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The extracellular matrix is a complex network of proteins and other molecules that are essential for the support, integrity, and structure of cells and tissues within the human body. The genes ZNF469 and PRDM5 each produce extracellular-matrix-related proteins that, when mutated, have been shown to result in the development of brittle cornea syndrome. This dysfunction results from aberrant protein function resulting in extracellular matrix disruption. Our group recently identified and published the first known associations between variants in these genes and aortic/arterial aneurysms and dissection diseases. This paper delineates the proposed effects of mutated ZNF469 and PRDM5 on various essential extracellular matrix components, including various collagens, TGF-B, clusterin, thrombospondin, and HAPLN-1, and reviews our recent reports associating single-nucleotide variants to these genes' development of aneurysmal and dissection diseases. [ABSTRACT FROM AUTHOR]

Purpose: After endovascular aneurysm repair (EVAR), an increased [18F]FDG uptake may be observed at PET/CT, being common to both vascular graft/endograft infection (VGEI) and sterile post-surgical inflammation. Increased non-specific metabolic activity, due to foreign body reaction, can persist for several years after surgery, thus complicating the interpretation of PET/CT studies. In this paper, we aimed to assess [18F]FDG distribution at different time-points after the implant of abdominal Endurant® endografts in patients without suspicion of infection. Methods: We retrospectively evaluated [18F]FDG/CT in 16 oncological patients who underwent abdominal aortic aneurysm exclusion with Endurant® grafts. Patients had no clinical suspicion of infection and were followed up for at least 24 months after scan. [18F]FDG PET/CT scans were interpreted using both visual and semi-quantitative analyses. Results: The time between the EVAR procedure and [18F]FDG PET/CT ranged between 1 and 36 months. All grafts showed mild and diffuse [18F]FDG uptake without a focal pattern. Mean values of SUVmax were 2.63 ± 0.48 (95% CI 2.38–2.88); for SUVmean 1.90 ± 0.33 (95% CI 1.72–2.08); for T/B ratios 1.43 ± 0.41 (95% CI 1.21–1.65). SUVmax and SUVmean were not correlated to the time elapsed from the procedure, but we observed a declining trend in T/B ratio over time. Conclusions: Endovascular implant of Endurant® grafts does not cause a significant inflammatory reaction. The evidence of faint and diffuse [18F]FDG uptake along the graft can reliably exclude an infection, even in early post-procedural phases. Therefore, in patients with a low probability of VGEI, [18F]FDG PET/CT can also be performed immediately after EVAR. [ABSTRACT FROM AUTHOR]

The advent and refinement of complex endovascular techniques in the last two decades has revolutionized the field of vascular surgery. This has allowed an effective minimally invasive treatment of extensive disease involving the pararenal and the thoracoabdominal aorta. Fenestrated-branched EVAR (F/BEVAR) now represents a feasible technical solution to address these complex diseases, moving the proximal sealing zone above the renal-visceral vessels take-off and preserving their patency. The aim of this paper was to provide a narrative review on the peri-operative management of patients undergoing F/BEVAR procedures for juxtarenal abdominal aortic aneurysm (JAAA), pararenal abdominal aortic aneurysm (PRAA) or thoracoabdominal aortic aneurism (TAAA). It will focus on how to prevent, diagnose, and manage the complications ensuing from these complex interventions, in order to improve clinical outcomes. Indeed, F/BEVAR remains a technically, physiologically, and mentally demanding procedure. Intraoperative adverse events often require prolonged or additional procedures and complications may significantly impact a patient's quality of life, health status, and overall cost of care. The presence of standardized preoperative, perioperative, and postoperative pathways of care, together with surgeons and teams with significant experience in aortic surgery, should be considered as crucial points to improve clinical outcomes. Aggressive prevention, prompt diagnosis and timely rescue of any major adverse events following the procedure remain paramount clinical needs. [ABSTRACT FROM AUTHOR]

