Arterial aneurysm and dissection: toward the evolving phenotype of Tatton-Brown-Rahman syndrome.

BACKGROUND: Tatton-Brown-Rahman syndrome (TBRS) is a rare disorder, caused by DNMT3A heterozygous pathogenic variants, and first described in 2014. TBRS is characterised by overgrowth, intellectual disability, facial dysmorphism, hypotonia and musculoskeletal features, as well as neurological and psychiatric features. Cardiac manifestations have also been reported, mainly congenital malformations such as atrial septal defect, ventricular septal defect and cardiac valvular disease. Aortic dilatation has rarely been described. METHODS: Here we have undertaken a detailed clinical and molecular description of eight previously unreported individuals, who had TBRS and arterial dilatation and/or dissection, mainly thoracic aortic aneurysm (TAA). We have also reviewed the seven previously published cases of TAA in individuals with TBRS to try to better delineate the vascular phenotype and to determine specific follow-up for this condition. RESULTS: We include eight new patients with TBRS who presented with arterial aneurysms mainly involving aorta. Three of these patients presented with dissection that required critical surgery. CONCLUSIONS: Arterial aneurysms and dissections are a potentially lethal, age-dependent manifestation. The prevalence of aortic disease in individuals with TBRS is far in excess of that expected in the general population. This cohort, together with individuals previously published, illustrates the importance to consider dilatation/dissection, mainly in aorta but also in other arteries. Arterial vascular weakness may therefore also be a cardinal feature of TBRS and vascular surveillance is recommended.

Incisional Negative Pressure Wound Therapy After Revascularisation Surgery in Patients with Peripheral Arterial Disease: A Randomised Trial (PICO-Vasc Study).

OBJECTIVE: This study assessed the potential benefits of using incisional negative pressure wound therapy (iNPWT) for patients undergoing revascularisation due to peripheral arterial disease. METHODS: A prospective randomised controlled trial was conducted to compare the inguinal application of iNPWT vs. standard surgical dressings. Patients were enrolled from February 2021 to November 2022. A total of 133 groin incisions were randomised (66 intervention group, 67 control group). The randomisation sequence was carried out by permuted blocks and allocation assigned by opening opaque envelopes once the revascularisation procedure had finished. Wound healing and complication rates were assessed at post-operative days 5, 14, and 30. Primary and secondary endpoints were: 30 day post-operative surgical site infection (SSI) and surgical site occurrence (SSO) rates, defined as a surgical wound complication other than a SSI. Post-operative SSI was defined according to the US Centers for Disease Control and Prevention criteria. SSO included: wound dehiscence, seroma or lymphocele, haematoma, and lymphorrhagia. The study was registered at ClinicalTrials.gov database (NCT04840576) and reported according to the CONSORT guidelines. RESULTS: iNPWT did not modify the 30 day inguinal SSI and SSO rates (16.7% vs. 20.9% and 37.9% vs. 44.8%; p = .53, relative risk [RR] 0.999, 95% confidence interval [CI] 0.52 - 1.88 and p = .42, RR 1.29, 95% CI 0.89 - 1.86, respectively). It reduced the early SSO rate (19.7% vs. 35.8%; p = .044, RR 1.45, 95% CI 1.047 - 2.013) and post-operative seroma rate (4.6% vs. 19.4%; p = .014, RR 1.73, 95% CI 1.296 - 2.397). CONCLUSION: There were no differences in SSI and SSO rates, although statistically significant reductions in early SSO rates and seroma were found in the intervention group.

Multicenter Comparative Analysis of Late Open Conversion in Patients With Adherence and Nonadherence to Instructions for Use Endovascular Aneurysm Repair.

PURPOSE: The widespread adoption of endovascular aneurysm repair (EVAR) as preferred treatment modality for abdominal aortic aneurysm (AAA) has enlarged the number of patients needing open surgical conversion (OSC). The relationship between adherence to Instructions For Use (IFU) and EVAR long-term outcomes remains controversial. The aim of this study is to compare preoperative differences and postoperative outcomes between EVAR patients not adjusted to IFU and adjusted to IFU who underwent OSC. METHODS: This multicenter retrospective study reviewed 33 explanted EVARs between January 2003 and December 2019 at 14 Vascular Units. Patients were included if OSC occurred >30 days after implantation and excluded if explantation was performed to treat an endograft infection, aortic dissection, or traumatic transections. Variables analyzed included baseline characteristics, adherence to IFU, implant and explant procedural details, secondary reinterventions, and postoperative outcomes. RESULTS: Fifteen explanted patients (15/33, 45.5%) were identified not accomplished to IFU (out-IFU) at initial EVAR vs 18 explanted patients adjusted (in-IFU). During follow-up, a mean of 1.73±1.2 secondary reinterventions were performed, with more type I endoleaks treated in the subgroup out-IFU: 16.7% vs 6.3% in-IFU patients and more type III endoleaks (8.3% vs 0%). Patients out-IFU had shorter mean interval from implant to explant: 47.60±28.8 months vs 71.17±48. Type II endoleak was the most frequent indication for explantation. Low-flow endoleaks (types II, IV, V) account for 44% of indications for OSC in subgroup of patients in-IFU, compared with 13.3% in patients out-IFU and high-flow endoleaks (types I and III) were the main indication for patients out-IFU (33.3% vs 16.7% in-IFU). Total endograft explantation was performed in 57.5% of cases (19/33) and more suprarenal clamping was required in the subgroup out-IFU. Overall, 30-day mortality rate was 12.1% (4/33): 20% for patients out-IFU and 5.6% in-IFU. CONCLUSIONS: In our experience, type II endoleak is the most common indication for conversion and differences have been found between patients treated outside IFU with explantation taking place earlier during follow-up, mainly due to high-flow endoleaks and with higher mortality in comparison with patients adjusted to IFU. Ongoing research is required to delve into these differences.

