Spontaneous dissection of the superior mesenteric artery related to COVID-19.

Background: Spontaneous peripheral dissections are rare, and in a substantial number of cases, the underlying aetiology remains unclear. Patients and methods: We report the case of a 63-year-old male patient with a recent asymptomatic SARS-CoV-2 infection who presented with sudden-onset intermittent abdominal pain. Imaging studies revealed a dissection of the superior mesenteric artery (SMA) and large-vessel vasculitis involving the SMA as well as the carotid, subclavian, axillary and femoropopliteal arteries. In the absence of other predisposing factors, we supposed an association with prior COVID-19 and performed a systematic review of the literature to search for similar cases with arterial dissection related to acute or recent SARS-CoV-2 infection. Results: We identified 25 cases, including ours: 13 males and 12 females, with a median age of 48 years. In 22/25 patients, arterial dissection occurred within 4 weeks after the diagnosis of COVID-19 and involved the cerebral (11/25; 44%), coronary (10/25; 40%), splanchnic (3/25; 12%) and renal (2/25; 8%) arteries. Conclusions: Although initially known for its respiratory manifestations, it has become evident that SARS-CoV-2 not only infects pneumocytes but also enters the vascular endothelium, leading to endothelial dysfunction and hypercoagulability and - as shown in our case - large-vessel vasculitis, which may predispose patients to intramural haemorrhage and arterial dissection.

Endovascular interventions in outpatient care.

Endovascular arterial revascularisations for the treatment of symptomatic peripheral arterial disease are constantly increasing in importance and number due to the changing age structure and high numbers of comorbidities in the German population. Patients with peripheral artery disease are often at increased risk for peri- and post-procedural complications including severe cardiovascular events. Due to limited financial and human resources and considerable risks of hospitalization, endovascular interventions that were previously reserved for hospitalized patients are now progressively considered to be performed as day case procedures. More than one third of these procedures are performed in Germany by internists with a specialization in angiology. In the current position paper the German Society of Angiology endorsed by the European Society of Vascular Medicine, summarizes the requirements and risk factors to be considered for the planning, safe performance and post procedural care of endovascular revascularizations in outpatients. The performance of endovascular procedures for peripheral artery disease both in hospitalised and outpatients should be accompanied by a mandatory quality assurance process that should not only capture procedural data, but also require documentation of complications and longterm outcome.

Venous thromboembolism in patients with COVID-19 (SARS-CoV-2 infection) - a position paper of the German Society of Angiology (DGA).

As observed in other infections with a systemic inflammatory response, severe COVID-19 is associated with hypercoagulability and a prothrombotic state. Currently, there is growing evidence that pulmonary embolism and thrombosis contribute to adverse outcomes and increased mortality in critically ill patients with COVID-19. The optimal thromboprophylactic regimen for patients with COVID-19 is not known. Whereas pharmacologic thromboprophylaxis is generally recommended for all hospitalized COVID-19 patients, adequate dosing of anticoagulants remains a controversial issue. Therefore, we summarize current evidence from the available literature and, on behalf of the German Society of Angiology (DGA), we aim to provide advice to establish an improved and more uniform strategy for thromboprophylaxis in patients with COVID-19.

Antiphospholipid syndrome - an update.

Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia. It is characterized by the presence of antiphospholipid antibodies (APL) that are directed against phospholipid-binding plasma proteins, such as beta-2-glycoprotein I (b2GPI). Its main manifestations are recurrent vascular thromboses (so-called "thrombotic APS") and pregnancy complications ("obstetric APS"). According to the current consensus criteria, a persistently positive functional lupus anticoagulant (LA) assay and/or the presence of anti-b2GPI and/or anti-cardiolipin antibodies, together with clinical symptoms, is mandatory for the diagnosis of APS. Other clinical features, such as thrombocytopenia, Coombs-positive haemolytic anaemia, heart valve disease, renal microangiopathy and neurologic disorders are also common in APL-positive patients. APS can be associated with other autoimmune disorders, such as systemic lupus erythematosus. In rare cases, catastrophic APS (CAPS) occurs, with the development of excessive thrombosis at multiple sites, usually affecting small vessels and leading to multi-organ dysfunction and organ failure. Treatment usually comprises antithrombotic therapy using antiplatelet and anticoagulant agents. However, there is no consensus concerning the intensity or duration of therapy. Despite apparently adequate anticoagulation, the risk of recurrent thrombosis remains high. For patients with CAPS, a combined therapeutic approach that includes anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulin seems to be the best treatment option. Keywords: Antiphospholipid syndrome, lupus anticoagulants, anti-cardiolipin, anti-beta-2-glycoprotein I, vascular thrombosis, pregnancy complication.

