Previous Deep Vein Thrombosis Research Articles

Cost-Effectiveness of Performing Reference Ultrasonography in Patients with Deep Vein Thrombosis.

The diagnosis of recurrent ipsilateral deep vein thrombosis (DVT) with compression ultrasonography (CUS) may be hindered by residual intravascular obstruction after previous DVT. A reference CUS, an additional ultrasound performed at anticoagulant discontinuation, may improve the diagnostic work-up of suspected recurrent ipsilateral DVT by providing baseline images for future comparison. To evaluate the cost-effectiveness of routinely performing reference CUS in DVT patients. Patient-level data (n = 96) from a prospective management study (Theia study; NCT02262052) and claims data were used in a decision analytic model to compare 12 scenarios for diagnostic management of suspected recurrent ipsilateral DVT. Estimated health care costs and mortality due to misdiagnosis, recurrent venous thromboembolism, and bleeding during the first year of follow-up after presentation with suspected recurrence were compared. All six scenarios including reference CUS had higher estimated 1-year costs (€1,763-€1,913) than the six without reference CUS (€1,192-€1,474). Costs were higher because reference CUS results often remained unused, as 20% of patients (according to claims data) would return with suspected recurrent DVT. Estimated mortality was comparable in scenarios with (14.8-17.9 per 10,000 patients) and without reference CUS (14.0-18.5 per 10,000). None of the four potentially most desirable scenarios included reference CUS. One-year health care costs of diagnostic strategies for suspected recurrent ipsilateral DVT including reference CUS are higher compared to strategies without reference CUS, without mortality benefit. These results can inform policy-makers regarding use of health care resources during follow-up after DVT. From a cost-effectiveness perspective, the findings do not support the routine application of reference CUS.

Successful Treatment of Massive Pulmonary Embolism with Rescue Fibrinolysis in Young Patient with Homocystinemia - Case Report

Abstract Pulmonary embolism (PE) is the most frequently missed diagnosis in the urgent clinical department with serious consequences. Patients with unprovoked PE have increased risk of recurrent PE. Approximately 5 to 8% of PE patients have inherited thrombophilias. A solated homocystinemia is a rare cause of unprovoked acute pulmonary embolism. Timely diagnosis and proper treatment can prevent complications, costs and mortality and provide patient better quality of life. We are presenting a 42-year-old woman was admitted to our emergency department with the first episode of severe dyspnea and chest pain. She had no history of previous cardiovascular or respiratory disease and no history of previous pulmonary embolism (PE) or deep vein thrombosis (DVT). Urgent echocardiography showed indirect signs of pulmonary embolism which was confirmed by the pulmonary artery CT angiography performed one day after the patient’s admission. After two days of heparin infusion, she developed a hemodynamic instability with cardiogenic shock and was treated successfully with fibrinolysis. After the clinical stabilization, she was put on the rivaroxaban therapy, which was recommended for additional six months. The thrombophilia profile was done two weeks after stopping the therapy with rivaroxaban. The thrombophilia panel came back positive for high levels of homocysteine (67 μmol/L), with other thrombophilia results within normal limits. The patient was stable during the follow-up period. Pulmonary embolism should be always suspected in younger patients with acute severe dyspnea even without provocable risk factors. High suspicion level and fast diagnosis are lifesaving. In younger patients presented with unprovoked pulmonary embolism, clinicians should consider inherited prothrombotic factors and homocystinemia as a potential cause. Rescue fibrinolysis is a lifesaving therapy in hemodynamic worsening in intermediate high-risk PE patients. A longer anticoagulation therapy should be considered in these cases with novel oral anticoagulants that are recommended as safer and superior therapy.

Management of Recurrent Venous Thromboembolism in Severe Immune Thrombocytopenia: A Case Report and a Review of the Literature.

We report a case of a 58-year-old man with recurrent unprovoked deep vein thrombosis (DVT) and severe immune thrombocytopenia (ITP) with a platelet count of 19 × 109/L. We further review studies reporting venous thromboembolism (VTE) in patients with severe ITP (≤ 35 × 109/L) and identified 14 patients highlighting VTE risk factors and management of these patients. The present case had several risk factors for VTE (previous DVT, obesity, heterozygosity for factor V Leiden mutation, and previous splenectomy). The patient was initially treated with low-molecular-weight heparin followed by long-term apixaban treatment. The literature review together with our case demonstrates that VTE in severe ITP (≤ 35 × 109/L) can occur in patients with VTE risk factors and antithrombotic management of these patients can be achieved without bleeding depending on severity of thrombocytopenia either by full or reduced dose of anticoagulation together with ITP therapy.

