Hamostaseologie最新文献

Venous thromboembolism (VTE) is the third most common cardiovascular disease. For most patients, the standard of treatment has long consisted on low-molecular-weight heparin followed by vitamin K antagonists, but a number of clinical trials and, subsequently, post-marketing studies have shown that direct oral anticoagulants (DOACs) with or without lead-in heparin therapy are effective alternatives with fewer adverse effects. This evidence has led to important changes in the guidelines on the treatment of VTE, including pulmonary embolism (PE), with the DOACs being now recommended as the first therapeutic choice. Additional research has contributed to identifying low-risk PE patients who can benefit from outpatient management or from early discharge from the emergency department with DOAC treatment. There is evidence to support the use of DOACs in intermediate-risk PE patients as well as in high-risk patients receiving thrombolytic treatment. The use of DOACs has also been proven to be safe and effective in special populations of PE patients, such as patients with renal impairment, liver impairment, and cancer.

High-risk pulmonary embolism (PE) refers to a large embolic burden causing right ventricular failure and hemodynamic instability. It accounts for approximately 5% of all cases of PE but contributes significantly to overall PE mortality. Systemic thrombolysis is the first-line revascularization therapy in high-risk PE. Surgical embolectomy or catheter-directed therapy is recommended in patients with an absolute contraindication to systemic thrombolysis. Extracorporeal membrane oxygenation (ECMO) provides respiratory and hemodynamic support for the most critically ill PE patients with refractory cardiogenic shock or cardiac arrest. The complex management of these individuals requires urgent yet coordinated multidisciplinary care. In light of existing evidence regarding the utility of ECMO in the management of high-risk PE patients, a number of possible indications for ECMO utilization have been suggested in the literature. Specifically, in patients with refractory cardiac arrest, resuscitated cardiac arrest, or refractory shock, including in cases of failed thrombolysis, venoarterial ECMO (VA-ECMO) should be considered, either as a bridge to percutaneous or surgical embolectomy or as a bridge to recovery after surgical embolectomy. We review here the current evidence on the use of ECMO as part of the management strategy for the highest-risk presentations of PE and summarize the latest data in this indication.

Acute pulmonary embolism (PE) remains a significant cause of morbidity and requires prompt diagnosis and management. The prognosis of affected patients depends on the clinical severity. Therefore, risk stratification is imperative for therapeutic decision-making. Patients with high-risk PE need intensive care. These include patients who have successfully survived resuscitation, with obstructive shock or persistent haemodynamic instability. Bedside diagnostics by means of sonographic procedures are of outstanding importance in this high-risk population. In addition to the treatment of hypoxaemia with noninvasive and invasive techniques, the focus is on drug-based haemodynamic stabilisation and usually requires the elimination or reduction of pulmonary vascular thrombotic obstruction by thrombolysis. In the event of a contraindication to thrombolysis or failure of thrombolysis, various catheter-based procedures for thrombus extraction and local thrombolysis are available today and represent an increasing alternative to surgical embolectomy. Mechanical circulatory support systems can bridge the gap between circulatory arrest or refractory shock and definitive stabilisation but are reserved for centres with the appropriate expertise. Therapeutic strategies for patients with intermediate- to high-risk PE in terms of reduced-dose thrombolytic therapy or catheter-based procedures need to be further evaluated in prospective clinical trials.