National Diagnostic Reference Levels for Endovascular Aneurysm Repair and Optimisation Strategies.

OBJECTIVE: The International Commission on Radiological Protection (ICRP) has highlighted the large number of medical specialties using fluoroscopy outside imaging departments without programmes of radiation protection (RP) for patients and staff. Vascular surgery is one of these specialties and endovascular aneurysm repair (EVAR) is one of the most challenging procedures requiring RP guidance and optimisation actions. The recent European Directive on Basic Safety Standards requires the use and regular update of diagnostic reference levels (DRL) for interventional procedures. The objective of the study was to know the doses of patients undergoing EVAR with mobile Xray systems and with hybrid rooms (fixed Xray systems), to obtain national DRLs and suggest optimisation actions. METHODS: The Spanish Chapter of Endovascular Surgery launched a national survey that involved hospitals for 10 autonomous communities representing the 77% of the Spanish population (46.7 million inhabitants). Patient dose values from mobile Xray systems were available from nine hospitals (sample of 165 EVAR procedures) and data from hybrid rooms, from seven hospitals, with dosimetric data from 123 procedures. The initial national DRLs have been obtained, as the third quartile of the median values from the different centres involved in the survey. RESULTS: The proposed national DRLs are 278 Gy cm2 for hybrid rooms and 87 Gy cm2 for mobile Xray systems, and for cumulative air kerma (cumulative AK) at the patient entrance reference point, 1403 mGy for hybrid rooms, and 292 mGy for mobile systems. CONCLUSION: An audit of patient doses for EVAR procedures to identify optimised imaging protocol strategies is needed. It is also appropriate to evaluate the diagnostic information required for EVAR procedures. The increase by a factor of 3.2 (for kerma area product) and 4.8 (for cumulative AK) in the DRLs needs to be justified when the procedures are performed in the hybrid rooms rather than with mobile Xray systems.

One- and two-stage surgical revision of peri-prosthetic joint infection of the hip: a pooled individual participant data analysis of 44 cohort studies.

One-stage and two-stage revision strategies are the two main options for treating established chronic peri-prosthetic joint infection (PJI) of the hip; however, there is uncertainty regarding which is the best treatment option. We aimed to compare the risk of re-infection between the two revision strategies using pooled individual participant data (IPD). Observational cohort studies with PJI of the hip treated exclusively by one- or two-stage revision and reporting re-infection outcomes were retrieved by searching MEDLINE, EMBASE, Web of Science, The Cochrane Library, and the WHO International Clinical Trials Registry Platform; as well as email contact with investigators. We analysed IPD of 1856 participants with PJI of the hip from 44 cohorts across four continents. The primary outcome was re-infection (recurrence of infection by the same organism(s) and/or re-infection with a new organism(s)). Hazard ratios (HRs) for re-infection were calculated using Cox proportional frailty hazards models. After a median follow-up of 3.7 years, 222 re-infections were recorded. Re-infection rates per 1000 person-years of follow-up were 16.8 (95% CI 13.6-20.7) and 32.3 (95% CI 27.3-38.3) for one-stage and two-stage strategies respectively. The age- and sex-adjusted HR of re-infection for two-stage revision was 1.70 (0.58-5.00) when compared with one-stage revision. The association remained consistently absent after further adjustment for potential confounders. The HRs did not vary importantly in clinically relevant subgroups. Analysis of pooled individual patient data suggest that a one-stage revision strategy may be as effective as a two-stage revision strategy in treating PJI of the hip.

Could Preoperative Neck Anatomy Influence Follow-up of EVAR?