Raynaud's phenomenon and digital ischaemia--pharmacologic approach and alternative treatment options.

The primary goal of therapy is to reduce the frequency and intensity of Raynaud's attacks and to minimize the related morbidity rather than to cure the underlying condition. Treatment strategies depend on whether Raynaud's phenomenon (RP) is primary or secondary. All patients should be instructed about general measures to maintain body warmth and to avoid triggers of RP attacks. Pharmacologic intervention can be useful for patients with severe and frequent RP episodes that impair the patient's quality of life. Calcium channel blockers are currently the most prescribed and studied medications for this purpose. There has been limited evidence for the efficacy of alpha-1-adrenergic receptor antagonists, angiotensin receptor blockers, topical nitrates or fluoxetine to treat RP. The intravenously administered prostacyclin analogue iloprost can reduce the frequency and severity of RP attacks and is considered a second-line therapy in patients with markedly impaired quality of life, critical digital ischaemia and skin ulcers who are at risk for substantial tissue loss and amputation. Phosphodiesterase inhibitors (e.g., sildenafil) can also improve RP symptoms and ulcer healing whereas endothelin-1 receptor antagonists (e.g., bosentan) are mainly considered treatment options in secondary prevention for patients with digital skin ulcers related to systemic sclerosis. However, their use in clinical practice has been limited by their high cost. Antiplatelet therapy with low-dose aspirin is recommended for all patients who suffer from secondary RP due to ischaemia caused by structural vessel damage. Anticoagulant therapy can be considered during the acute phase of digital ischaemia in patients with suspected vascular occlusive disease attributed to the occurrence of new thromboses. In patients with critical digital ischaemia, consideration should be given to hospitalisation, optimisation of medical treatment in accordance with the underlying disease and evaluation for a secondary, possibly reversible process that is causing or aggravating the clinical symptoms.

Treatment of pregnancy-associated venous thromboembolism - position paper from the Working Group in Women's Health of the Society of Thrombosis and Haemostasis (GTH).

Venous thromboembolism (VTE) is a major cause of maternal morbidity during pregnancy and the postpartum period. However, because there is a lack of adequate study data, management strategies for pregnancy-associated VTE must be deduced from observational stu-dies and extrapolated from recommendations for non-pregnant patients. In this review, the members of the Working Group in Women's Health of the Society of Thrombosis and Haemostasis (GTH) have summarised the evidence that is currently available in the literature to provide a practical approach for treating pregnancy-associated VTE. Because heparins do not cross the placenta, weight-adjusted therapeutic-dose low molecular weight heparin (LMWH) is the anticoagulant treatment of choice in cases of acute VTE during pregnancy. No differences between once and twice daily LMWH dosing regimens have been reported, but twice daily dosing seems to be advisable, at least peripartally. It remains unclear whether determining dose adjustments according to factor Xa activities during pregnancy provides any benefit. Management of delivery deserves attention and mainly depends on the time interval between the diagnosis of VTE and the expected delivery date. In particular, if VTE manifests at term, delivery should be attended by an experienced multidisciplinary team. In lactating women, an overlapping switch from LMWH to warfarin is possible. Anticoagulation should be continued for at least 6 weeks postpartum or for a minimum period of 3 months. Although recommendations are provided for the treatment of pregnancy-associated VTE, there is an urgent need for well-designed prospective studies that compare different management strategies and define the optimal duration and intensity of anticoagulant treatment.

Diagnosis of pregnancy-associated venous thromboembolism - position paper of the Working Group in Women's Health of the Society of Thrombosis and Haemostasis (GTH).