OC 34.3 Walking Capacity in Patients with Acute Deep Vein Thrombosis is Associated with the Location of Thrombosis and is Reduced in Patients with a History of Previous Deep Vein Thrombosis

OC 34.3 Walking Capacity in Patients with Acute Deep Vein Thrombosis is Associated with the Location of Thrombosis and is Reduced in Patients with a History of Previous Deep Vein Thrombosis

OA11 Osteoporosis, denosumab, and hypocalcaemia in multimorbid renal impairment

Abstract Introduction Osteoporosis affects approximately 30% of patients with chronic kidney disease (CKD) at stages 3-5, with 10% of women and 5% of men experiencing a fragility fracture within a given three-year follow up period. First-line management of osteoporosis with bisphosphonates is often precluded in CKD, with alendronate and zoledronate being contraindicated in creatinine clearances of < 35mL/min, and risedronate if < 30mL/min. Denosumab, a RANK-ligand inhibiting monoclonal antibody, can be used in cases of poorer renal function, however causes hypocalcaemia more commonly than bisphosphonates. Concomitant CKD compounds this risk if creatinine clearance falls below 30mL/min, presenting additional challenges for management. Case description A 75-year old woman with known osteoporosis and chronic kidney disease secondary to hypertension and a pauci-immune necrotising glomerulonephritis was found to have a serum calcium of 2.08mmol/L approximately three months after commencing six-monthly denosumab. Her lumbar spine T-score on dual x-ray absorptiometry showed severe osteoporosis at −2.9, with a fragility fracture of the humerus sustained four years prior. Her baseline eGFR was 23mL/min/1.73m2, and creatinine clearance was 22mL/min. Her past medical history was extensive and included secondary hyperparathyroidism due to CKD and resulting vitamin D deficiency, with a PTH of 23.0pmol/L, serum calcium of 2.26mmol/L, and a serum 25-OH vitamin D3 level of 33.5nmol/L four months prior to denosumab therapy. The history also included Sjögren’s syndrome on long-term daily prednisolone 7.5mg, alternate-day hydroxychloroquine 200mg, and daily leflunomide 10mg for accompanying synovitis of the hands, hypothyroidism controlled with daily levothyroxine 50mcg, hypertension controlled with daily 2.5mg bisoprolol, peripheral neuropathy secondary to vitamin B12 deficiency on three-monthly hydroxocobalamin, myocardial infarction with percutaneous coronary intervention to three arteries, diverticular disease with abscess perforation requiring laparotomy and intensive care admission, and a previous deep vein thrombosis on long-term anticoagulation with apixaban. The patient had received risedronate in the past while her creatinine clearance still permitted this. Her vitamin D deficiency had been managed orally with Adcal-D3 and colecalciferol 800IU once daily, which was uptitrated to 1600IU once daily prior to commencing denosumab. However, despite the patient’s serum vitamin D levels rising from 33.5 to 37.2nmol/L in the months preceding her first denosumab infusion, serum calcium had fallen below normal range on post-infusion blood tests. Serum phosphate returned at 0.91mmol/L, and vitamin D had fallen to 23.1nmol/L in the three months following denosumab treatment. Consequently, the patient was commenced on longer term intensive vitamin D supplementation. Discussion This case raises the issue of multifactorial hypocalcaemia against a backdrop of osteoporosis, persistent vitamin D deficiency, and medical comorbidity limiting management options. The significance of CKD in this case highlights the importance of monitoring and replacing vitamin D. While the hypocalcaemia detected at three months post-infusion may have occurred in part due to the direct effect of denosumab, the patient’s vitamin D deficiency no doubt also played a role in this, and failing to detect the deficiency may have resulted in premature termination of denosumab treatment. This case also highlights the extended timeframe in which such hypocalcaemia may present. Case-series literature describes hypocalcaemia manifesting at, on average, eight days post-infusion in patients with CKD-associated hyperparathyroidism. However we demonstrate here that extended lag times up to twelve weeks are possible; the patient was symptomatic with leg cramping at the time of clinic follow up. Future practice may benefit from establishing monitoring regimes for those with CKD and osteoporosis receiving denosumab, or bisphosphonates toward the lower end of permitted renal function. Key learning points Chronic kidney disease both limits available management options in osteoporosis, and increases the likelihood of complications from the remaining available agents – illustrated here with hypocalcaemia. Hypocalcaemia in a patient with chronic kidney disease and osteoporosis may be driven by several factors: hyperphosphataemia, vitamin D deficiency, and the effect of denosumab to prevent mobilisation of calcium from bone to serum can all be culprits. Calcium, phosphate, and vitamin D all need to be tightly monitored in medically complex patients on treatment for osteoporosis.