BACKGROUND: The aim of the study was to assess the clinical utility of strict CT scan surveillance after endovascular abdominal aneurysm repair (EVAR) and evaluate whether the anatomy of abdominal aortic aneurysm (AAA) neck (favorable/hostile) influences regular imaging control. METHODS: A retrospective study of AAA patients who underwent EVAR with aortobi-iliac endoprostheses during 2006-2013 was conducted. Exclusion criteria included other types of devices. Variables analyzed were technical and clinical success, morbimortality, complications (such as endoleaks, sac enlargement), reinterventions, reintervention-free survival, and survival rate. Preoperative CT scans were performed and repeated at 1, 6 (in selective cases), 12, and 24 months postoperatively. Patients were divided into two groups according to preoperative anatomic characteristics: group I (favorable neck) and group II (hostile neck: angle > 60°, length < 15 mm, diameter > 28 mm, and calcification or circumference thrombus ≥50%). RESULTS: A total of 127 patients with AAA (96.8% male) were included in the study. The mean age of the patients was 75.9 years (range: 51-90 years). The mean AAA diameter was 62.1 mm. Hostile neck was found in 52 patients (40.9%). The technical and clinical success rate was 100% and 30-day mortality was 0.8%. The reintervention-free survival rate was 97.6%, 96.1%, and 93.7% and the survival rate was 97.6%, 96.9%, and 91.3%, during follow-up at 6, 12, and 24 months, respectively. Accumulated complications in proximal sealing occurred in 0%, 0%, and 1.6% in group I and 1.9%, 6.1%, and 7.7% in group II at 1, 12, and 24 months, respectively. Type II endoleaks occurred in 24.3%, 14.3%, and 11.4% in group I and 9.8%, 6.3%, and 6.8% in group II at 1, 12, and 24 months, respectively. No increased diameter was detected at 6 and 12 months. No differences were observed in reinterventions and mortality rate depending on anatomy. CONCLUSIONS: CT scans performed at 6 and 12 months postoperatively did not detect complications or need for reintervention in patients with favorable necks, even in the presence of endoleaks type II, and could therefore be omitted. Hostile necks may compromise proximal sealing and require regular imaging follow-ups.

A randomized controlled noninferiority trial comparing radiofrequency with stripping and conservative hemodynamic cure for venous insufficiency technique for insufficiency of the great saphenous vein.

OBJECTIVE: The quality of available evidence regarding new minimally invasive techniques to abolish great saphenous vein reflux is moderate. The present study assessed whether radiofrequency ablation (RFA) was noninferior to high ligation and stripping (HLS) and conservative hemodynamic cure for venous insufficiency (CHIVA) for clinical and ultrasound recurrence at 2 years in patients with primary varicose veins (VVs) due to great saphenous vein (GSV) insufficiency. METHODS: We performed a randomized, single-center, open-label, controlled, noninferiority trial to compare RFA and 2 surgical techniques for the treatment of primary VVs due to GSV insufficiency. The noninferiority margin was set at 15% for absolute differences. Patients aged >18 years with primary VVs and GSV incompetence, with or without clinical symptoms, C2 to C6 CEAP (Clinical, Etiologic, Anatomic, Pathophysiologic) clinical class, and GSV diameter >4 mm were randomized with a 1:1:1 ratio to RFA, HLS, or CHIVA. The rate of clinical recurrence at 24 months was the primary endpoint and was analyzed using a delta noninferiority margin of 15%. Ultrasound recurrence, safety, and quality of life were secondary endpoints. RESULTS: From December 2012 to June 2015, 225 limbs had been randomized to RFA, HLS, or CHIVA (n = 74, n = 75, and n = 76). Clinical follow-up and Doppler ultrasound examinations were performed at 1 week and 1, 6, 12, and 24 months postoperatively. No differences in postoperative complications or pain were observed among the three groups. RFA was noninferior to HLS and CHIVA for clinical recurrence at 24 months, with an estimated difference in recurrence of 3% (95% confidence interval [CI], -4.8% to 10.7%; noninferiority P = .002) and -7% (95% CI, -17% to 3%; P < .001), respectively. For ultrasound recurrence, RFA was noninferior to CHIVA, with an estimated difference of -34% (95% CI, -47% to -20%; noninferiority P < .001) at 24 months. However, noninferiority could not be demonstrated compared with HLS (5.9%; 95% CI, -4.1 to 15.9; P = .073). No differences were found in quality of life among the three groups. CONCLUSIONS: RFA was shown to be noninferior in terms of clinical recurrence to HLS and CHIVA in the treatment of VVs due to GSV insufficiency.

Registry of Valvular and Vascular Allograft Transplants in the Autonomous Community of Catalonia, Spain (ReVAC): Design of the Registry.

BACKGROUND: In recent years, there have been significant changes in the perception of valvular and vascular allograft transplants. Despite the constant evolution of the field of transplant and the involvement of multiple surgical specialties, there is not an official registry for administrative and clinical control. This study aims to design a registry of vascular and valvular allograft transplantation in Catalonia, Spain (ReVAC). MATERIALS AND METHODS: Three consecutive focal groups were designed. Focal groups established administrative, technical, and clinical requirements of ReVAC. ReVAC included patients with a transplanted cryopreserved vascular segment or cardiac valves that were distributed by Catalan tissue banks to public and private hospitals in Catalonia. Ten hospitals were involved in this study. Data were collected on 380 patients between January 1995 and November 2015. RESULTS: The project resulted in the completion of a growing platform available online. ReVAC was divided into the following 3 levels: patient-related, surgery-related, and transplant-related data. Online access is available through the website of applications of the "Generalitat de Catalunya." CONCLUSION: ReVAC has been useful for administrative and clinical control of transplants in Catalonia. Currently, data related to outcomes of arterial allografts are available, reinforcing the current guideline-supported indications, as well as opening a window for further analysis and guideline creation.