.82), but not ruptured aortic aneurysm (1.02, 0.67–1.58). Systolic blood pressure was not associated with risk of aortic aneurysm overall or for any subtype. Diastolic blood pressure was positively associated with aortic aneurysm (1.74, 1.26–2.41, p trend <0.0001) for ≥ 110 vs. <80 mmHg, abdominal aortic aneurysm (1.95, 1.28–2.96, p trend <0.0001), unspecified site aortic aneurysm (2.02, 0.94–4.33, p trend =0.005), non-ruptured aortic aneurysm (1.79, 1.29–2.47), and aortic aneurysm mortality (2.32, 0.56–9.58, p trend <0.0001), and with ruptured aortic aneurysm (2.48, 1.22–5.03, p trend <0.0001 for 100–109 vs. <80 mmHg), while the association with thoracic aortic aneurysm was less clear (1.30, 0.64–2.63). These associations were strengthened and positive associations emerged for systolic blood pressure and abdominal and non-ruptured aortic aneurysm in sensitivity analyses when excluding participants with prevalent ischemic heart disease, stroke, those using hypertension medications and the first 3 years of follow-up. Conclusion We found that hypertension status and higher diastolic blood pressure were associated with increased risk of aortic aneurysm overall and most aortic aneurysm subtypes. No association was observed for systolic blood pressure. Although further studies are needed on aortic aneurysm subtypes, these findings provide strong support that controlling blood pressure is important for reducing the risk of aortic aneurysm.

A new appraisal of the management of acute aortic dissection is timely because of recent developments in diagnostic strategies (including biomarkers and imaging), endograft design, and surgical treatment, which have led to a better understanding of the epidemiology, risk factors, and molecular nature of aortic dissection. Although open surgery is the main treatment for proximal aortic repair, use of endovascular management is now established for complicated distal dissection and distal arch repair, and has recently been discussed as a pre-emptive measure to avoid late complications by inducing aortic remodelling.

Heritable thoracic aortic aneurysms and dissections (hTAAD) are life-threatening complications of well-known syndromic diseases or underdiagnosed nonsyndromic heritable forms (nshTAAD). Both have an autosomal dominant transmission and are genetically heterogeneous. Our objective was to describe the relevance of molecular diagnosis in these patients and the contribution of each gene in nshTAAD. Two hundred twenty-six consecutive nshTAAD probands, either young (<45 years) sporadic or familial cases were included. A next-generation sequencing capture panel comprising 23 known disease-causing genes was performed. Class 4 or 5 variants were identified in 18% of the nshTAAD probands, while class 3 variants were found in 10% of them. The yield in familial cases was greater than in sporadic cases. SMAD3 and FBN1 genes were the major disease-causing genes. Unexpectedly, no premature termination codon variant was identified in the FBN1 gene. Furthermore, we report for the first time that aortic dissection or surgery occurred significantly more often and earlier in probands with a class 4 or 5 pathogenic variant. This study indicates that genetic screening using NGS is efficient in young and familial nshTAAD. The presence of a pathogenic variant has a possible predictive value, which needs to be further investigated because it may influence care.

Acute aortic dissection (AD) is a critical condition characterized by high mortality and frequent misdiagnoses, primarily due to symptom overlap with other medical pathologies. This study explores the diagnostic utility of ChatGPT 4.0, an artificial intelligence model developed by OpenAI, in identifying acute AD from patients’ presentations and general physical examination findings documented in published case reports. A systematic search was conducted on the PubMed database using the search term “acute aortic dissection,” applying filters for articles published within the past year and categorized as case reports. The primary symptoms and physical examination details from each case were inputted into ChatGPT 4.0, which was prompted to generate three differential diagnoses and one main provisional diagnosis based on the case presentation. The search yielded a total of 163 results, from which 10 case reports were randomly selected. The patient demographics across all 10 case reports demonstrated an age range of 29 to 82 years, with equal gender distribution (5 males, 5 females) and hypertension as the most prevalent baseline comorbidity. ChatGPT 4.0 accurately identified acute AD as one of the top three differential diagnoses in all selected cases and identified acute AD as the provisional diagnosis in five of the 10 cases. In conclusion, while ChatGPT 4.0 demonstrates potential in suggesting acute AD as a differential diagnosis based on clinical data, its role should be considered supportive rather than definitive. Based on our findings, it could serve as an early, cost-effective, and quick screening tool, helping physicians adopt a “think aorta” approach.