Pregnancy and the postpartum period are associated with an increased risk of venous thromboembolism (VTE). Over the past decade, new diagnostic algorithms have been established, combining clinical probability, laboratory testing and imaging studies for the diagnosis of deep vein thrombosis (DVT) and pulmonary embolism (PE) in the non-pregnant population. However, there is no such generally accepted algorithm for the diagnosis of pregnancy-associated VTE. Studies establishing clinical prediction rules have excluded pregnant women, and prediction scores currently in use have not been prospectively validated in pregnancy or during the postpartum period. D-dimers physiologically increase throughout pregnancy and peak at delivery, so a negative D-dimer test result, based on the reference values of non-pregnant subjects, becomes unlikely in the second and third trimesters. Imaging studies therefore play a major role in confirming suspected DVT or PE in pregnant women. Major concerns have been raised against radiologic imaging because of foetal radiation exposure, and doubts about the diagnostic value of ultrasound techniques in attempting to exclude isolated iliac vein thrombosis grow stronger as pregnancy progresses. As members of the Working Group in Women's Health of the Society of Thrombosis and Haemostasis (GTH), we summarise evidence from the available literature and aim to establish a more uniform strategy for diagnosing pregnancy-associated VTE.

Monitoring anticoagulant therapy with vitamin K antagonists in patients with antiphospholipid syndrome.

Because of the possible interference of antiphospholipid antibodies (APL) with the phospholipid component of thromboplastin reagents, concerns have been raised about the validity of international normalized ratio (INR) testing to monitor anticoagulant therapy with vitamin K antagonists in patients with antiphospholipid syndrome (APS). To investigate the reliability of the INR, we determined the INR using various prothrombin time (PT) assays and compared the results with those of a chromogenic factor X (CFX) assay. The study cohort consisted of 40 APS patients and 100 APL-negative patients who were on anticoagulant therapy for reasons other than APS. The agreement (i.e. the percentage of patients with a difference ≤0.5 INR units) between the PT-derived INR and CFX-derived INR equivalents was only moderate in both patient groups. The best agreement with CFX-derived INR equivalents was observed for the Thromborel S reagent in APS patients (69.1 %) and for Neoplastin Plus in APL-negative patients (72.0 %). Regarding the results for the point-of-care system CoaguChek XS, an agreement between the INR and the CFX-derived INR equivalent was less frequently observed in the APS patients (55.6 vs. 67.8 %; p = 0.050). When considering all 3058 pairs of INR tests within the international sensitivity index (ISI)-calibrated range of 1.5 to 4.5 s, we did not observe a higher variability of INR values in either the APS patient group or the subgroup of APS patients positive for lupus coagulants compared with the APL-negative controls. In conclusion, monitoring vitamin K antagonists (VKA) therapy with laboratory INR measurements seems to be suitable for the majority of APS patients.

Raynaud’s phenomenon - assessment and differential diagnoses.

Raynaud’s phenomenon (RP) is characterised by paroxysmal reversible episodes of vasospasm, usually involving peripheral small vessels of the fingers or toes and resulting in a triple-colour change starting with pallor and followed by cyanosis and erythema. Attacks are typically triggered by cold or emotional stress. The diagnosis of RP can be made on the basis of the patient’s clinical symptoms. Primary RP occurs without underlying disease and is considered a benign condition. A normal erythrocyte sedimentation rate, negative testing for antinuclear antibodies, normal nailfold capillaries and the absence of structural micro- or macrovascular damage and other diseases lead to the diagnosis of primary RP. Digital photoplethysmography and pulse contour analysis can be used as an additional tool to exclude structural macro- or microvascular disease. In contrast, secondary RP is associated with other diseases, mainly connective tissue diseases such as systemic sclerosis. If there is a suspicion of secondary RP, a thorough laboratory and vascular assessment is required to make the diagnosis of underlying disease. Acrocyanosis and erythromelalgia are additional functional vascular disorders that can be easily distinguished when patients are carefully assessed for their history and clinical symptoms.

Thrombophilia and risk of VTE recurrence according to the age at the time of first VTE manifestation.