The human aorta is a high-risk area for vascular diseases, which are commonly restored by thoracic endovascular aortic repair. In this paper, we report a promising shear-activated targeted nanoparticle drug delivery strategy to assist in the treatment of coarctation of the aorta and aortic aneurysm. Idealized three-dimensional geometric models of coarctation of the aorta and aortic aneurysm are designed, respectively. The unique hemodynamic environment of the diseased aorta is used to improve nanoparticle drug delivery. Micro-carriers with nanoparticle drugs would be targeting activated to release nanoparticle drugs by local abnormal shear stress rate (SSR). Coarctation of the aorta provides a high SSR hemodynamic environment, while the aortic aneurysm is exposed to low SSR. We propose a method to calculate the SSR thresholds for the diseased aorta. Results show that the upstream near-wall area of the diseased location is an ideal injection location for the micro-carriers, which could be activated by the abnormal SSR. Released nanoparticle drugs would be successfully targeted delivered to the aortic diseased wall. Besides, the high diffusivity of the micro-carriers and nanoparticle drugs has a significant impact on the surface drug concentrations of the diseased aortic walls, especially for aortic aneurysms. This study preliminary demonstrates the feasibility of shear-activated targeted nanoparticle drug delivery in the treatment of aortic diseases and provides a theoretical basis for developing the drug delivery system and novel therapy. [ABSTRACT FROM AUTHOR]

As the global population ages, the issue of abdominal aortic aneurysm continues to grow. With the evolution of new devices and refined operative technique, aneurysm treatment via endovascular aortic repair is becoming increasingly favourable. This, however, is not without drawbacks, where regular surveillance is paramount to long‐term success and detection of post‐procedure complications. Of these complications, endoleak is the most notable and poses the greatest risk of potential future aortic rupture. The purpose of this review paper is to discuss the armada of imaging modalities used in the detection and evaluation of endoleak and their varying usefulness. Plain abdominal X‐ray is a cost‐effective tool in detecting gross graft abnormalities such as stent migration or deformity (kinking or fracture). Though it may raise suspicion for endoleak, X‐ray does not allow accurate classification of endoleak type when used alone. Duplex ultrasonography quantifies both aortic anatomy and real time flow dynamics. Most screening programmes are conducted using two‐dimensional ultrasound. Unfortunately, observer and equipment variability may lead to surveillance discrepancies—but reduced when utilising a dedicated vascular sonography laboratory. Contrast enhanced ultrasonography is a promising alternative to computed tomography, though still is emerging. Computed tomography angiography certainly has disadvantages (ionising radiation, contrast‐nephropathy, limited differentiation of endoleak type)—however, it provides near‐real surgical dimensions and highlights graft complications and concomitant disease (such as neighbouring infection). With widespread availability and short scan time, it certainly remains valuable in surveillance. Magnetic resonance angiography has a similar sensitivity to computed tomography (minus the radiation), however is plagued by movement and metal artefact. Other novel modalities in endoleak surveillance include four‐dimensional ultrasound, multiplanar intra‐operative probes, nuclear medicine and wall stress analysis. [ABSTRACT FROM AUTHOR]

Background: Shared decision-making (SDM) is known to improve quality of care. Particularly in vascular surgery treatment options are often preference-sensitive. Unfortunately, vascular surgeons infrequently apply SDM. Decision support tools (DSTs) have been shown to be helpful in SDM.Objective: This article describes the development process of three different DSTs to help vascular surgeons and patients apply SDM.Patient Involvement: Patients' information needs were obtained via focus group meetings. Fifty-two patients and eighteen vascular surgeons not involved in the development process evaluated the comprehensibility and usability of the DST-prototypes.Methods: A multidisciplinary steering group commissioned the development of the three DSTs according to international standards.Results: Digital decision aids and paper-based consultation cards and decision cards were developed for patients with an abdominal aortic aneurysm, carotid artery disease, intermittent claudication or varicose veins. Patients preferred the use of the decision aids followed by consultation cards, whereas vascular surgeons preferred to use decision cards followed by decision aids.Discussion: Decision aids, consultation cards and decision cards for four vascular diseases are now available to all vascular surgeons and patients in the Netherlands. The DSTs were well received by both surgeons and patients. English versions are also available. [ABSTRACT FROM AUTHOR]

The frozen elephant trunk technique for various thoracic aortic diseases is widely accepted to facilitate future downstream aortic surgery. However, in some cases, the descending aorta is unsuitable for cross-clamping due to progressive aneurysmal changes or dense adhesions to surrounding structures, and frozen elephant trunk retrieval becomes challenging. This paper presents a case of successful frozen elephant trunk retrieval by partial clamping of the descending aortic aneurysm without dissection of peri-aneurysmal adhesions, and subsequent encircling. [ABSTRACT FROM AUTHOR]

