Investigating the Link between Alpha-1 Antitrypsin Deficiency and Abdominal Aortic Aneurysms.

BACKGROUND: Alpha-1-Antitrypsin (AAT) is one of the major plasmatic protease inhibitors. In the last decade, an association between Alpha-1-Antitrypsin Deficiency (AATD) and Abdominal Aortic Aneurysms (AAA) has been hypothesized. Multiple factors may be involved in AAA's etiopathogenesis, and an underlying structural defect of the extracellular matrix (ECM) is always present. AATD could be a reasonable risk factor for AAA because it is related to protease/antiprotease imbalance and enhanced ECM degradation of the vessel wall. METHODS: We performed genotyping of 138 patients hospitalized in the Vascular Surgery Division of the ASST-Spedali Civili di Brescia, Italy, for nontraumatic rupture of AAA. The second purpose was to observe the distribution of main nongenetic risk factors for AAA between patients with and without AATD. RESULTS: Out of 138 patients, 22 were found with AATD: 16 MS, 1 SS, 3 MZ, and 2 with a new rare AAT variant. When compared to the general Italian population, our cohort's frequency of deficient S allele was significantly higher (7.8 vs. 2.2% respectively, P < 0.01), whereas the deficient Z allele was similar (1.1 vs. 1.3% respectively, P > 0.05). Although we found no differences in age, gender, hypertension, diabetes, and smoke habits between AAA patients with and without AATD, hyperlipidemia was significantly less frequent in patients with AATD (46.4 vs. 12.5% respectively, P < 0.05). CONCLUSIONS: In our AAA patients' cohort, the S allele frequency was higher than in the general Italian population. Our results support the hypothesis that AATD might be a risk factor for AAA.

The role of emergency presentation and revascularization in aneurysms of the peripancreatic arteries secondary to celiac trunk or superior mesenteric artery occlusion.

OBJECTIVE: The goal of this study was to analyze our 10-year experience in the treatment of aneurysms of the collateral circulation secondary to steno-occlusions of the celiac trunk (CT) or superior mesenteric artery (SMA). METHODS: In the last 10 years, 32 celiac-mesenteric aneurysms were detected (25 true aneurysms and seven pseudoaneurysms) in 25 patients with steno-occlusion of the CT or SMA. All cases were diagnosed and treated at our center, with either surgical or endovascular approach. As open surgery, we performed aneurysmectomy and revascularization; as endovascular treatment we performed both the embolization (or graft exclusion) of the aneurysm sac, and embolization of afferent and efferent arteries. RESULTS: Sixteen patients (64%) underwent endovascular treatment, accounting for 66% of aneurysms (21/32). Six patients (24%) and seven associated aneurysms (22%) underwent open surgery. Three asymptomatic patients (12%), representing a total of four aneurysms (12%), were not treated. For endovascular procedures, the technical success rate was 90%, with a 56% clinical success rate. For open surgery, clinical and technical success were achieved in five patients (83%) and six procedures (86%), respectively. Sixty-eight percent of patients (17/25) were treated in an emergency setting, using either endovascular (88%) or open (12%) approaches. Although technical success was achieved in more than 85% of these procedures for both approaches, clinical success was reached less frequently among patients with an acute presentation (P = .041). Regardless of the type of treatment, CT or SMA revascularization during the first procedure did not show an increased rate of clinical success (P = .531). However, we reported four cases of visceral ischemia after an endovascular approach without revascularization, with three open surgical corrections required. The mean follow-up was 41 months (range, 0-136 months). CONCLUSIONS: Neither of the approaches described qualifies as a standard optimal choice. We suggest a tailored therapeutic approach based on the clinical condition at the time of diagnosis and specific vascular anatomy.

Sequential multiple visceral arteries dissections without aortic involvement.