BACKGROUND: Whether screening for thrombophilia is useful for patients after a first episode of venous thromboembolism (VTE) is a controversial issue. However, the impact of thrombophilia on the risk of recurrence may vary depending on the patient's age at the time of the first VTE. PATIENTS AND METHODS: Of 1221 VTE patients (42 % males) registered in the MAISTHRO (MAin-ISar-THROmbosis) registry, 261 experienced VTE recurrence during a 5-year follow-up after the discontinuation of anticoagulant therapy. RESULTS: Thrombophilia was more common among patients with VTE recurrence than those without (58.6 % vs. 50.3 %; p = 0.017). Stratifying patients by the age at the time of their initial VTE, Cox proportional hazards analyses adjusted for age, sex and the presence or absence of established risk factors revealed a heterozygous prothrombin (PT) G20210A mutation (hazard ratio (HR) 2.65; 95 %-confidence interval (CI) 1.71 - 4.12; p < 0.001), homozygosity/double heterozygosity for the factor V Leiden and/or PT mutation (HR 2.35; 95 %-CI 1.09 - 5.07, p = 0.030), and an antithrombin deficiency (HR 2.12; 95 %-CI 1.12 - 4.10; p = 0.021) to predict recurrent VTE in patients aged 40 years or older, whereas lupus anticoagulants (HR 3.05; 95%-CI 1.40 - 6.66; p = 0.005) increased the risk of recurrence in younger patients. Subgroup analyses revealed an increased risk of recurrence for a heterozygous factor V Leiden mutation only in young females without hormonal treatment whereas the predictive value of a heterozygous PT mutation was restricted to males over the age of 40 years. CONCLUSIONS: Our data do not support a preference of younger patients for thrombophilia testing after a first venous thromboembolic event.

Management of complications related to central venous catheters in cancer patients: an update.

Central venous catheters (CVCs) are important for the treatment of patients with cancer, especially in the perioperative and palliative care settings. These devices not only allow for the administration of chemotherapy, parenteral nutrition, and other intravenous therapies, but they may also improve the patients' quality of life by reducing the need for repeated peripheral venipunctures. Thrombotic and infectious complications are common, especially in the long-term use of CVCs. There are different types of thrombotic complications associated with CVCs, that is, a thrombotic occlusion of the catheter, a mural thrombus at the catheter tip and classical deep vein thrombosis, which occurs most frequently in the upper extremity where the majority of long-term catheters are inserted. Infections are common complications associated with CVCs. Patients with cancer who receive intensive chemotherapy and those patients who undergo hematopoietic stem cell transplantation have a markedly increased risk for insertion site and bloodstream infections. In this review, the epidemiology and risk factors that predispose patients to CVC-related thrombosis and infection are discussed. The diagnostic and therapeutic options according to the published data and the current guidelines are summarized and data for establishing primary and secondary preventative strategies are provided.

Point-of-care coagulation testing for assessment of the pharmacodynamic anticoagulant effect of direct oral anticoagulant.

BACKGROUND: This investigation was carried out with already available point-of-care testing (POCT) systems for coagulation parameters to evaluate the qualitative and semiquantitative determination of the time- and concentration-dependent anticoagulant effects of the direct oral anticoagulants rivaroxaban and dabigatran. METHODS: The whole blood prothrombin time (PT), activated partial thromboplastin time (aPTT), and activated clotting time (ACT) were determined using the GEM PCL Plus coagulation system. Whole blood PT was also measured on the CoaguCheck XS instrument. In addition, PT and aPTT values were obtained in citrated plasma using the PT reagent Neoplastin Plus and the STA APTT reagent. Drug concentrations of rivaroxaban and dabigatran were determined with a chromogenic anti-Xa assay and the hemoclot assay, which are reported to have good agreement with liquid chromatography coupled with tandem mass spectrometry measurements. POCT was performed in 27 consecutive patients who received rivaroxaban 10, 15, or 20 mg once daily and in 15 patients receiving dabigatran 110 or 150 mg twice daily. Blood samples were collected predose and 2 hours after observed drug intake at steady state. RESULTS: Two hours after observed rivaroxaban administration, the whole blood PT measured on the GEM PCL Plus was prolonged by an average of 64.5% in comparison with predose levels. Less differentiation was observed for rivaroxaban when the PT was measured on the CoaguCheck XS instrument or in plasma (prolongation of 24.1% and 36.8%, respectively). After 2 hours observed dabigatran administration, the whole blood aPTT was comparable with plasma values and was prolonged by 23.5% in comparison with trough values. Significant concentration-dependent prolongations of the activated clotting time were observed to different extents for both direct anticoagulants. CONCLUSIONS: Direct oral anticoagulants display variable ex vivo effects on different POCT-assays. POCT for aPTT is sensitive to increased concentrations of dabigatran, whereas the PT-POCT assessed with test systems such as the GEM PCL Plus may be helpful to measure the pharmacodynamic anticoagulant effects of rivaroxaban in emergency clinical situations.