Inflammatory aortic diseases are broadly classified into three categories according to the degree of inflammation: atherosclerosis, atherosclerosis with excessive inflammation, and aortitis/periaortitis. This paper presents a case of a 39-year old man with aneurysmal dilatation of thoracic aorta and aortic valve insufficiency. The aortic wall showed thickening and wrinkled "tree bark" appearance as well as apparent scarring of the intima. Histological examination revealed intimal hyperplasia, a granulomatous/giant cell pattern in the inner tunica media, a few epithelioid macrophages, abundant chronic lymphoplasmacytic and histiocytic inflammation and discrete fibrinoid necrosis. The histological findings were indicative of Horton's disease, but no typical clinical features were present. The case illustrates the difficulties involved in diagnosing inflammatory aortic diseases where it may be challenging to arrive at a specific diagnosis despite the knowledge of medical history, and available macroscopic and histological findings. [ABSTRACT FROM AUTHOR]

A conference paper about the use of an endovascular approach in the treatment of patients with nontraumatic rupture of the thoracic aorta is presented. It summarizes 5 cases of patients who were given local anesthesia for ruptured thoracic aortic aneurysms (rTAA). These patients have a history of arterial hypertension or hypotension, back pain, coronary artery disease, anemia, bilateral blue-toe syndrome, coronary artery bypass grafting and abdominal aortic aneurysm (AAA).

Endovascular implantation of a stent graft in the abdominal aorta (endovascular aneurysm repair - EVAR) is a widely accepted alternative to open surgery in treatment of abdominal aortic aneurysms. Although EVAR is connected with a significant reduction in the risk of peri- and post-operative complications, it does not eliminate them totally. Long-term surveillance of post-EVAR patients is aimed at early detection of and fast reaction to a group of complications called endovascular leaks. Currently, the gold standard in leak diagnostics is computed tomography angiography (CTA). The other methods are ultrasonography, magnetic resonance (MR) angiography, intra-aneurysm sac pressure measurement, X-ray, and digital subtraction angiography (DSA). Despite many analyses based on long-term research, emphasising the high value and competitiveness of less invasive tests such as US or X-ray compared to CTA, it is still difficult for them to win the trust and acceptance of clinicians. The persisting view is that computed tomography is the test that finally resolves any inaccuracies. Consequently, a patient with a number of concurrent diseases is subject to absurdly high radiation exposure and effects of a radiocontrast agent within a short time. It is therefore logical to acknowledge that the EVAR-related risk is catching up with the open surgery risk, while the endovascular procedure is much more costly. Nevertheless, the status of CTA as the gold standard ultimately seems to be unthreatened. This paper presents a description of the diagnostic imaging tests that make it possible to detect any vascular leaks and to develop strategies for therapeutic processes. [ABSTRACT FROM AUTHOR]

A conference paper about the use of hybrid surgical techniques in patients with thoracoabdominal aortic aneurysm (TAAA). The techniques are successful in patients who underwent open TAAA repairs and for those who were treated for chronic aortic dissection. There are differences in applying the techniques depending on the condition of the arteries, the choice of models for starting the repairs and procedures for spinal cord protection.

Acute aortic dissection is a common disease caused by primary tear in the aortic intima or rupture of vasa vasorum. Every year 5-30 per million are affected by that. Untreated aortic dissection (dissecting aortic aneurysm) carries a high mortality rate. More than 90% of acute dissections present with acute pain in chest or back or both. Aortic dissection may mimic other more common conditions and high index of suspicion is required for prompt diagnosis and emergency treatment. In this paper, six patients with acute aortic dissection are presented who had atypical symptoms. Syncope, abdominal pain and diarrhea, dyspnea of exertion, severe weakness, faint and epigastric pain were chief presenting symptoms in these patients. Absence of severe chest pain has made the authors to present this study as case report. These cases signify the high clinical suspicion in diagnosis and treatment of aortic dissection. Furthermore, treatment of chronic hypertension is a strong modality in reducing the risks of aortic dissection. [ABSTRACT FROM AUTHOR]

Abstract: Image-based blood flow computation provides great promise for the evaluation of vascular devices and assessment of surgical procedures. However, many previous studies employ idealized arterial and device models or only patient-specific models from the image data after device deployment, since the tools for model construction are unavailable or limited and tedious to use. Moreover, in contrast to retrospective studies from existing data, there is a pressing need for prospective analysis with the goal of surgical planning. Therefore, it is necessary to construct models with deployed devices in a fast, virtual and interactive fashion. The goal of this paper is to develop new geometric methods to deploy stents or stent grafts virtually to patient-specific geometric models constructed from a 3D segmentation of medical images. A triangular surface representing the vessel lumen boundary is extracted from the segmentation. The diseased portion is either clipped and replaced by the surface of a deployed device or rerouted in the case of a bypass graft. For diseased arteries close to bifurcations, bifurcated device models are generated. A method to map a 2D strut pattern on the surface of a device is also presented. We demonstrate three applications of our methods in personalized surgical planning for aortic aneurysms, aortic coarctation, and coronary artery stenosis using blood flow computation. Our approach enables prospective model construction and may help to expand the throughput required by routine clinical uses in the future. [Copyright &y& Elsevier]

