Late diagnosis of Marfan syndrome is associated with unplanned aortic surgery and cardiovascular death.

BACKGROUND: Marfan syndrome (MFS) guidelines recommend optimal pharmacological therapy (OPT) and replacement of the ascending aorta (RAA) at 5.0cm diameters to prevent acute type A aortic dissection (ATAAD) and death. The effect of early MFS diagnosis and initiation of therapy on outcomes is not known. OBJECTIVE: To evaluate the effect of age at MFS diagnosis and therapy initiation on delayed RAA and death. METHODS: This retrospective observational cohort study with long-term follow-up included consecutive patients with MFS, pathogenic FBN1 variant, and regular visits to a European Reference Network Center. We considered MFS diagnosis at age ≥21 years late, and OPT initiation at age <21 years early. Outcomes were delayed RAA with aneurysm diameter >5.0cm or ATAAD, and death from all causes. We used landmark design starting at age 21 years to determine associations with outcomes. RESULTS: The study group consisted of 288 patients (45.1% male), including 169 patients with late MFS diagnosis (58.7%) and 63 with early OPT (21.9%). During mean follow-up of 25±14.7 years, 78 patients had delayed RAA, with 42 operations for ATAAD and 36 for aneurysms ≥5.0cm. There were 33 deaths, including 11 deaths late after ATAAD. All deaths were cardiovascular. Late diagnosis, but not early OPT, showed univariate association with delayed RAA (P<0.001) and death (P=0.025). Multivariate Cox regression analysis confirmed late diagnosis as predictor of delayed RAA (hazard ratio (HR)=8.01; 95% confidence interval (95%CI) 2.52-25.45; P<0.001) and death (HR=4.68; 95%CI 1.17-18.80; P=0.029). CONCLUSIONS: Late diagnosis of Marfan syndrome is associated with delayed surgery and death.

Prevalence and Influence of Genetic Variants on Follow-Up Results in Patients Surviving Thoracic Aortic Therapy.

Background/Objective: To investigate the prevalence and effects of genetic variants (GVs) in survivors of thoracic aortic dissection/aneurysm repair. Methods: Patients aged 18-80 years who survived follow-up after cardiosurgical or endovascular repair of thoracic aortic aneurysm or dissection at a single tertiary center between 2008 and 2019 and underwent genetic testing were enrolled. The exclusion criteria were age >60 years, no offspring, and inflammatory- or trauma-related pathogenesis. Follow-up entailed computed tomography-angiography at 3 and 9 months and annually thereafter. All patients underwent genetic analyses of nine genes using next-generation sequencing. In cases of specific suspicion, the analysis was expanded to include 32 genes. Results: The study included 95 patients. The follow-up period was 3 ± 2.5 years. GVs were detected in 40% of patients. Correlation analysis according to primary diagnosis showed no significant correlation in disease persistence, progression, or in reintervention rates in aneurysm patients and a correlation of disease persistence with genetic variants according to variant class in dissection patients (p = 0.037). Correlation analysis according to follow-up CD finding revealed that patients with detected dissection, irrespective of original pathology, showed a strong correlation with genetic variants regarding disease progression and reintervention rates (p = 0.012 and p = 0.047, respectively). Conclusions: The prevalence of VUS is high in patients with aortic pathology. In patients with dissected aorta in the follow-up, irrespective of original pathology, genetic variants correlate with higher reintervention rates, warranting extended-spectrum genetic testing. The role of VUS may be greater than is currently known.

Association of adverse pregnancy outcomes with cardiovascular risk profiles in later life: Current insights from the Hamburg City Health Study (HCHS).

