Medium-term outcomes after inferior vena cava reconstruction for acute and chronic deep vein thrombosis and retroperitoneal fibrosis.

OBJECTIVE: In the present study, we evaluated the technical and clinical outcomes after endovascular inferior vena cava (IVC) reconstruction in patients with nonmalignant obstruction. METHODS: The preoperative, procedural, and follow-up medical records and imaging studies were retrospectively reviewed for 59 consecutive patients who had undergone endovascular IVC reconstruction for nonmalignant obstruction from February 2014 to January 2019. The patients were classified into three groups according to the quality of their infrainguinal inflow vessels. The outcomes measured were the primary, primary-assisted, and secondary patency rates, reintervention rates, and symptomatic resolution. RESULTS: The indications for treatment were post-thrombotic syndrome (n = 41), acute deep vein thrombosis (n = 12), and retroperitoneal fibrosis (n = 6). The median patient age was 37 years, 11 months, 71.2% were men, and 32.2% had a diagnosis of thrombophilia, with no significant difference in these demographics between the three inflow groups. The median follow-up duration was 2 years, 3 months (range, 6 months to 6 years, 5 months). The whole-cohort primary patency was 91.2%, 71.0%, and 24.1% at 1, 3, and 5 years, respectively. Secondary patency was 76.7% at 1 year and 66.4% at both 3 and 5 years. Inflow group A (no post-thrombotic disease in the femoral or deep femoral veins) demonstrated significantly higher primary patency compared with group B (stenotic disease in one or two infrainguinal inflow veins; P = .009) and significantly higher secondary patency than for both groups B (P = .008) and C (all three infrainguinal inflow veins had stenosed or femoral and/or deep femoral vein occlusion was present; P = .04). In post-thrombotic syndrome patients, the Villalta scores had decreased from a mean of 14.2 to 8.1 at 1 year and 6.8 at 2 years, and the Venous Insufficiency Epidemiological and Economic Study Quality of Life score had improved from a mean of 19.8 to 54.1 and 57.3 at 1 and 2 years, respectively. In the patients with acute deep vein thrombosis, the Villalta score was 2.8 at 1 year and 0 at 2 years. In the patients with retroperitoneal fibrosis, the Venous Insufficiency Epidemiological and Economic Study Quality of Life score had improved from a mean of 25.3 at baseline to 44 at 6 months. CONCLUSIONS: Endovascular IVC reconstruction for nonmalignant obstruction can achieve good patency and clinical improvement, although the outcomes were poorer for patients with post-thrombotic disease of the femoral and deep femoral veins.

Effect of thrombophilia on clinical outcomes of chronic post-thrombotic patients after iliofemoral stenting with nitinol venous stents.

OBJECTIVE: Thrombophilia is a prothrombotic condition that increases the risk of venous thromboembolism. It is unclear whether the presence of thrombophilia alters the clinical outcomes after deep venous stenting. The aim of the present study was to examine the relationship between thrombophilia and outcomes after stenting for post-thrombotic syndrome. METHODS: Consecutive patients (2012-2017) receiving a nitinol venous stent for chronic post-thrombotic venous occlusive disease with a minimum of 18 months of follow-up in one center using the same anticoagulation protocol were included. The clinical history and thrombophilia testing results were reviewed. The outcomes were stent patency, which was assessed using duplex ultrasonography at 24 hours, 2 and 6 weeks, 3 months, 6 months, and annually thereafter; and reinterventions, which were performed when the stent diameter was <50% or occluded. RESULTS: Of the 136 patients who had undergone intervention, 55 (40%) had had a provoked deep vein thrombosis (DVT) and 81 (60%) had had an unprovoked DVT and had therefore undergone thrombophilia testing. Of the 81 patients, 38 (47%) had had either inherited (n = 19; 50%) or acquired (n = 19; 50%) thrombophilia. Of the 136 patients who had undergone stenting, 68 had required reintervention (50%) during follow-up to maintain stent patency. Of the 55 patients with a provoked DVT, 29 (53%) had required reintervention. Of the 81 patients with an unprovoked DVT, 39 (48%) had required reintervention (P = .420). Of the 38 patients with unprovoked DVT and thrombophilia, 17 (45%) had required reintervention. Of the 43 patients with unprovoked DVT and no thrombophilia, 22 (51%) had required reintervention (P = .766). The cumulative patency rate was 80% for patients with provoked DVT and 88% for those with unprovoked DVT (P = .193). The presence of thrombophilia was not associated with patency loss (92% cumulative patency for patients with thrombophilia and 84% for patients without thrombophilia; P = .307). CONCLUSIONS: Using our anticoagulation protocol, patients with and without thrombophilia had similar clinical outcomes after deep venous stenting and should not be excluded from iliofemoral venous stenting. We found no significant differences in outcomes in conjunction with appropriate postoperative anticoagulation therapy.