Background/Aim. Abdominal aorta aneurysm (AAA) represents a pathological enlargment of infrarenal portion of aorta for over 50% of its lumen. The only treatment of AAA is a surgical reconstruction of the affected segment. Until the late XX century, surgical reconstruction implied explicit, open repair (OR) of AAA, which was accompanied by a significant morbidity and mortality of the treated patients. Development of endovascular repair of (EVAR) AAA, especially in the last decade, offered another possibility of surgical reconstruction of AAA. The preliminary results of world studies show that complications of such a procedure, as well as morbidity and mortality of patients, are significantly lower than with OR of AAA. The aim of this paper was to present results of comparative clinical prospective study of early inflammatory response after reconstruction of AAA between endovascular and open, conventional surgical technique. Methods. A comparative clinical prospective study included 39 patients, electively operated on for AAA within the period of December 2008 -- February 2010, divided into two groups. The group I counted 21 (54%) of the patients, 58-87 years old (mean 74.3 years), who had been submited to EVAR by the use of excluder stent graft. The group II consisted of 18 (46%) of the patients, 49-82 (mean 66.8) years, operated on using OR technique. All of the treated patients in both groups had AAA larager than 50 mm. The study did not include patients who have been treated as urgent cases, due to the rupture or with simptomatic AAA. Clinical, biochemical and inflamatory parameters in early postoperative period were analyzed, in direct postoperative course (number of leucocytes, thrombocytes, serum circulating levels of cytokine - interleukine (IL)-2, IL-4, IL-6 and IL-10). Parameters were monitored on the zero, first, second, third and seventh postoperative days. The study was approved by the Ethics Commitee of the Military Medical Academy. Results. The study showed a statistically significantly shorter time of treatment in the EVAR group (average 90 min) compared to the OR group (average 136 min). Also, there was a statistically significantly less blood loss in the patients operated on by the use of EVAR surgery (average 60 mL) as compared to the patients treated with OR techinique (average 495 mL), as well as a shorter postoperative hospitalization of patients in the EVAR group (average 4 days) compared to the OR group (average 8 days). The OR group was detected with a statistically significant increase of leucocytes and statistically significant fall of the number of thrombocytes in comparison with the EVAR group in all the investigated terms. A significant concentration rise of IL-2 in the OR group and concentration rise of IL-6 in the EVAR group was shown 24 hours after the procedure, whereas on the second postoperative day there was detected a significant fall of IL-6 in the EVAR group. IL-4 concentration in the OR group was significantly higher as of the third postoperative day in comparison to the EVAR group. There was no significant difference in IL-10 concentration between the groups. Conclusion. The EVAR techinique is a safer and less invasive and less traumatic procedure for patients than the OR of AAA. Following the EVAR, there are less inflammatory reactions in the early postoperative periodas compared to the OR and therefore less possibility of the development of systemic inflammatory respons syndrome in patients treated. [ABSTRACT FROM AUTHOR]

Abstract: It is well-established that blood viscosity plays a significant role in the determination of the health of the individual. It has been reported that many cardiovascular diseases are associated with blood viscosity. In this paper, the dynamic behaviors of aortic aneurysm subject to physiological blood flow with normal and high viscosities are presented. Fluid–structure interaction (FSI) method was used in the computational simulation. The influence of blood viscosity on flow dynamics within the aneurysm sac, aneurismal diameter, cross sectional shape, wall axial displacement and wall shear stress (WSS) was studied in detail. This investigation uncovered the correlations between blood viscosity and the dynamic behaviors of aortic aneurysm, which have rarely been found in existing literatures. We believe that these findings may provide important implications for individualized endovascular treatment for patient with aortic aneurysm. [Copyright &y& Elsevier]