BACKGROUND AND AIMS: Adverse pregnancy outcomes (APO) have been related to increased cardiovascular (CV) risk and mortality in later life. Underlying pathomechanisms for the development of CV disease in these women are not yet fully understood. In this study, we aimed to investigate the relationship between APO and individual CV risk profiles in later life. METHODS: We used cross-sectional data from 10,000 participants enrolled in the Hamburg City Health Study (HCHS). We analysed self-reported APO, CV risk factors and health status, including biomarkers, electrocardiogram, echocardiography and vascular ultrasound. To examine associations, Wilcoxon rank sum test and Pearson's χ2-test were performed. Multivariable-adjusted regression models were calculated to determine associations. RESULTS: N = 1970 women who reported pregnancies were included. Median age was 63 years, 8.7 % reported gestational hypertension (gHTN), 18 % excessive weight gain and 2.4 % gestational diabetes. Ten percent had delivered newborns with birth weight <2.5 kg, 14 % newborns with birth weight >4 kg. In multivariable-adjusted models, significant associations between APO, CV risk profiles and cardiac remodeling were identified. gHTN correlated with higher body mass index (BMI) (Beta 1.68, CI 95 % 0.86-2.50; p < 0.001), hypertension (OR 4.58, CI 95 % 2.79-7.86; p < 0.001), left ventricular remodeling (e.g. left ventricular mass index (Beta 4.46, CI 95 % 1.05-7.87; p = 0.010)) and myocardial infarction (OR 3.27, CI 95 % 0.94-10.07; p = 0.046). CONCLUSIONS: In this population-based sample, APO were associated with CV risk profiles and cardiac remodeling in later life, suggesting early manifestations of future CV risk during pregnancy. Prospective data is needed for individual risk stratification in women with APO.

Detection of wound healing disorders after major amputations by measurements of the microcirculation: A prospective single-center study.

Introduction: Although major amputations can often be avoided due to evolving methods of endovascular and surgical revascularizations techniques, in patients with chronic limb-threatening ischemia, it is still necessary in some cases. Aim of this study was the detection of wound healing disorders through intraoperative microcirculation measurements in major limb amputations. Materials and methods: In this single-center clinical study, patients with an indication for major amputation were enrolled prospectively. Cause of amputation, patients' comorbidities including cardiovascular risk profile were assessed. Macrocirculation, as well as microcirculation were assessed. Microcirculation measurements were performed by fluorescence angiography with the administration of indocyanine green. A preoperative measurement was obtained at the amputation level, followed by three additional measurements of the amputation stump postoperatively. Wound healing was monitored and correlated with the microcirculatory findings, based on the perfusion parameters ingress and ingress rate, calculated in the indocyanine green fluorescence video sequences of the amputation stumps. Results: Forty-five patients were enrolled, including 19 (42%) below-the-knee amputations and 26 (58%) above-the-knee amputations. When considering the need for revision, a change in the microperfusion parameters was observed postoperatively. The mean value for ingress was significantly lower directly postoperatively in stumps requiring revisions (5 ± 0 A.U. versus 40.5 ± 42.5 A.U., p < 0.001). The mean value of ingress rate behaved similarly (0.15 ± 0.07 A.U./s versus 2.8 ± 5.0 A.U./s, p = 0.005). The evaluation of indocyanine green measurements when wound healing disorders occurred also showed nonsignificant differences in the mean values. Conclusion: Fluorescence angiography after major lower limb amputations appears to be an option of depicting microperfusion. Especially, the early postoperative detection of reduced perfusion can indicate a subsequent need for revision. Therefore, this method could possibly serve as a tool for intraoperative quality control after major limb amputation.

Editor's Choice - Vascular Registries Contributing to VASCUNET Collaborative Abdominal Aortic Aneurysm Outcome Projects: A Scoping Review.