Venous Thromboembolism Prophylaxis in Foot and Ankle Surgery: A Worldwide Survey

Current recommendations on thromboprophylaxis for foot and ankle (FA) surgery are often inconsistent and generally based on weak evidence. The aim of this survey study was to evaluate the current practice among orthopedic surgeons regarding venous thromboembolism (VTE) prophylaxis following FA surgery. From February 2019 to March 2020, an online questionnaire was sent by e-mail to orthopedic societies across the world. The questionnaire was hosted by the International Society of Thrombosis and Haemostais RedCAP platform. Topics of interest were VTE rates following FA surgery, duration and type of thromboprophylaxis, bleeding complications, VTE risk factors for prophylaxis and use of risk assessment. A total of 693 FA orthopedic surgeons from all continents completed the survey of whom 392 (57%) performed more than 200 FA procedures per year. A total of 669/693 (97%) respondents stated that thromboprophylaxis is necessary in FA surgeries. When thromboprophylaxis was prescribed, half of surgeons prescribed it for the duration of immobilization. Acetylsalicylic acid, low molecular weight heparin and direct-oral anticoagulants were, in this order, the preferred choice. Acetylsalicylic acid and low molecular weight heparin were predominantly prescribed in North America and Europe, respectively. Previous deep vein thrombosis, immobility, obesity and inherited thrombophilia were considered the main risk factors indicative of thromboprophylaxis use. In this survey, most surgeons agree that thromboprophylaxis is indicated for FA surgery, but the prescription, type and duration of prophylaxis differs greatly with a large intercontinental discrepancy. These survey results could be a foundation for developing uniform guidelines to optimize thromboprophylactic strategies in FA procedures around the world.

Association between the degree of iliac venous outflow obstruction by intravascular ultrasound and lower limb venous reflux

ObjectiveThe present study aims to evaluate the association between the degree of iliac venous outflow obstruction (IVOO) identified by intravascular ultrasound (IVUS) and venous reflux presentation in the lower limbs on duplex ultrasound (DU). MethodsPatients with bilateral chronic venous insufficiency, CEAP (clinical-etiology-anatomy-pathophysiology) C3 to C6, and a visual analog scale score for pain >5 underwent DU for reflux evaluation of the deep venous system (reflux ≥1 second); superficial system, great saphenous vein, and small saphenous vein (reflux ≥0.5 second); and perforator system (reflux ≥0.35 second). All patients underwent IVUS in the iliac venous territory. The area of the impaired venous segments was categorized as obstruction <50% (category 1), 50% to 79% (category 2), and ≥80% (category 3). The venous clinical severity score and reflux multisegment score (RMS) were assessed. ResultsA total of 51 patients (n = 102 limbs; age, 50.53 ± 14.5 years; 6 men) were included. The predominant clinical severity CEAP class was C3 in 54 of 102 limbs (52.9%). The mean VCSS was 14.3 ± 6.7. A severe RMS (≥3) was registered in 63.4% of the limbs. Of the 102 limbs, 51 (50%) presented with category 1, 27 (26.5%) with category 2, and 24 (23.5%) with category 3. Previous deep vein thrombosis (DVT) was associated with critical obstruction (odds ratio [OR], 3.65; 95% confidence interval [CI], 1.29-10.38; P = .015). The superficial and perforator venous systems had no association with the degree of IVOO. Deep venous reflux (DVR) had a significant association with significant IVOO (obstruction ≥50%; OR, 6.44; 95% CI, 2.19-18.93; P = .001) and critical IVOO (obstruction ≥80%; OR, 4.57; 95% CI, 1.70-12.27; P = .003) and a significant linear association with the IVOO degree and reflux in the femoral veins (P < .001) and popliteal vein (P = .008). Significant lesions were significantly more likely to develop in the left limb (OR, 5.76; 95% CI, 2.46-13.50; P < .001). After multivariate analysis, DVR remained a predictor for significant and critical obstruction (P < .003 and P < .012, respectively). Left limb and previous DVT remained as predictors for IVOO of ≥50% and ≥80% (P < .001 and P = .043, respectively). ConclusionsWe found a significant linear association between the degree of IVOO and reflux in the deep venous system on DU. Limbs with DVR, a severe RMS, loss of respiratory variation on DU, and previous DVT were more likely to be affected by IVOO of ≥50%, especially with left leg involvement.

Pregnancy outcomes in 16 women with previous catheter-directed thrombolysis and stenting for acute left iliofemoral deep vein thrombosis.

Whether iliac vein stents affect pregnancy or whether they will be affected by pregnancy was rarely reported in the literature. The aim of this study was to report our experience of pregnancy in women who received thrombolysis and stenting for a previous deep vein thrombosis (DVT). 16 patients with previous stenting for treatment of acute left iliofemoral DVT who had subsequent pregnancies were identified. Patient demographic information and outcomes were recorded. Duplex scanning and venography were performed as scheduled to evaluate the patency of stents in perinatal period and at 12months after delivery. None of the patients had a hemorrhagic complication and recurrent venous thromboembolism during their pregnancies and postpartum. No stent compression/occlusion and structural damage occurrence in all patients during follow-up. Pregnancy managed with prophylactic dalteparin resulted in no re-thrombotic events or structural damage to the iliac stents and did not appear to negatively affect pregnancy outcomes.