Approximately 6000 men die every year from ruptured abdominal aortic aneurysm in England and Wales. Randomised clinical trials and a large pilot study have shown that ultrasound screening of men aged 65 years can prevent about half of these deaths. However, there is a significant perioperative morbidity and mortality from interventions to repair the detected aneurysm. This paper explores the ethical issues of screening men for abdominal aortic aneurysm. It is concluded that a population screening programme for abdominal aortic aneurysm offers a clear balance of good over harm. It is therefore ethically justified, as long as men are given adequate information at every stage of the process. Each man has the right to be properly informed, regardless of whether he accepts the invitation to be screened and, if an aneurysm is detected, whether or not he accepts treatment. [ABSTRACT FROM AUTHOR]

Background. There have been isolated case reports of arterial disease in males with Alport syndrome (AS), a systemic disorder of Type IV collagen. In this paper, we describe five new cases of AS associated with significant aortic disease including dissection and aneurysm.Methods. We present brief clinical descriptions of five males with AS and aortic disease. We performed immunohistochemical analysis of the expression of the α5 chain of Type IV collagen in skin basement membranes from a previously reported family with AS and associated aortic disease and in the aortic media of male mice with X-linked Alport syndrome (XLAS) due to a nonsense mutation in the COL4A5 gene.Results. Three of the five patients exhibited aneurysm and dissection of the thoracic aorta, occurring at 25–32 years of age, while one had aortic dilatation and another had aortic insufficiency. All five men required renal replacement therapy by age 20. Immunohistochemistry of skin biopsy specimens in previously reported male siblings with aortic disease confirmed that they had XLAS. We further found that the α5 chain of Type IV collagen is abnormally absent from aortic media of transgenic mice with XLAS.Conclusions. Early onset aortic disease may be an unusual feature of AS. Screening of men with AS for aortic abnormalities may be clinically indicated in some families. [ABSTRACT FROM AUTHOR]

Abstract: In stress analysis of membrane-like biological structures, the geometry constructed from in vivo image, which often corresponds to a deformed state, is routinely taken as the initial stress-free geometry. In this paper, we show that this limitation can be completely removed using an inverse elastostatic approach, namely, a method for finding the initial geometry of an elastic body from a given deformed state. We demonstrate the utility of the inverse approach using a patient-specific abdominal aortic aneurysm model, and identify the scope of error in stress estimation in the conventional approach within a realistic range of material parameter variations. [Copyright &y& Elsevier]

Abstract: In this paper we present an approach to analyse the migration of stent grafts after endovascular aortic aneurysm repair. Stent graft migration and kinking or buckling of the graft can cause the perilous risk of rupture. Our approach allows describing the occurring movements quantitatively towards the aim to find common patterns related to different endograft devices or to dangerous changes in morphology. We proceed by defining the spinal canal as reference system and by investigating the movements of the graft relative to it. Here, we segment the spinal canal as well as the stent graft at different time points and we apply a point set registration algorithm in order to calculate the transformation to the spinal canal reference system and to determine the occurring motion of the stent. First results illustrate that essentially rigid motion occurs in case of thoracic aortic aneurysms. [Copyright &y& Elsevier]

Abstract: In the Australian Medical Journal of March 1883, there is a report entitled, “Valvular incompetency of pulmonary artery, with regurgitation”, presented by Dr. William Pegus. Dr. Pegus presented this paper about his own peculiar heart problem, which was different from any in the textbooks of that time, to a group of doctors in 1882. He described his symptoms, the progress of the condition and the treatment he received, the most helpful of which was the use of Turkish baths. There was discussion on the possible diagnosis but at the conclusion of the presentation it was felt that all the symptoms could not be explained and therefore the diagnosis was uncertain. In the light of present day knowledge, the clinical picture is typical of a rupture of aneurysm of a sinus of Valsalva into the right ventricle. Rupture of such an aneurysm was first described by Hope in 1839 in his book on diseases of the heart and great vessels, the diagnosis being made at autopsy. Dr. Pegus was probably the first person in Australia to publish a detailed description of the symptoms and clinical signs and to give an account of the progress of the condition with his own thoughts about the treatment available at the time. [Copyright &y& Elsevier]

Deep vein thrombosis and pulmonary embolism, aortic aneurysm and aortic dissection, as well as peripheral arterial atherosclerosis, are frequently diagnosed vascular disorders. In this paper, the authors present the case of coexistence of common pathologies of the cardiovascular system in a patient with pain in the right lower limb. The presented images provide a didactically valuable overview of serious cardiovascular pathologies. This article highlights the value of computed tomography angiography in diagnosis of cardiovascular life-threatening conditions, especially as a result of proper medical interview and physical examination. [ABSTRACT FROM AUTHOR]