OBJECTIVE: Vascular surgery registries report on procedures and outcomes to promote patient safety and drive quality improvement. International registries have contributed significantly to the VASCUNET collaborative abdominal aortic aneurysm (AAA) outcome projects. This scoping review aimed to outline the national registries in vascular surgery that currently participate in the VASCUNET collaborative AAA projects. METHODS: A scoping review of all published VASCUNET AAA studies and validation reports between 1997 and 2024 was undertaken. A survey was conducted among representatives of the international vascular registries contributing to VASCUNET collaborative AAA projects. RESULTS: Currently, vascular registries from 10 countries (Australia, Denmark, Finland, Hungary, Iceland, New Zealand, Norway, Sweden, Switzerland, and the UK) contribute to the current VASCUNET collaborative AAA project, of which eight have national coverage. In the past, three countries (Germany, Malta, and Italy) have participated in previous VASCUNET AAA projects, and a further three countries (Serbia, Greece, and Portugal) have planned participation in future projects. External validity is high for all current registries, with most reporting rates of > 90%. The majority have internal validation processes to assess data accuracy. VASCUNET mediated validation has also been performed by the consortium for five countries to date (Hungary, Sweden, Denmark, Malta, and Switzerland), for which a high degree of external and internal validity was identified. Most registries have established mechanisms for data linkage with national administrative datasets or insurance claims datasets and contribute to quality improvement through regular reporting to participating centres. CONCLUSION: National vascular registries from nations participating in the VASCUNET collaborative AAA projects are largely comprehensive, with high case ascertainment rates and good quality data with internal quality assurance. This provides a template for new registries wishing to join the VASCUNET collaboration and a benchmark for future research.

Evaluation of the Value of Histological Examination for the Prediction of Genetic Thoracic Proximal Aortopathies.

Background: Heritable connective tissue disorders are often accompanied by an increased risk for thoracic aortic aneurysm and dissection (TAAD). Profound knowledge of the underlying pathology may have an impact on individual treatment, systematic follow-up, and early detection by the screening of offspring. The aim of this study, based in a single high-volume tertiary center, was an analysis of the diagnostic validity of histopathologic findings in patients with TAAD due to these findings' accuracy in diagnosing heritable connective tissue disorders. Methods: Therefore, genetic testing by next-generation sequencing (NGS) was performed to evaluate the correlations. In total, 65 patients with TAAD undergoing surgical treatment before the age of 60 years or with age up to 80 years if they had offspring at the time of the procedure were included in the analysis. Results: In our cohort, no certain correlation of histological findings to the results of genetic diagnostics in patients with clinically relevant aortic pathology could be shown. Patients with histopathologic findings for heritable connective tissue disorder and a positive gene variant were 11.6 years younger than patients without mutation and without histological evidence for connective tissue disorder. Conclusions: Genetic clarification is useful to define the specific genotype of the disease of the aortic wall in the case of non-specific histological characteristics.

Short Term Outcomes and Treatment Intensity of Major Cardiovascular Emergencies During the COVID-19 Pandemic in Germany.

OBJECTIVE: There has been a decline in hospital admission rates in many countries since the beginning of the COVID-19 pandemic. Patient selection differed from episodes before the pandemic. This study investigated changes in baseline characteristics as well as the short term mortality rate and probability of receiving an invasive procedure while considering sex disparities. METHODS: Claims data provided by Germany's second largest insurance fund, BARMER, were used. Patients without COVID-19 who were treated for ST segment elevation myocardial infarction (STEMI), non-ST segment elevation myocardial infarction (NSTEMI), acute limb ischaemia (ALI), and stroke between 1 January 2018 and 31 December 2021 were included. Outcomes were compared separately for both sexes between the years before the pandemic (2018/2019) and during the pandemic (2020/2021). Propensity scores with exact matching were used to balance confounders including age, drug prescriptions in the previous year, federal state, month of admission, domiciliary care, and the Elixhauser comorbidities. Short term death and probability of invasive procedures were determined using cumulative incidence functions and Cox regressions. RESULTS: The cohort consisted of 140 989 hospitalisations of 122 340 individual patients (48.3% female) with 102 960 matched cases. Baseline characteristics were similar between episodes in the unmatched cohort. Earlier discharge was observed for all strata except for males with ALI or STEMI, where the probability of early discharge was unchanged. The probability of receiving an invasive procedure was increased for both sexes with ALI, NSTEMI, and STEMI but not for stroke. The analyses suggested neither a statistically significant increase of the in hospital mortality rate nor the 30 day mortality rate after the pandemic started. CONCLUSION: There was no evidence for a direct or indirect impact of the pandemic on major short term hospital outcomes. While the probability of receiving an invasive procedure increased for STEMI, NSTEMI, and ALI, the overall short term mortality rate was unaffected for both sexes.