Twelve-month end point results from the evaluation of the Zilver Vena venous stent in the treatment of symptomatic iliofemoral venous outflow obstruction (VIVO clinical study)

BackgroundIn the present study, we evaluated the safety and effectiveness of the Zilver Vena venous stent in the treatment of patients with symptomatic iliofemoral outflow obstruction. MethodsThe VIVO clinical study was a prospective, nonrandomized, multicenter study that enrolled patients with symptomatic obstruction of one iliofemoral venous segment. Included were patients with Clinical, Etiological, Anatomical, Pathophysiological (CEAP) clinical classification of ≥3 or a Venous Clinical Severity Score (VCSS) pain score of ≥2. All patients received a self-expanding venous stent (Zilver Vena venous stent; Cook Ireland Ltd, Limerick, Ireland). The primary safety end point was 30-day freedom from major adverse events. The primary effectiveness end point was the 12-month rate of primary quantitative patency by venography as determined by the core laboratory. The secondary end point was the change in the VCSS from baseline to 1 and 12 months. Additional measures included freedom from clinically driven reintervention; change in the CEAP C classification, Venous Disability Score (VDS), and Chronic Venous Disease Quality of Life Questionnaire (CIVIQ) scores from baseline to 12 months; and stent durability measures. ResultsBetween December 2013 and October 2016, 243 patients (70% female; mean age, 53 ± 15 years; 67.5% with current or previous deep vein thrombosis) were enrolled at 30 institutions. Iliac vein compression by the iliac artery (n = 191; 78.6%) was the primary indication for stent placement. The mean lesion length was 98.6 ± 69.8 mm. The 30-day freedom from major adverse events rate was 96.7%, greater than the literature-defined performance goal of 87% (95% confidence interval [CI], 93.5%-98.6%; P < .0001). The 12-month primary quantitative patency rate was 89.9%, greater than the literature-defined performance goal of 76% (95% CI, 85.1%-93.4%; P < .0001). The change in the VCSS from baseline was −3.0 (95% CI, −3.5 to −2.6; P < .0001) at 1 month and −4.2 (95% CI, −4.7 to −3.7; P < .0001) at 12 months, demonstrating clinical improvement. Similarly, significantly (P < .0001) fewer symptoms over time (from preprocedure through 12 months) were measured using the clinical measures of VDS, CEAP C classification, and CIVIQ. The 12-month rate of freedom from clinically driven reintervention was 95.8% ± 1.3%. Through 12 months, no stent fractures and one clinical migration (Clinical Events Committee adjudicated the latter as technique-related due to device undersizing at placement) had occurred. ConclusionsThe 12-month results of the VIVO study have demonstrated the safety and effectiveness of the Zilver Vena venous stent for the treatment of symptomatic iliofemoral venous outflow obstruction, including clinical symptom improvement compared with baseline.

Prevalence and Associated Factors of Post-Thrombotic Syndrome in Patients With Deep Vein Thrombosis

Background: Post-thrombotic syndrome (PTS) occurs as a long-term complication of deep vein thrombosis (DVT). The aim of this study was to evaluate the prevalence of PTS and its associated factors in a cohort of patients with lower extremity DVT in Sri Lanka. Methods: A descriptive cross-sectional study was conducted in 80 patients who were assessed 6 months to 2 years after the acute episode of DVT. They were assessed using Vilalta scale to diagnose and grade the severity of PTS, anthropometry to calculate body mass index (BMI), duplex ultrasound scans, patients’ clinic record including international normalized ratio (INR) charts and an interviewer administered questionnaire. Categorical data were analyzed using Chi-square test and continuous variables were analyzed using analysis of variance (ANOVA) to determine association. Results: Prevalence of PTS was 45.5% (36/80) according to Villalta scale. Recurrent DVT (P = 0.0002), high BMI (P = 0.073), and previous venous in competence (P = 0.032), showed significant association with PTS. Proximal DVT demonstrated a strong association with PTS (P = 0.002). Residual thrombosis at 3 months was strongly associated with PTS (P &lt; 0.001), however, with a bias of very low sample with repeat duplex at 3 months post event. Awareness of PTS was extremely low (n = 0, 0%). Practice of elastic compression stockings (ECSs) use as a preventative measure of PTS failed to show any significant association (P = 0.61). Conclusions: This study showed a significant prevalence of PTS among patients with previous DVT. Development of PTS is probably multi factorial. A validated risk assessment tool should be used to identify at-risk patients as prevention is the best approach to this irreversible morbidity. J Curr Surg. 2022;12(2):38-44 doi: https://doi.org/10.14740/jcs459

Predictors of Clinical Outcomes of Pharmacomechanical Catheter-Directed Thrombolysis for Acute Iliofemoral Deep Vein Thrombosis: Analysis of a Multicenter Randomized Trial