Background This article reports in detail a case of syphilitic aortic dissection (AD) with facial swelling as the initial symptom. Case presentation A 62-year-old male had a 2-week history of facial swelling. CT examination revealed ascending aortic aneurysm and thrombosis. Computed tomography angiography (CTA) revealed ascending AD aneurysm and multiple ulcers. Syphilis antibody positive. Blood oxygen saturation was 92%-96%. The patient underwent ascending aortic replacement, total arch replacement, and elephant stent implantation 2 days after admission. The superior vena cava pressure decreased from 65 to 19 mmHg after surgery. The patient was extubated 6 h after surgery and was active the next day. Blood oxygen saturation returned to normal, and facial swelling reduced. CTA at 1 week confirmed the surgery’s success. Conclusion This case underscores the rarity of syphilitic AD presenting as facial swelling. Timely recognition and individualized treatment strategies can achieve favorable outcomes.

Purpose Smooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle–dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management. Methods Medical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed. Results All patients had congenital mydriasis and related pupillary abnormalities at birth and presented in infancy with a patent ductus arteriosus or aortopulmonary window. Patients had cerebrovascular disease characterized by small vessel disease (hyperintense periventricular white matter lesions; 95%), intracranial artery stenosis (77%), ischemic strokes (27%), and seizures (18%). Twelve (36%) patients had thoracic aortic aneurysm repair or dissection at median age of 14 years and aortic disease was fully penetrant by the age of 25 years. Three (9%) patients had axillary artery aneurysms complicated by thromboembolic episodes. Nine patients died between the ages of 0.5 and 32 years due to aortic, pulmonary, or stroke complications, or unknown causes. Conclusion Based on these data, recommendations are provided for the surveillance and management of SMDS to help prevent early-onset life-threatening complications.

Objective This study aims to assess the performance of various scoring systems in predicting the 28-day mortality of patients with aortic aneurysms (AA) admitted to the intensive care unit (ICU). Methods We utilized data from the Medical Information Mart for Intensive Care-IV (MIMIC-IV) to perform a comparative analysis of various predictive systems, including the Glasgow Aneurysm Score (GAS), Simplified Acute Physiology Score (SAPS) III, SAPS II, Logical Organ Dysfunction System (LODS), Sequential Organ Failure Assessment (SOFA), Systemic Inflammatory Response Syndrome (SIRS), and The Oxford Acute Illness Severity Score (OASIS). The discrimination abilities of these systems were compared using the area under the receiver operating characteristic curve (AUROC). Additionally, a 4-knotted restricted cubic spline regression was employed to evaluate the association between the different scoring systems and the risk of 28-day mortality. Finally, we conducted a subgroup analysis focusing on patients with abdominal aortic aneurysms (AAA). Results This study enrolled 586 patients with AA (68.39% male). Among them, 26 patients (4.4%) died within 28 days. Comparative analysis revealed higher SAPS II, SAPS III, SOFA, LODS, OASIS, and SIRS scores in the deceased group, while no statistically significant difference was observed in GAS scores between the survivor and deceased groups ( P  = 0.148). The SAPS III system exhibited superior predictive value for the 28-day mortality rate (AUROC 0.805) compared to the LODS system (AUROC 0.771), SOFA (AUROC 0.757), SAPS II (AUROC 0.759), OASIS (AUROC 0.742), SIRS (AUROC 0.638), and GAS (AUROC 0.586) systems. The results of the univariate and multivariate logistic analyses showed that SAPS III was statistically significant for both 28-day and 1-year mortality. Subgroup analyses yielded results consistent with the overall findings. No nonlinear relationship was identified between these scoring systems and 28-day all-cause mortality (P for nonlinear > 0.05). Conclusion The SAPS III system demonstrated superior discriminatory ability for both 28-day and 1-year mortality compared to the GAS, SAPS II SIRS, SOFA, and OASIS systems among patients with AA.

A male patient in his 50s presented to the emergency department with a three-day history of shortness of breath, New York Heart Association class IV, and oxygen desaturation. His physical examination revealed a large volume radial pulse with bibasal crepitation in the lungs and a soft diastolic murmur in the aortic area on auscultation of his heart. He was managed on the line of decompensated heart failure. Transthoracic echocardiography showed a dissection flap in the ascending aorta with acute severe aortic regurgitation. A subsequent urgent CT angiography of the whole aorta confirmed a complex type A aortic dissection with an aneurysmal ascending aorta. An emergency type A aortic dissection repair (modified Bentall’s procedure) was done. The patient made a good recovery, was discharged successfully 2 weeks after the procedure and was doing well on postoperative follow-up.