Screening for Peripheral Vascular Stiffness in Lipedema Patients by Automatic Electrocardiogram-Based Oscillometric Detection.

Body mass index (BMI) is seen as a predictor of cardiovascular disease (CVD) in lipedema patients. A valid predictor of CVD is increased aortic stiffness (IAS), and previous research described IAS in lipedema. However, it is not known if this applies to all patients. In this cross-sectional single-center cohort study, peripheral pulse wave velocity (PWV) as a non-invasive indicator of aortic stiffness was measured in 41 patients with lipedema, irrespective of stage and without pre-existing cardiovascular conditions or a history of smoking and a maximum body mass index (BMI) of 35 kg/m2. Automatically electrocardiogram-triggered oscillometric sensor technology by the Gesenius-Keller method was used. Regardless of the stage of lipedema disease, there was no significant difference in PWV compared to published standard values adjusted to age and blood pressure. BMI alone is not a predictor of cardiovascular risk in lipedema patients. Measuring other anthropometric factors, such as the waist-hip ratio or waist-height ratio, should be included, and the existing cardiovascular risk factors, comorbidities, and adipose tissue distribution for accurate risk stratification should be taken into account. Automated sensor technology recording the PWV represents a valid and reliable method for health monitoring and early detection of cardiovascular risks.

[Recommendations for the specialist further training of nursing personnel on intensive care units in the treatment of abdominal aortic aneurysms: results of a modified Delphi procedure with experts]. / Empfehlungen zur Fachweiterbildung von Pflegefachpersonen der Intensivstation bei der Behandlung des Bauchaortenaneurysmas: Ergebnisse eines modifizierten Delphi-Verfahrens mit Expert:innen.

INTRODUCTION: The medical development in the previous 15 years and the changes in treatment reality of the comprehensive elective treatment of abdominal aortic aneurysms necessitate a re-evaluation of the quality assurance guidelines of the Federal Joint Committee in Germany (QBAA-RL). In the current version this requires a specialist further training quota for nursing personnel in intensive care wards of 50%. The quota was determined in 2008 based on expert opinions, although a direct empirical evidence base for this does not exist. METHODS: Representatives from the fields of patient representation, physicians, nursing personnel and other relevant interface areas were invited to participate in a modified Delphi procedure. Following a comprehensive narrative literature search, a survey and focus group discussions with national and international experts, a total of three anonymized online-based voting rounds were carried out for which previously determined key statements were assessed with a 4­point Likert scale (totally disagree up to totally agree). In addition, the expert panel had also defined a recommendation for a minimum quota for the specialist training of nursing personnel on intensive care wards in the treatment of abdominal aortic aneurysms, whereby an a priori agreement of 80% of the participants was defined as the consensus limit. RESULTS: Overall, 37 experts participated in the discussions and three successive voting rounds (participation rate 89%). The panel confirmed the necessity of a re-evaluation of the guideline recommendations and recommended the introduction of a shift-related minimum quota of 30% of the full-time equivalent of nursing personnel on intensive care wards and the introduction of structured promotional programs for long-term elevation of the quota. CONCLUSION: In this national Delphi procedure with medical and nursing experts as well as representatives of patients, the fundamental benefits and needs of professional specialist qualifications in the field of intensive care medicine were confirmed. The corresponding minimum quota for specialist further training of intensive care nursing personnel should generally apply without limitations to specific groups. The expert panel stipulates a shift-related minimum quota for intensive care nursing personnel with specialist training of 30% of the nursing personnel on intensive care wards and the obligatory introduction of structured and transparent promotion programs for the long-term enhancement.

Adherence and Persistence to Antiplatelet Therapy in Lower Extremity Peripheral Arterial Disease: A Danish Population Based Cohort Study.