Management of acute and chronic iliofemoral venous outflow obstruction: a multidisciplinary team consensus.

The aim of this manuscript was to establish a consensus for the management of acute and chronic venous obstruction among specialists in the UK. Specialist physicians representing vascular surgery, interventional radiology and hematology were invited to 3 meetings to discuss management of acute and chronic iliofemoral obstruction. The meetings outlined controversial areas, included a topic-by-topic review; and on completion reached a consensus when greater than 80% agreement was reached on each topic. Physicians from 19 UK hospitals agreed on treatment protocols and highlighted areas that need development. Potential standard treatment algorithms were created. It was decided to establish a national registry of venous patients led by representatives from the treating multidisciplinary teams. Technical improvements have facilitated invasive treatment of patients with acute and chronic venous obstruction; however, the evidence guiding treatment is weak. Treatment should be conducted in centers with multi-disciplinary input; robust, coordinated data collection; and regular outcome analysis to ensure safe and effective treatment and a basis for future evolvement.

Midterm outcomes in postpartum women following endovenous treatment for acute iliofemoral deep vein thrombosis.

BACKGROUND: The development of post-thrombotic syndrome (PTS) after iliofemoral deep vein thrombosis (DVT) continues to be a considerable issue for both pregnant and postpartum women with rates as high as 70% among those managed with anticoagulation alone. This study aims to characterize the outcomes of interventional treatment for acute iliofemoral DVT in this at-risk population. METHODS: A retrospective analysis of all postpartum patients treated for acute iliofemoral DVT with lysis and stenting between January 2012 and December 2017 at a referral center. Patient demographics, risk factors, procedural factors. and complications were collected. Post-treatment outcomes were compared with all nonpostpartum females treated within the same time period. These included the severity of PTS evaluated using the Villalta scale, duration of vessel patency and factors affecting reintervention timing and success. Further detailed review of cases needing reintervention was also conducted through a retrospective review of documentation and an analysis of all imaging by a consultant radiologist. RESULTS: A total of 11 postpartum women were identified. The median age was 28 years (range, 22-41 years) and intervention was performed at a median of 3 weeks after birth (range 2-12 weeks). No major or minor complications associated with intervention were reported in any patients. The median Villalta score was 3 at 6 months, improving to 2 at 12 months. Overall, two patients were classified as mild having PTS (18%), with no cases of moderate to severe PTS. On comparison with nonpostpartum (n = 68) Villalta scores, no significant difference in outcome was observed at 6 months (median score, 3; range, 0-15 months; P = .95) or at 1 year (median score, 1; range, 0-15; P = .84). Cumulative patency at 1 year was found to be 64% in postpartum women compared with 93% in nonpostpartum women. The postpartum state was found to be a significant predictor of cumulative patency loss (hazard ratio, 0.10; 95% confidence interval, 0.02-0.62; P = .01). However, no significant difference in primary and primary-assisted patency was observed. Of the postpartum patients, 55% required reintervention (6/11) compared with 29% of nonpostpartum patients (20/68). The mean time to initial reintervention was 62 days (range, 7-233 days). Reintervention was unsuccessful in all cases presenting with 100% vessel occlusion (4/11), but successful in both cases with partial occlusion (2/11). Analysis of the etiologic factors associated with reintervention revealed that all reintervention cases were associated with technical failure to fully lyse and stent beyond residual disease at the initial procedure. No technical, flow, or hematologic factors were identified in the four cases that retained primary patency. CONCLUSIONS: This study suggests that percutaneous intervention to achieve early thrombus removal and venous stenting provides a favorable alternative to conservative therapies owing to its potential to decrease the severity of PTS. Completion of lysis and adequate stenting of disease is essential to prevent reocclusion, for which reintervention carries a lower likelihood of success. Further research is warranted to further characterize the appropriate management of postpartum women with iliofemoral DVT.

Two Year Outcome After Chronic Iliac Vein Occlusion Recanalisation Using the Vici Venous Stent®.