PurposeTo identify the baseline patient characteristics that predict who will benefit from pharmacomechanical catheter-directed thrombolysis (PCDT) of acute iliofemoral deep vein thrombosis (DVT). Materials and MethodsIn the Acute Venous Thrombosis: Thrombus Removal with Adjunctive Catheter-Directed Thrombolysis (ATTRACT) multicenter randomized trial, 381 patients with acute iliofemoral DVT underwent PCDT and anticoagulation or anticoagulation alone. The correlations between baseline factors and venous clinical outcomes were evaluated over 24 months using post hoc regression analyses. Interaction terms were examined to evaluate for differential effects by treatment arm. ResultsPatients with clinically severe DVT (higher baseline Villalta score) experienced greater effects of PCDT in improving 24-month venous outcomes, including moderate or severe postthrombotic syndrome (PTS) (odds ratios [ORs] and 95% confidence intervals [CIs] per unit increase in the baseline Villalta scores were as follows: for PCDT, OR, 1.08 [95% CI, 1.01–1.15]; for control, OR, 1.20 [95% CI, 1.12–1.29]; Pinteraction = .03), PTS severity (between-arm differences in the Villalta [Pinteraction = .004] and Venous Clinical Severity Scale [VCSS] [Pinteraction = .002)] scores), and quality of life (between-arm difference in the Venous Insufficiency Epidemiological and Economic Study Quality of Life score; Pinteraction = .025). Patients with previous DVT had greater effects of PCDT on 24-month PTS severity than those in patients without previous DVT (mean [95% CI] between-arm difference in the Villalta score, 4.2 [1.56–6.84] vs 0.9 [−0.44 to 2.26], Pinteraction = .03; mean [95% CI] between-arm difference in the VCSS score, 2.6 [0.94–4.21] vs 0.3 [−0.58 to 1.14], Pinteraction = .02). The effects of PCDT on some but not all outcomes were greater in patients presenting with left-sided DVT (Villalta PTS severity, Pinteraction = .04; venous ulcer, Pinteraction = .0499) or a noncompressible popliteal vein (PTS, Pinteraction = .02). The effects of PCDT did not vary by sex, race, ethnicity, body mass index, symptom duration, hypertension, diabetes, or hypercholesterolemia. ConclusionsIn patients with acute iliofemoral DVT, greater presenting clinical severity (higher baseline Villalta score) and a history of previous DVT predict enhanced benefits from PCDT.

Portomesenteric Vein Thrombosis After Laparoscopic Sleeve Gastrectomy: A Single-Institution Report.

Laparoscopic sleeve gastrectomy (LSG) is an effective weight-loss operation. Portomesenteric vein thrombosis (PMVT) is an important complication of LSG. We identified four cases of PMVT after LSG at our institution in women aged 36-47 with BMIs ranging from 44-48kg/m2. All presented 8-19 days postoperatively. Common symptoms were nausea, vomiting, and abdominal pain. Thrombotic risk factors were previous deep vein thrombosis and oral contraceptive use. Management included therapeutic anti-coagulation, directed thrombolysis, and surgery. Complications were readmission, bowel resection, and bleeding. Discharge recommendations ranged from 3-6 months of anticoagulation using various anticoagulants. No consensus was reached on post-treatment hypercoagulable work up or imaging. All cases required multi-disciplinary approach with Surgery, Interventional Radiology, and Hematology. As PMVT is a rare but potentially morbid complication of LSG, further development of tools that quantify preoperative thrombotic risk and clear guidance regarding use of anticoagulants are needed for prevention and treatment of PMVT following LSG.

External validation of Villalta score in high-middle income country patients with deep vein thrombosis.

Post-thrombotic syndrome (PTS) is a late complication that does not have a cure yet, with a prevalence estimated between 20 to 75%, associated with previous deep vein thrombosis event. Although the Villalta score (VS) is the gold-standard clinical tool for diagnostic and prognostic evaluation of PTS, there are currently no VS intra-rater agreement established and no validation studies for VS’ application into Brazilian Portuguese. We sought to translate and validate VS reliability systematically; and, secondarily, to compare the ultrasound findings with the severity of PTS.We systematically translated the original VS into Brazilian Portuguese (BP). Fifty participants who underwent two outpatient visits were evaluated using the translated VS. We assessed its intra-rater and inter-rater agreement and compared BP VS versus CEAP clinical component (CEAP C), and the clinical PTS severity versus the duplex ultrasound (DUS) findings. The study and its report followed the Guidelines for Reporting Reliability and Agreement Studies.The intra-rater evaluation of VS grades had a simple Kappa coefficient of 0.73, and the simple Kappa coefficient inter-rater for VS grades was 0.67. When VS was compared to CEAP C, it established a remarkably high correlation over 0.9. There was difference among VS values compared to DUS initial deep vein thrombosis territory, with femoropopliteal showing higher values than distal veins. Higher VS values were correlated to DUS venous recanalization and reflux.There was a substantial inter-rater and intra-rater agreement when the BP VS was applied; and when compared to CEAP C, VS showed a high correlation. When VS grading was compared to DUS characteristics, there were significant statistical and clinical correlation, with presence of reflux and recanalization showing higher VS values. This external VS validation also changes the clinical practice allowing the VS use in a different population and establishes the VS intra-rater agreement.