Current Topics from the Revised German S2k Guideline on Diagnosis and Treatment of Venous Thromboembolism.

It is an honor and a great pleasure for us to be guest editors for this special issue of Hämostaseologie - Progress in Haemostasis, which addresses important issues surrounding the complex of venous thromboembolism (VTE). In February 2023, the revised guideline on "Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism" has been published on the website of the Association of the Scientific Medical Societies in Germany (AWMF)1. This guideline was drawn up under the leadership of the German Society of Angiology (DGA), and representatives of 17 scientific societies contributed to its content. As an S2k guideline, its recommendations are consensus based and are the result of a systematic review and evaluation of current evidence and consideration of the benefits and harms of diagnostic and therapeutic options. In this special issue, guideline authors provide a comprehensive overview of selected guideline topics which might be of clinical relevance to our readers and our community of haemostaseologists.

[Venous thromboembolism - was is new in the revised AWMF guideline?] / Venöse Thromboembolie ­ was ist neu in der aktualisierten AWMF-Leitlinie?

For the diagnosis of a lower-extremity deep vein thrombosis (LEDVT), venous duplex ultrasound is the method of first choice. If a qualified ultrasonography is not timely available, D-dimer testing, and limited ultrasound protocols (point-of-care ultrasound, POCUS) can contribute to therapeutic decision-making when clinical probability is low. A DOAC-based treatment regimen is preferable to a vitamin K antagonist for both acute therapy and secondary prophylaxis of venous thromboembolism (VTE). Treatment with DOACs is unproblematic up to a body weight (BW) of 120 kg or a body mass index (BMI) of 40 kg/m². Weight restrictions are no longer recommended for apixaban and rivaroxaban, but determination of DOAC trough and peak levels is recommended in the extremely obese and patients after bariatric surgery. In cancer-associated VTE, the direct factor Xa inhibitors are a good and safe alternative to low-molecular weight heparins (LMWH) for many patients; the adherence to oral therapy is also higher. Meaningful initial documentation and structured follow-up after LEDVT and pulmonary embolism (PE) are important in order to make an individualized risk-benefit assessment at the end of the therapy phase with regard to continued pharmacological secondary prophylaxis and to reassess patients' symptoms indicating post-thrombotic syndrome (PTS) or chronic thromboembolic pulmonary hypertension (CTEPH).

Recurrent Venous Thromboembolism in Patients on Anticoagulation: An Update Based on the Revised AWMF S2k Guideline.

In the recently updated German S2k Guideline "Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism," a new chapter was incorporated about recurrent venous thromboembolism (VTE) in patients on anticoagulation treatment. Despite the high efficacy of anticoagulation in most patients, approximately 2% experience a recurrent VTE event while receiving anticoagulant drugs. The proper diagnosis of the recurrent VTE is important and possible only with the knowledge of localization and thrombus burden of the primary VTE event. Possible reasons for recurrent VTE events in patients on anticoagulation are non-adherence to medication, sub-therapeutic drug levels due to resorption disorders or drug interactions, or concomitant disease with high thrombogenicity. Cancer is the most common underlying disease, but it is important to investigate and understand possible other causes whenever a breakthrough VTE event occurs. This results in the recommendation that in patients with VTE recurrence on therapeutic anticoagulation, in particular, the presence of malignant disease, antiphospholipid syndrome, and rare diseases like paroxysmal nocturnal hemoglobinuria or Behçet's disease should be considered. For VTE recurrence during heparin therapy, heparin-induced thrombocytopenia type II needs to be ruled out, even if platelet counts are within the normal range. Although the mechanisms of recurrence on anticoagulation can be evaluated in a certain degree, clinical evidence for the management of recurrent VTE in anticoagulated patients is minimal and mainly based on expert opinion. Switching anticoagulant medication and intensifying anticoagulant treatment are possible options.