OBJECTIVE/BACKGROUND: The aim was to assess two year outcomes with placement of the Vici Venous Stent® in patients with chronic iliofemoral venous occlusions (complete blockage). METHODS: This was a retrospective single centre study comprising patients treated with the Vici Venous Stent for venographically verified iliofemoral venous occlusion and post-thrombotic syndrome (Villalta score ≥ 5 points) at least 12 months after acute deep vein thrombosis. Venography and intravascular ultrasound were used peri-operatively; duplex ultrasound was used to assess stent patency during follow up. RESULTS: Eighty-eight patients (101 limbs) had stent placement between March 2014 and October 2016. Median pre-treatment Villalta score was 14 (range 5-33). Stenting extended across the inguinal ligament in 63 limbs (62%) in order to land in a healthy venous segment. Six patients (7%) required endophlebectomy and fistula creation. Median imaging follow up was 21 months (range 0-41 months). Primary, assisted primary and secondary patency rates at one year were 59%, 78%, and 87%, respectively, and two years 51%, 73%, and 82%, respectively. Forty-three limbs (43%) had re-intervention (lysis, venoplasty, and/or placement of stent) during follow up; median time to re-intervention was 32 days (range 0-520 days). At 24 months, 37 of 53 limbs (70%) with available Villalta assessment showed clinically significant improvement (>30% reduction of baseline score). Villalta scores at the 6, 12, and 24 month clinical follow up were significantly lower than before stenting (p < .001, all time points). In a subset analyses of limbs with stenting terminating above and below the inguinal ligament, secondary cumulative patency rates at 24 months were 90% and 79%, respectively; clinical outcome showed 58% vs. 73% of limbs with clinically significant improvement, respectively. There was no statistically significant difference in patency or clinical outcomes. CONCLUSION: The Vici Venous Stent is associated with a good secondary patency rate and durable and substantial symptomatic resolution in patients with chronic post-thrombotic occlusions, regardless of whether stents extended beneath the inguinal ligament.

The effect of hydroxychloroquine on haemostasis, complement, inflammation and angiogenesis in patients with antiphospholipid antibodies.

Objectives: HCQ has been described as having a beneficial effect in patients with APS but its mechanism of action is unclear. We hypothesized that HCQ may have effects on subnormal angiogenesis, inflammation and haemostatic biomarkers seen in APS. The aim of our study was to assess laboratory markers [annexin A5 (AnxA5) anticoagulant activity, tissue factor (TF) levels, thromboelastography (TEG), CRP, Bb, C3a and VEGF] in HCQ-naïve patients with aPL at baseline and after commencing HCQ. Methods: Twenty-two patients with aPL [20 female, 2 male, median age 55 (range 18-70) years] had blood taken pre- and 3 months after starting HCQ 200 mg daily. Results: Soluble TF levels were significantly reduced comparing baseline and 3 months after HCQ commencement [401.8 (152.8) vs 300.9 (108) pg/ml (P = 0.010)]. No significant changes were found in the following [reported as pre- and post-HCQ commencement, mean (s.d.)]: AnxA5 anticoagulant ratio [187.1 (29.5) vs 193 (31) (P = 0.157)], anti-domain1 ß2 glycoprotein1 IgG activity [1.8 (2) vs 1.2 (1.4) µg/ml (P = 0.105)], complement C3a-des-Arg [147.8 (84.5) vs 154.4 (88.1) ng/ml (P = 0.905)], complement Bb [1.3 (0.7) vs 1.1 (0.7) µg/ml (P = 0.422)], VEGF [68.8 (40) vs 59.4 (19.6) pg/ml (P = 0.454)] and CRP [7 (3.5) vs 7 (3.9) µg/ml (P = 0.917)]. TEG results including TEG reaction time, achievement of clot firmness, TEG maximum amplitude and TEG percentage lysis 30 and 60 min after maximum amplitude showed no significant difference. Conclusion: HCQ significantly reduced soluble TF levels in patients with aPL. No significant change was observed in AnxA5 activity, anti-domain 1 IgG activity, TEG, CRP, complement Bb and C3a-des-Arg, and VEGF. Further studies of a larger patient cohort are needed.

Meeting the family: measuring effectiveness of family meetings in a specialist inpatient palliative care unit.