POS1345 BEYOND PULMONARY ARTERY ANEURYSM; PULMONARY INVOLVEMENT IN BEHCET’S SYNDROME

BackgroundPulmonary involvement (PI) other than pulmonary artery aneurysm (PAA) in Behcet’s syndrome (BS) is still an area of investigation.ObjectivesHerein, we aimed to retrospectively evaluate all types of pulmonary involvement associated with Behcet’s syndrome (BS).MethodsAmong 800 BS patients according to International Study Group for Behcet’s Disease (ISG) criterias, 28 patients were selected based on their radiologic examination consistent with BS-related PI. Demographic features, other clinical manifestations of BS, treatment modalities and types of PI were analyzed.ResultsThe overall prevalence of PI was estimated 3,5% among all BS patients. PI was more common in males (82.1% vs 17.9%). Mean age for BS diagnosis and onset of PI were as following; 32 ± 10,9 and 37 ± 11,4 years. Deep vein thrombosis (DVT) was the most common accompanying vascular involvement (53,6%). PAA, pulmonary vasculitis (PV), and pulmonary thromboembolism (PTE) were seen in 7 (25%), 13 (46,3%), and 18 (64,4%) of patients, respectively. In 5 patients, intracardiac thrombosis was present simultaneously in the right ventricle. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) as first line. Warfarin used in 18 patients (Table 1) Mortality was seen in 3 patients during follow up (1 due to PAA bleeding, others with unknown causes).Table 1.Characteristics of pulmonary involvement in BS patientsMale/Female, (n), (%)23/5 (82,1%/17,9%)Age of diagnosis, years (Mean± SD)32 ± 10,9Age of pulmonary involvement, years (Mean ± SD)37 ± 11,4Oral ulcer, (n), (%)28 (100%)Genital ulcer, (n), (%)20 (71,4%)Osteofollicular lesion,(n), (%)20 (71,4%)Erythema nodosum, (n), (%)13 (46,4%)Uveitis, (n), (%)8 (28,6%)DVT, (n), (%)15 (53,6%)Cardiac involvement, (n), (%)5 (17,8%)Pulmonary involvement, (n)-PAA, (n), (%)287 (25%)-PV, (n), (%)13 (46,4%)-PTE, (n), (%)18 (64,3%)Immunosuppression (first line agents)26/28-Steroid, (n), (%)26/28-CYC, (n), (%)22/26 (84,6%)-AZA, (n), (%)4/26 (15%)Anticoagulation (warfarin), (n), (%)18PAA; pulmonary artery aneurysm, PTE; pulmonary thromboembolism, PV; pulmonary vasculitis, CYC; cyclophosphamide, AZA; azathioprine, DVT; deep vein thrombosisConclusionDespite the importance of PAA in BS patients, capillaritis and thromboembolism other than PAA may occur commonly in BS as well. Onset at young age, male gender and previous DVT seem to be significant risk factors for the development of PI.

P323 MYOCARDIAL INVOLVEMENT IN ANTI–PHOSPHOLIPID SYNDROME: A CASE OF A YOUNG BREASTFEEDING WOMAN

Abstract Introduction In Antiphospholipid Syndrome (APS) myocardial tissue can be involved through immune–mediated or thrombotic mechanisms, giving chest pain and increase of myocardial cytolysis markers. This may occur without any signs of myocardial injury at the moment of echocardiography, coronarography and cardiac magnetic resonance (CMR). The aim is to increase the awareness about this life–threating condition.Case summary. We present the case of 26–year–old woman few days after childbirth, affected by APS in anticoagulant therapy with previous deep vein thrombosis and without any other cardiovascular risk factors. She was symptomatic for intermittent chest–epigastric pain, fever and skin livedo reticularis. Lab Tests: TnI 750 ng/L, C–reactive protein 9.6 mg/dL, D–dimer 1693 µg/L; anemia; Antithyroid Antibodies and ANA 1:160 (SSB/LA). Blood cultures and COVID test were negative.Results. Echocardiography showed normal left/right ventricular function, but minimal pericardial effusion was present. Pulmonary Angio–CT revealed small thromboembolic event, ground–glass lungs compatible with hemorrhagic alveolitis.After a few days, the patient presented increased epigastric pain, headache, vomiting up to presenting a comatose state. Thrombotic or hemorrhagic events with cerebral CT and MR were excluded. Total–body CT was negative, except for peri–splenic and recto–uterine pouches.For the increase of TnI up to 4741 ng/L, the patient underwent coronary angiography which demonstrated non–obstructive coronary arteries. The assembled Neuro–Cardio–Rheumatology team suspected a rapidly developing Catastrophic APS which was developing quickly with multi–organ and life–threatening involvement.The patient underwent 4 cycles of plasmapheresis, intravenous human IgG and corticosteroids, with rapid clinical improvement. CMR subsequently demonstrated a small transmural late enhancement area on lateral left ventricle wall.The patient was discharged from the hospital on Day 6 post–therapies.Conclusions. APS may involve more organs, including myocardial tissue with different mechanisms of damage and high mortality rate. The presented case poses a multidisciplinary challenge, because thrombotic multiorgan microangiopathy may be not always diagnosed. Imaging methods such as CMR could be optimized with adenosine stress–CMR. Clinical attention is required among women with APS, to reach early diagnosis of myocardial thrombotic microangiopathy and to establish the best effective treatment.

Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension.

BackgroundAntiphospholipid syndrome is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. The aim of this study is to evaluate the efficacy and risk of pulmonary endarterectomy in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension.MethodsData were prospectively collected and retrospectively analyzed, for patients who underwent pulmonary endarterectomy between March 2011 and March 2020.ResultsSeventeen patients (4 male and 13 female) were identified. Thirteen patients had primary antiphospholipid syndrome and 4 had secondary antiphospholipid syndrome. The mean age was 34.82 ± 10.07 years and the mean time interval between the diagnosis and surgery was 26.94 ± 17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, 5 patients had a history of recurrent abortions, and 2 patients had hemoptysis. Following surgery, mean pulmonary artery pressure decreased from 47.82 ± 13.11 mm Hg to 22.24 ± 4.56 mm Hg (P < .001), and pulmonary vascular resistance improved from 756.50 ± 393.91 dyn/s/cm−5 to 298.31 ± 132.84 dyn/s/cm−5 (P < .001). There was no in-hospital mortality with a mean follow-up of 75.29 ± 40.21 months. The functional capacity of all patients improved from 269.46 ± 111.7 m to 490 ± 105.34 m on a 6-minute walking test.ConclusionsPulmonary endarterectomy is a safe and curative treatment in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced chronic thromboembolic pulmonary hypertension team is critical in the management of these unique patients.

P102 The hidden face of Behcet’s syndrome; central nervous system involvement, a single-center experience

Abstract Background/Aims Neurologic involvement in Behcet’s syndrome (BS) remains an unelucidated area that needs further attention. In this study, we aimed to analyze the patterns of neurologic involvement and evaluate their association with other clinical features. Methods 800 patients with BS diagnosis according to The International Criteria for Behcet’s Disease (ICBD) followed in our center were retrospectively screened and 55 patients with central nervous system involvement and 20 patients with non-vascular BS were enrolled in this study. These patients were classified as sinus vein thrombosis (SVT), parenchymal involvement, and mixed (SVT and parenchymal). These groups were compared in terms of demographic and clinical features. Results Thirty-two (58,2%) of the patients were male. Distribution of neurologic involvement were as follows; 22 (40%) with isolated sinus vein thrombosis (SVT), 25 (%45) with isolated parenchymal, and 8 (%15) with mixed pattern. Central nervous involvement was the diagnostic feature of BS in 25 patients. Headache was the most common symptom followed by neurologic deficits and dizziness (44%, 25%, 16%). The most common sites of involvement were subcortical and periventricular areas followed by brainstem (63% vs 37%). In SVT group, positive family history of BS (p &amp;lt; 0.001) and previous deep vein thrombosis (DVT) (p:0.004) were found higher. Smoking history, erythema nodosum, cardiac and pulmonary involvement were significantly higher in parenchymal group (p &amp;lt; 0.001) (Table 1). Conclusion Although brainstem involvement is considered as more characteristic in BS, white matter and periventricular involvement were also found as common in our study. Notably, these subgroup analyses may suggest the speculation that BS shows clusters even within the neuro-vascular involvement. Disclosure R. Yıldırım: Other; Reşit Yıldırım is a member of Rheumatology journal as an associate editor. M. Dinler: None. A.E. Arslan: None. N.S.Y. Bilge: None. T. Kaşifoğlu: None.

Deep Venous Thrombosis and Ulcers of Lower Limbs: Ultrasound Findings in 156 Patients.

Venous ulcers (VUs) of lower limbs affect 1% of Western population. In most cases, ultrasounds show only superficial venous insufficiency (SVI), but a deep venous insufficiency (DVI) may also be present without a history of deep vein thrombosis (DVT). To assess SVI and DVI in DVT-positive and DVT-negative patients with VU, a retrospective cohort of 123 patients entered the study (50 male and 73 female, minimum age 29 years and maximum age 90 years, and mean 70.6 years). In 56 patients (45.5%), ulcer was on the right leg, in 52 (42.3%) on the left leg, and in 15 patients (12.2%), ulcer was bilateral, resulting in a total number of 138 limbs in the study. Sixty-six patients suffered DVT, while in 72 anamnesis was negative. Color duplex ultrasound was performed on both limbs, which revealed insufficiencies of superficial and/or deep veins in 18 limbs which had not been affected by an ulcer or a previous DVT. So the study was on 156 limbs. SVI were substantially overlapping in two groups ( p -value = 0.593), while combined SVI and DVI was 72.5% in DVT positive limbs ( p -value = 0.001). In 70% of cases with a femoral vein insufficiency ( p -value = 0.036) or popliteal vein insufficiency (PVI) ( p -value 0,003), a DVT history was present. Of 18 limbs, although not affected by ulcer or previous DVT, eight were positive for DVI (two femoral veins and six popliteal veins). In the patient with VU, the history of DVT is a strong predictor of DVI insufficiency. In DVT-positive patients with ulcer, the number of "combined superficial and deep insufficiencies" appears to be particularly significant and surgical treatment must take this into account. A previous DVT has a low impact on great and small saphenous insufficiencies in ulcer patients; these were substantially overlapping in DVT-positive and DVT-negative patients. The 18 limbs with DVI and SVI without ulcer and DVT history were unexpected result. We think these patients must have a close follow-up to avoid the onset of a VU.