Compression Therapy in Acute Deep Venous Thrombosis of the Lower Limb and for the Prevention of Post-Thrombotic Syndrome.

BACKGROUND: After an acute deep venous thrombosis (DVT) of the lower limb, 20% to 63% of patients develop post-thrombotic syndrome (PTS). In this review, we address the efficacy of compression therapy in the treatment of acute DVT of the lower limb, and for the prevention of PTS. METHODS: 12 randomized controlled trials (RCTs) and one meta-analysis, with a total of 3751 patients, were identified in a structured literature search. RESULTS: Two RCTs showed that adding compression therapy to drug treatment in the first 9 days of the acute phase of lower limb DVT led to more rapid pain relief (p<0.050) and less swelling (remaining difference in circumference, 1 cm versus 3 cm, p<0.050). As for the prevention of PTS, four RCTs showed a short-term benefit or no benefit of compression therapy. In three further RCTs, medical compression stockings (MCS) brought about a 16% to 27% absolute reduction of the frequency and severity of PTS (47% vs. 20 %, p<0.001; 40% vs. 21% (95% confidence intervals [29.9; 50.1] and [12.7; 29.5], respectively; and 58% vs. 42%, relative risk [RR] 0.73 [0,55; 0.96]). The benefit of MCS was also confirmed in a recent meta-analysis (RR 0.66 [0.44; 0.99], I2 = 88%). Thigh-length MCS were not superior to knee-length MCS for the prevention of PTS (33% vs. 36%, hazard ratio [HR] 0.93 [0.62; 1.41]). Individual, symptomoriented tailoring of the duration of treatment was not inferior to a fixed treatment duration of 24 months (29% vs. 28%; odds ratio [OR] 1.06 [0.78;1.44]). CONCLUSION: Compression therapy relieves symptoms in acute DVT and lessens the frequency and severity of PTS. It is therefore recommended as standard treatment.

Management of Deep Vein Thrombosis: An Update Based on the Revised AWMF S2k Guideline.

Deep vein thrombosis (DVT) and pulmonary embolism (PE) are the most common manifestations of venous thromboembolism (VTE). Most DVTs affect the lower-extremity veins. Since the symptoms of DVT are non-specific, a prompt and standardised diagnostic work-up is essential to minimise the risk of PE in the acute phase and to prevent thrombosis progression, post-thrombotic syndrome and VTE recurrence in the long-term. Only recently, the AWMF S2k guidelines on Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism have been revised. In the present article, we summarize current evidence and guideline recommendations focusing on lower-extremity DVT (LEDVT). Depending on whether the diagnostic work-up is performed by a specialist in vascular medicine or by a primary care physician, different diagnostic algorithms are presented that combine clinical probability, D-dimer testing and diagnostic imaging. The diagnosis of ipsilateral recurrent DVT poses a particular challenge and is presented in a separate algorithm. Anticoagulant therapy is an essential part of therapy, with current guidelines clearly favouring regimens based on direct oral anticoagulants over the traditional sequential therapy of parenteral anticoagulants and vitamin K antagonists. For most DVTs, a duration of therapeutic-dose anticoagulation of at least 3 to 6 months is considered sufficient, and this raises the question of the risk of VTE recurrence after discontinuation of anticoagulation and the need for secondary prophylaxis in the long-term. Depending on the circumstances and trigger factors that have contributed to the occurrence of DVT, management strategies are presented that allow decision-making taking into account the individual bleeding risk and patient's preferences.