Isolated dissection of visceral arteries without associated aortic pathology is very rare. Risk factors, etiology, and natural history of this pathology continue to be unclear, and the guidelines for clinical management remain to be defined. We present a case not described previously, with sequential dissections of the celiac trunk, superior mesenteric artery, and renal arteries without aortic involvement. The patient presented with severe back thoracic and abdominal pain and without evidence of peritonitis. An abdominal angio-CT scan showed dissection of the superior mesenteric artery (SMA), with partial thrombosis of the false lumen and subsequent stenosis of around 60%. Conservative treatment with anticoagulants seemed to be appropriate in the beginning, because the patient became asymptomatic spontaneously within a few hours and angio-CT showed dissection but no ischemic lesions. On day 10 after onset, however, he again indicated severe back thoracic and abdominal pain. Emergent CT was performed. The prior SMA dissection appeared to be worse due to increased size of the false lumen, followed by SMA stenosis (about 75‒80%). In addition, dissection of the celiac artery and both renal arteries could be seen. The patient underwent angiography and stenting of the main trunk of the SMA, with good clinical and radiologic outcome. Double oral antiplatelet therapy was then introduced. An angio-MRI scan 6 months later showed stability of the multiple dissections.

Long-term outcomes of endovascular aortic repair with flared iliac limb endografts in patients with abdominal aortic aneurysm and aneurysmal common iliac arteries.

BACKGROUND: The aim of this study was to evaluate the long-term outcomes of endovascular aneurysm repair with flared iliac limb grafts in patients with abdominal aortic aneurysm (AAA) and aneurysmal common iliac arteries (CIAs). METHODS: This is a multicenter, retrospective, observational cohort study that involves four tertiary referral hospitals between May 1, 2005, and April 30, 2019. Primary outcomes were freedom from aneurysm-related mortality (ARM), and freedom from iliac-related reintervention. RESULTS: We studied 995 aneurysmal iliac limbs in 795 (85.2%) patients who met the inclusion criteria. Median AAA diameter was 55mm (IQR: 51-60). Early mortality occurred in 3 (0.4%) patients. The median of follow-up time was 52 months (IQR: 26-88). Estimated freedom from ARM was 99±0.002% (95% CI: 99-99.9) at 1 year, and 99±0.004% (95% CI: 97.9-99.6) at 5-years. Chronic obstructive pulmonary disease (HR=6.4, 95% CI: 1.7-24.0, P=0.006), chronic kidney disease (HR=5.5, 95% CI: 1.4-21.9, P=0.016), and the presence of an aneurysmal left CIA (HR=5.3, 95% CI: 1.0.5-27.4, P=0.044) was associated with ARM. There were 42 (7.3%) late iliac-related events (limb occlusion, N.=5; iliac-related endoleaks, N.=37). Estimated freedom from iliac-related reintervention was 98±0.003% (95% CI: 97-99) at 1 year, and 95±0.01% (95% CI: 92.7-96.7) at 5-years, which was associated with an aneurysmal right CIA (HR=2.2, 95% CI: 1.3-3.9; P=0.005), and age ≥78 years (HR=1.9, 95% CI: 1.01-1.3; P=0.039). CONCLUSIONS: EVAR flared iliac limb grafts showed a high rate of freedom from ARM and a low reintervention rate. Owing to these results, it can be a durable and stable alternative for patients aged >78 years.

Prediction of abdominal aortic aneurysm calcification by means of variation of high-sensitivity C-reactive protein.