MALE PATIENT WITH DISSECTION OF THE POSTERIOR CEREBRAL ARTERY AND OCULOMOTOR NERVE PALSY: A CASE REPORT AND A REVIEW FROM THE LITERATURE

Spontaneous intracranial artery dissection is an uncommon and probably under diagnosed cause of stroke and subarachnoid hemorrhage that is defined by the occurrence of a hematoma in the wall of an intracranial artery. We introduce a case of dissection of the P2 segment of the posterior cerebral artery (PCA) presented with new onset headache and unilateral, left oculomotor nerve palsy. The patient had a diagnosed thrombophilia and previous deep vein thrombosis. Digital subtraction angiography (DSA) revealed dissection of the P1-P2 segment of the PCA with occlusion of the distal branches P3-P4. CT excluded acute stroke. The patient was put on anticoagulant therapy, with clinical improving after 2 weeks. The left third nerve had been distorted because of vascular compression.&nbsp; Conclusion: Isolated dissection of the PCA is rare. Multicenter prospective studies with standardized protocols for diagnosis, imaging, and follow-up of intracranial artery dissection are needed. Keywords: dissection, PCA

325 Dealing with cardiac amyloidosis diagnosis: keep calm and use the magnifying glasses!

Abstract Methods and results Case report— male, 71 years old. Past medical history—arterial hypertension, dyslipidemia, tobacco abuse. COPD on nocturnal CPAP therapy. Rheumatic polymyalgia on steroid therapy. Previous unprovoked deep vein thrombosis on anticoagulation with rivaroxaban. Bilateral carpal tunnel surgeries 8 years ago. Spontaneous left biceps tendon rupture 4 year ago. IgA kappa monoclonal gammopathy of undetermined significance (MGUS). Mild interventricular septum (IVS) hypertrophy on echocardiography since 2018. In 2019 IVS was 18 mm with granular sparkling appearance. In February 2020 he was hospitalized for initial heart failure and COPD exacerbation. In 2021 he developed worsening dyspnoea. He underwent cardiological evaluation in a spoke hospital and a cardiac magnetic resonance (CMR) suggested infiltrative cardiomyopathy. Bone scintigraphy showed moderate cardiac uptake (Perugini Score 2). Following haematological evaluation, fat pad biopsy was performed, and amyloid was detected on Congo red staining. Classification of the amyloid fibril protein was not performed. Bone marrow biopsy, even though of suboptimal quality, was negative for amyloid and for plasma cellular infiltration. Bone marrow aspirate showed 11% of plasma cells and multiple myeloma was therefore hypothesized. Recent medical history—he was evaluated in our Cardiac Amyloid Outpatient Clinic in May 2021. He was symptomatic for dyspnoea (NYHA class III) and exercise intolerance, diffuse osteo-muscolar pain, and extremities paresthesia. His blood pressure was on the low side of normality with necessity of anti-hypertensive therapy downgrading. Signs and symptoms of hematological disease were not present. We required to analyse the fat pad specimen in order to perform amyloid fibril protein typing; with immunoelectron microscopy, transthyretin (TTR) was identified as the amyloid fibrils precursor (no light chains could be identified). We considered performing endomyocardial biopsy to exclude the coexistence of ATTR amyloidosis and light chains (AL) amyloidosis in the heart but, given the history, clinical picture, and fat pad biopsy results, we felt that cardiac ATTR was the most probable diagnosis and we decided to proceed with a close cardiological and haematological follow-up. TTR genetic testing is ongoing. Conclusions ATTR cardiac amyloidosis is an emerging cause of heart failure, especially with preserved ejection fraction, in the older population. However, these patients frequently present with dysproteinemias and bone marrow abnormalities, up to multiple myeloma, raising the issue of differential diagnosis between ATTR and AL amyloidosis. According to the latest European Consensus Document, in the presence of cardiac uptake at bone scintigraphy (Grades 1–3) and positive haematologic tests, histological confirmation (usually cardiac) is necessary to subtype amyloid infiltration. In our case, the patient had positive Congo Red-stained fat pad biopsy, but the typing of the amyloid deposition was not performed. After referral to a Center with a Cardiac Amyloid Outpatient Clinic with a specialized Pathology Unit, we could further proceed with diagnostic workup and identify the amyloid deposition as ATTR; of note, fat pad biopsy is positive in just 15–25% of ATTR amyloidosis. Moreover, close collaboration with Hematology was necessary to assess the risk of AL amyloidosis and to provide a close and targeted follow-up. Endomyocardial biopsy was not performed after consideration of the various elements suggestive for ATTR cardiac amyloidosis, but the patient will be evaluated periodically and closely to potentially reassess this decision.