Splanchnic or visceral vein thromboses (VVTs) are atypical thrombotic entities and include thrombosis of the portal vein, hepatic veins (Budd-Chiari syndrome), mesenteric veins, and splenic vein. All VVTs have in common high 30-day mortality up to 20% and it seems to be difficult to diagnose VVT early because of their rarity and their wide spectrum of unspecific symptoms. VVTs are often associated with myeloproliferative neoplasia, thrombophilia, and liver cirrhosis. VVT is primarily diagnosed by sonography and/or computed tomography. In contrast to venous thromboembolism, D-dimer testing is neither established nor helpful. Anticoagulation is the first-line therapy in patients with stable circulation and no evidence of organ complications. Anticoagulation improves significantly recanalization rates and stops the progress of thrombosis. Low-molecular-weight heparin, vitamin K antagonists, as well as direct-acting oral anticoagulants are possible anticoagulants, but it is noteworthy to be aware that all recommendations supporting the off-label use of anticoagulants are based on poor evidence and consist predominantly of case series, observational studies, or studies with small case numbers. When choosing a suitable anticoagulation, the individual risk of bleeding and thrombosis must be weighted very carefully. In cases of bleeding, bowel infarction, or other complications, the optimal therapy should be determined on a case-by-case basis by an experienced multidisciplinary team involving a surgeon. Besides anticoagulation, there are therapeutic options including thrombectomy, balloon angioplasty, stenting, transjugular placement of an intrahepatic portosystemic shunt, liver transplantation, and ischemic bowel resection. This article gives an overview of current diagnostic and therapeutic strategies.

In survivors of acute pulmonary embolism (PE), the post-PE syndrome (PPES) may occur. In PPES, patients typically present with persisting or progressive dyspnea on exertion despite 3 months of therapeutic anticoagulation. Therefore, a structured follow-up is warranted to identify patients with chronic thromboembolic pulmonary disease (CTEPD) with normal pulmonary pressure or chronic thromboembolic pulmonary hypertension (CTEPH). Both are currently understood as a dual vasculopathy, that is, secondary arterio- and arteriolopathy, affecting the large and medium-sized pulmonary arteries as well as the peripheral vessels (diameter < 50 µm). The follow-up algorithm after acute PE commences with identification of clinical symptoms and risk factors for CTEPH. If indicated, a stepwise performance of echocardiography, ventilation-perfusion scan (or alternative imaging), N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level, cardiopulmonary exercise testing, and pulmonary artery catheterization with angiography should follow. CTEPH patients should be treated in a multidisciplinary center with adequate experience in the complex therapeutic options, comprising pulmonary endarterectomy, balloon pulmonary angioplasty, and pharmacological interventions.

Patients with cancer are prone to develop venous thromboembolism (VTE) with negative impact on quality of life, morbidity, and mortality. Treatment of established VTE is often complex in patients with cancer. Treatment of cancer-associated VTE (CAT) basically comprises initial and maintenance treatment, for 3 to 6 months, secondary preventions, and treatment in special situations. Therapeutic anticoagulation is the treatment of choice in CAT. In addition to the efficacy and safety of low-molecular-weight heparin (LMWH) that had been recommended for decades, direct oral anti-factor Xa inhibitors, a subgroup of direct oral anticoagulants (DOACs), demonstrated their advantages along with the accompanying concerns in several randomized controlled treatment trials of CAT. The latest guidelines, such as the German AWMF-S2k Guideline "Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism," agree with each other on most aspects with respect to the treatment of CAT. Encompassing recent clinical studies, and meta-analyses, as well as the focus on some special management aspects of CAT, the objective of this review is to present a current overview and recommendations for the treatment of CAT.

Therapeutic management of acquired von Willebrand syndrome (AVWS) can be challenging, particularly in cases of AVWS associated with monoclonal IgM such as Waldenström macroglobulinemia (WM) where several therapeutic options may be ineffective. Here, we describe the case of an 88-year-old patient who developed AVWS during follow-up for WM. The presence of a severe bleeding symptomatology not controlled by several therapies (plasma-derived von Willebrand factor, plasmapheresis) led us to introduce a supplementation with recombinant von Willebrand factor, vonicog α (Veyvondi, Takeda, Japan), starting at a dose of 50 IU/kg/d. This supplementation allowed clinical (no further bleeding) and biological (hemoglobin level, von Willebrand factor parameters) improvements. Because of the persistence of bleeding risk factors, the treatment was maintained at a prophylactic dose (20 UI/kg three times a week), without recurrence of bleeding events for a period of 9 months.