OBJECTIVE: Abdominal aortic aneurysms are a major cause of death in developed countries, and thrombus and calcification of the aneurysm have been linked to increased complications. This study was conducted in order to identify the biochemical marker associated to the presence of intraluminal thrombus or calcification progression of the aneurysm. DESIGN: Several clinical laboratory parameters were measured in patients with abdominal aortic aneurysms, in particular those already demonstrated to be related to the pathology, such as lipoprotein (a), white blood cell count, fibrinogen and high-sensitivity C-reactive protein. Most of the patients were analysed for the presence of thrombus or aorta calcification using CT angiography. RESULTS: Unlike previous findings, we found no association between intraluminal thrombus formation and lipoprotein (a), but we evidenced that patients with lower grade of calcification tend to have higher plasma high-sensitivity C-reactive protein values compared with patients with a higher degree of calcification. Instead, no association was found with either white blood cell count or fibrinogen level. CONCLUSIONS: This study suggests that high-sensitivity C-reactive protein is a useful biomarker to assess the evolution of calcification and could be used in triaging patients to identify those who should undergo a rapid imaging, thus allowing prompt initiation of treatment or rule-out suspicious patients from non-essential imaging repetition.

Spinal cord ischemia after simultaneous and sequential treatment of multilevel aortic disease.

OBJECTIVES: The aim of the present study is to report a risk analysis for spinal cord injury in a recent cohort of patients with simultaneous and sequential treatment of multilevel aortic disease. METHODS: We performed a multicenter study with a retrospective data analysis. Simultaneous treatment refers to descending thoracic and infrarenal aortic lesions treated during the same operation, and sequential treatment refers to separate operations. All descending replacements were managed with endovascular repair. RESULTS: Of 4320 patients, multilevel aortic disease was detected in 77 (1.8%). Simultaneous repair was performed in 32 patients (41.5%), and a sequential repair was performed in 45 patients (58.4%). Postoperative spinal cord injury developed in 6 patients (7.8%). At multivariable analysis, the distance of the distal aortic neck from the celiac trunk was the only independent predictor of postoperative spinal cord injury (odds ratio, 0.75; 95% confidence interval, 0.56-0.99; P=.046); open surgical repair of the abdominal aortic disease was associated with a higher risk of spinal cord injury but did not reach statistical significance (odds ratio, 0.16; 95% confidence interval, 0.02-1.06; P=.057). Actuarial survival estimates at 1, 2, and 5 years after the procedure were 80%±5%, 68%±6%, and 63%±7%, respectively. Spinal cord injury did not impair survival (P=.885). CONCLUSIONS: In our experience, the risk of spinal cord injury is still substantial at 8% in patients with multilevel aortic disease. The distance of the distal landing zone from the celiac trunk is a significant predictor of spinal cord ischemia.

Lesson learned from early and long-term results of 327 cases of coexisting surgical abdominal diseases and aortic aneurysms treated in open and endovascular surgery.

Patients with abdominal aortic aneurysm (AAA) frequently have other abdominal pathologies of surgical interest (other diseases, OD). Out of 1,375 elective open aortic replacements for AAA, 315 cases with OD were subdivided in Group 1 (82 patients with "clean wound" OD) and Group 2 (233 patients with "clean-contaminated wound" OD). The results of the sub-groups in which OD was treated at the same time as AAA were analysed (1a, 66 cases and 2a, 86 cases) and compared with OD not treated at the same time as AAA (1b, 16 cases and 2b, 147 cases). EVAR was done in 12 patients with a infrarenal AAA and concomitant abdominal disease. In this group post-operative complications occurred in two patients (endoleaks) and no sign of endograft infection was developed. Mean follow-up was 36 months. Mortality was 0% in Group 1a, 1b, 2b and 5.8% in Group 2a. In Group 1a there were one haemoperitoneum, one ischaemic colitis and one graft infection. In Group 1b there were 4 nefrectomies for renal carcinoma and three emergency hernia repairs within 18 months from AAA operation. In Group 2a the follow-up was uneventful. In Group 2b there was no acute complication of OD and 57.2% of patients were subsequently operated for OD. In the EVAR group the 30-day and late mortality rates were 0 and 25%, respectively and all deaths were cancer-related. Contemporary correction of OD in open surgery for AAA should be performed in clean wound cases, while clean-contaminated operations can be done only in selected cases. EVAR is a valid alternative technique to open vascular surgery for the concomitant treatment of aortic aneurysms and abdominal pathologies.