OBJECTIVE: The World Health Organization (WHO) definition of palliative care as "an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness …" recognizes the importance of family members in this setting. In practice, family meetings account for a significant amount of the weekly workload in a specialist inpatient palliative care unit. Despite this, there is little empirical evidence to support the benefits of family meetings from the perspective of family members. METHOD: A prospective study over 6 months, invited a designated family member to complete a self-report instrument (SRI) and the Family Inventory of Needs (FIN) questionnaire prior to, immediately following, and 48 hours after a planned family meeting attended by several members of the multidisciplinary team. RESULTS: Thirty-one designated family members completed the study. The SRIs completed prior to a family meeting identified particular areas of concern and worry for family members, and also helped to generate an agenda based on the family's particular needs. The pre-meeting FIN identified areas of patient care of greatest importance to each family member, and asked them to rate whether particular care needs were presently met or unmet, in their opinion, by the healthcare team caring for the patient. Following the family meeting, repeat SRIs showed an overall reduction in concerns and increased confidence in dealing with those issues raised. Post-family meeting FIN scores confirmed a greater number of met care needs compared with pre-meeting scores, all of which were sustained over time. SIGNIFICANCE OF RESULTS: This study confirms the value of planned multidisciplinary family meetings for patients in specialist inpatient palliative care units. It identifies the often unmet needs of family members and the sustained benefits associated with formal family meetings.

The pathogenesis and management of the coagulopathy of acute promyelocytic leukaemia.

Coagulopathy occurs in most patients with (APML) and is life-threatening; therefore prompt diagnosis and recognition of any coagulation defect is imperative. Unfortunately haemorrhage remains a major cause of early death, preventing some from reaching treatment. The coagulopathy is caused directly or indirectly by the leukaemic cells through expression of activators of coagulation and fibrinolysis, proteases and cytokine generation, compounded by failure of platelet production due to marrow invasion. At presentation the predominant feature is usually hyperfibrinolysis. Since the introduction of all-trans retinoic acid (ATRA), patient outcome has dramatically improved; yet, haemorrhagic complications remain the most frequent cause of mortality. Thrombotic complications occur but are less well recognized and potentially underreported. Supportive measures and prompt initiation of ATRA currently represent the mainstay of treatment of the coagulopathy in patients with suspected APML, but unanswered questions remain as to the optimal approach to further decrease the associated haemorrhagic and thrombotic risks. In particular, it is unclear how to best predict and monitor the coagulopathy; whether there is a role for the early use of antifibrinolytics; the most appropriate trigger for giving fibrinogen replacement and the value of low-dose anticoagulation to suppress coagulation activation once fibrinolysis has been suppressed.

Glanzmann's thrombasthenia: report of a case and review of the literature.

Glanzmann's thrombasthenia is a rare congenital bleeding disorder. Patients usually present with mucocutaneous bleeding and excessive bleeding associated with trauma and/or surgery. Patients have an increased bleeding time and a normal platelet count with abnormal platelet function assays. Genetically, Glanzmann's thrombasthenia is associated with mutations in the genes which encode for glycoproteins, GPIIb or GPIIIa. Defects in these genes lead to a lack of or highly reduced expression of the glycoprotein complex (GPIIb/GPIIIa), resulting in platelet dysfunction. Bleeding is managed by platelet transfusions. Bone marrow transplants have been used successfully in rare cases. With proper supportive care Glanzmann's thrombasthenia has a very good prognosis.

Whole-Body MRI versus PET in assessment of multiple myeloma disease activity.

OBJECTIVE: The purpose of this study was to compare FDG PET; whole-body MRI; and the reference standard, bone marrow aspiration and biopsy, to determine the best imaging technique for assessment of disease activity in multiple myeloma. SUBJECTS AND METHODS: Twenty-four patients (13 women, 11 men; mean age, 67.1 years; range, 44-83 years) with bone marrow biopsy-proven multiple myeloma were included in the study. All patients underwent PET/CT and whole-body MRI within 10 days of each other. Eight patients underwent more than one follow-up PET/CT and whole-body MRI examination, for a total of 34 pairs of images. Activity was defined as lesions with a maximum standardized uptake value greater than 2.5 at PET and as evidence of marrow packing at whole-body MRI. PET and whole-body MRI results were correlated with findings at bone marrow aspiration biopsy. RESULTS: PET had a sensitivity of 59%, specificity of 75%, positive predictive value of 81%, and negative predictive value of 50% (p = 0.08). Whole-body MRI had a sensitivity of 68%, specificity of 83%, positive predictive value of 88%, and negative predictive value of 59% (p = 0.01). In 62% of cases, PET and whole-body MRI findings were concordant. When PET and whole-body MRI findings were concordant and positive, no false-positive results were found, yielding a specificity and a positive predictive value of 100% (p = 0.04). CONCLUSION: Whole-body MRI performed better than PET in the assessment of disease activity, having a higher sensitivity and specificity. The positive predictive value of whole-body MRI in the assessment of active disease was high at 88%. When used in combination and with concordant findings, PET and whole-body MRI were found to have a specificity and positive predictive value of 100%, which may be of value to clinicians assessing the effectiveness of aggressive and expensive treatment regimens.