Prevalence of thrombophilia according to age at the first manifestation of venous thromboembolism: results from the MAISTHRO registry.

Thrombophilia is a well-established risk factor for a venous thromboembolic event (VTE), and it has been proposed that hereditary thrombophilia may substantially contribute to the development of VTE in young patients. We aimed to analyse the prevalence of thrombophilia with special regard to the age of VTE manifestation. The study cohort consisted of 1490 patients (58% females) with a median age 43 years at the time of their first VTE. At least one thrombophilic disorder was identified in 50·1% of patients. The probability of detecting a hereditary thrombophilia declined significantly with advancing age (from 49·3% in patients aged 20 years and younger to 21·9% in patients over the age of 70 years; P < 0·001). This may be primarily attributed to the decreasing frequencies of the F5 R506Q (factor V Leiden) mutation and deficiencies of protein C or protein S with older age at the time of the initial VTE event. Moreover, thrombophilia was more prevalent in unprovoked compared with risk-associated VTE (57·7% vs. 47·7%; P = 0·001). The decline in the prevalence of hereditary thrombophilia with older ages supports the use of a selected thrombophilia screening strategy dependent on age and the presence or absence of additional VTE risk factors.

Patients with inferior vena cava thrombosis frequently present with lower back pain and bilateral lower-extremity deep vein thrombosis.

BACKGROUND: Inferior vena cava (IVC) thrombosis is rare, and data about the clinical presentation of patients are scarce. Therefore, we reviewed all cases of IVC thrombosis consecutively registered in the MAISTHRO (MAin-ISar-THROmbosis) database and described patients’ characteristics in terms of their clinical presentations in the acute setting of IVC thrombosis. PATIENTS AND METHODS: From the MAISTHRO registry, which enrolled 1470 consecutive patients with documented histories of venous thromboembolism, we identified 60 patients (0,4 %; females 60 %) with IVC thrombosis and 888 patients (60.4 %; females 55 %) with isolated lower-extremity deep vein thrombosis (LE-DVT). RESULTS: The median age at the time of IVC thrombosis manifestation was 36.5 years (9 to 83). IVC thrombosis was the initial VTE event in 47 patients (78 %). In the majority of cases, IVC thrombosis extended to the lower-extremity veins, and both lower extremities were affected in 17 cases (28 %). The initial clinical symptom of IVC thrombosis was lower back or abdominal pain which preceded typical symptoms of LE-DVT in 29 (48 %) patients. Symptomatic pulmonary embolism was more frequently observed in IVC thrombosis patients when compared to a sex- and age-matched subgroup of LE-DVT patients, although the difference was not significant (27 % vs. 12 %; p = 0.064). Malignant disease was the only established VTE risk factor with a higher prevalence among IVC thrombosis patients than patients with isolated LE-DVT (27 % vs. 9 %; p = 0.015). Congenital IVC anomalies were identified in another eight IVC thrombosis patients (13 %). CONCLUSIONS: IVC thrombosis should be considered a differential diagnosis for inexplicable lower back or abdominal pain especially in young patients. Malignant disease and congenital IVC anomalies seem to be predisposing factors for thrombosis involving the inferior vena cava.

[Thromboembolic disease - the angiologist's point of view]. / Thromboembolische Erkrankungen aus angiologischer Sicht.

Thrombosis and embolism are among the most common causes of death worldwide and, in addition to venous thromboembolism with the two main manifestations of deep venous thrombosis and pulmonary embolism, also include arterial thrombotic disease such as myocardial infarction, stroke and systemic arterial embolism. It is often the interaction of several predisposing factors that leads to the formation of an intravascular thrombus. Changes in the vascular wall, the blood flow and the composition of the blood (Virchow's triad) play a decisive role in both the arterial and venous vascular systems. The management of thromboembolic diseases requires a dedicated clarification of the cause and classification of the disease process in order to minimize the risk of recurrence and embolic complications through differentiated antithrombotic therapy. For this purpose, a risk-benefit analysis based on the individual case and regular reassessment are of particular importance. In this article, the pathophysiological concepts of venous and arterial thrombosis and the main therapeutic implications resulting from this are reviewed.