OBJECTIVE: Adherence to antiplatelet therapy is recommended but unexplored in patients with symptomatic lower extremity peripheral arterial disease (PAD). Therefore, this study aimed to determine adherence and persistence to antiplatelet therapy in patients with PAD, defined as intermittent claudication and chronic limb threatening ischaemia. DESIGN: Population based nationwide cohort study. METHODS: This study included all Danish citizens aged ≥ 40 years with a first inpatient or outpatient diagnosis of symptomatic PAD between 2010 - 2017, and who had at least one prescription claim for aspirin and/or clopidogrel within 90 days after diagnosis. Adherence was determined by the proportion of days covered (PDC) during the first year after diagnosis. Persistence was defined as no treatment gap ≥ 30 days between prescription renewals over three year follow up. RESULTS: A total of 39 687 patients were eligible for inclusion, of whom 23 279 (58.7%) claimed a prescription for aspirin and/or clopidogrel within 90 days of diagnosis. Among these, 12 898 (55.4%) were prevalent users, while the remainder comprised new users who initiated the therapy after the index PAD diagnosis. The mean PDC was 74.5% (SD 35.0%) for prevalent users and 60.5% (SD 30.5%) for new users. Adherence increased with age and number of concomitant drugs. The overall one year cumulative incidence treatment discontinuation was 13.0% (95% CI 12.5 - 13.4%) overall, 17.2% (CI 16.6 - 17.9%) for prevalent users, and 7.9% (CI 7.4 - 8.4%) for new users. At three year follow up, the cumulative incidence of discontinuation was 31.5% (CI 30.9 - 32.2%) overall, 44.6% (CI 43.7 - 45.4%) for prevalent users, and 14.6% (CI 13.9 - 15.3) for new users. CONCLUSION: Less than 60% of patients with newly diagnosed symptomatic PAD claimed a prescription for antiplatelet therapy within 90 days of diagnosis, and both adherence and persistence were moderate during the first year after diagnosis. These findings underscore the importance of efforts to improve the initiation and continuation of antiplatelet therapy in patients with PAD.

Editor's Choice - Outcomes Following Intact and Ruptured Aneurysm Repair across Nations: Analysis of International Registry Data from the VASCUNET Collaboration 2014 - 2019.

OBJECTIVE: To determine the peri-operative mortality rate for intact and ruptured abdominal aortic aneurysm (AAA) repair in 10 countries and to compare practice and outcomes over a six year period by age, sex, and geographic location. METHODS: This VASCUNET study used prospectively collected data from vascular registries in 10 countries on primary repair of intact and ruptured AAAs undertaken between January 2014 and December 2019. The primary outcome was peri-operative death (30 day or in hospital). Logistic regression models were used to estimate the association between peri-operative death, patient characteristics, and type of procedure. Factors associated with the use of endovascular aortic aneurysm repair (EVAR) were also evaluated. RESULTS: The analysis included 50 642 intact and 9 453 ruptured AAA repairs. The proportion of EVARs for intact repairs increased from 63.4% in 2014 to 67.3% in 2016 before falling to 62.3% in 2019 (p < .001), but practice varied between countries. EVAR procedures were more common among older patients (p < .001) and men (p < .001). Overall peri-operative mortality after intact AAA repair was 1.4% (95% confidence interval [CI] 1.3 - 1.5%) and did not change over time. Mortality rates were stable within countries. Among ruptured AAA repairs, the proportion of EVARs increased from 23.7% in 2014 to 35.2% in 2019 (p < .001). The average aortic diameter was 7.8 cm for men and 7.0 cm for women (p < .001). The overall peri-operative mortality rate was 31.3% (95% CI 30.4 - 32.2%); the rates were 36.0% (95% CI 34.9 - 37.2%) for open repair and 19.7% (95% CI 18.2 - 21.3%) for EVAR. This difference and shift to EVAR reduced peri-operative mortality from 32.6% (in 2014) to 28.7% (in 2019). CONCLUSION: The international practice of intact AAA repair was associated with low mortality rates in registry reported data. There remains variation in the use of EVAR for intact AAAs across countries. Overall peri-operative mortality remains high after ruptured AAA, but an increased use of EVAR has reduced rates over time.

A Systematic Review on Thoracic Endovascular Repair Outcomes in Blunt Thoracic Aortic Injuries.

PURPOSE: Blunt thoracic aortic injury (BTAI) represents a potentially life-threatening condition and thoracic endovascular aortic repair (TEVAR) is recommended as the first-line treatment (Class I level of evidence C) by the current guidelines. The aim of this systematic review was to determine the perioperative and mid-term follow-up outcomes of patients with BTAI treated with TEVAR. MATERIALS AND METHODS: We reviewed the English literature published between 2000 and 2022, via Ovid, using MEDLINE, EMBASE, and CENTRAL databases, until July 30, 2022. Observational studies and case series, with ≥5 patients, reporting on the perioperative and follow-up outcomes of patients who underwent TEVAR for BTAI were included. The Newcastle-Ottawa Scale was used to assess the risk of bias. Primary outcomes were technical success and 30-day mortality, cerebrovascular morbidity. Secondary outcomes were mortality and re-interventions during the mid-term follow-up. RESULTS: From 5201 articles identified by the literature search, 35 eligible studies were included in this review. All studies had a retrospective study design. In total, 991 patients were included. The mean age was 34.5±16.5 years (range=16-89 years). Technical success was 98.0% (odds ratio [OR], 95% confidence interval [CI]=0.98, 0.99, p<0.001, I2=0%). Mortality at 30 days was 5.0% (OR, 95% CI=0.03, 0.06, p<0.001, I2=5.56%). Spinal cord ischemia occurred in 1.0% (OR, 95% CI=0.01, 0.02, p<0.001, I2=0%) and stroke rate was 2.0% (OR, 95% CI=0.01, 0.02, p<0.001, I2=0%). The available follow-up was estimated at 29 months (range=3-119 months) with mortality rate at 2.0% (OR, 95% CI=0.01, 0.02, p<0.001, I2=0%) and re-intervention rate at 1.0% (OR, 95% CI=0.01, 0.02, p<0.001, I2=10.5%). CONCLUSION: Thoracic endovascular aortic repair showed high technical success and low early cerebrovascular morbidity and mortality rates. In the mid-term follow-up, the estimated mortality and re-intervention rates were also low. Furthermore, higher quality prospective studies are needed. CLINICAL IMPACT: Thoracic endovascular aortic repair (TEVAR) is recommended as the first line treatement in patients with blunt thoracic aortic injuries (BTAI). This systematic review of 35 retrospective studies and 991 patients showed high technical success (98.0%) with an associated 30-day mortality at 5.0% and low spinal cord ischemia (1%) and stroke rates (2.0%). Mid-term mortality and re-intervention rates reassure the effectiveness of TEVAR in BTAI cases.

Awareness of smoking cessation amongst German vascular surgeons.

Background: Smoking represents the well-known enemy of vascular well-being. Numerous previous studies emphasised the important role of smoking on the development and progression of atherosclerotic cardiovascular disease. The current study aimed to identify hurdles and barriers for an insufficient implementation of secondary prevention in the treatment of lower extremity peripheral arterial disease (PAD). Methods: All members of the German Society for Vascular Surgery and Vascular Medicine (DGG) with valid email addresses were invited to participate in an electronic survey on smoking. Results are descriptively presented. Results: Amongst 2716 invited participants, 327 (12%) submitted complete responses, thereof 33% women and 80% between 30 and 59 years old (87% board certified specialists). 83% were employed by hospitals (56% teaching hospital, 14% university, 13% non-academic) and 16% by outpatient facilities. 6% are active smokers (63% never) while a mean of five medical education activities on smoking cessation were completed during the past five years of practice. Only 27% of the institutions offered smoking cessation programs and 28% of the respondents were aware of local programs while a mean of 46% of their patients were deemed eligible for participation. 63% of the respondents deemed outpatient physicians primarily responsible for smoking cessation, followed by medical insurance (26%). Conclusions: The current nationwide survey of one scientific medical society involved in the care of patients with vascular disease revealed that smoking cessation, although being commonly accepted as important pillar of comprehensive holistic care, is not sufficiently implemented in everyday clinical practice.