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Case Reports in Medicine 2015Objectives. Presence of a factor-V Leiden mutation in a patient with myotonic dystrophy type 1 (DM1) has been reported only once. Here we report the second DM1 patient...
Objectives. Presence of a factor-V Leiden mutation in a patient with myotonic dystrophy type 1 (DM1) has been reported only once. Here we report the second DM1 patient carrying a factor-V mutation who died from long-term complications of this mutation. Case Report. A 66-year-old DM1 patient with multi-organ-disorder syndrome developed a first deep venous thrombosis (DVT) and consecutive pulmonary embolism (PE) at age 50 y. Acetyl-salicylic acid was given. One year later he experienced a second DVT; that is why phenprocoumon was started. Despite anticoagulation, he experienced a third DVT bilaterally and a second PE bilaterally at 61 y; that is why a vena cava filter was additionally deployed. Despite therapeutic anticoagulation, he experienced a vena cava filter thrombosis at age 62 y. Genetic workup revealed a heterozygous factor-V mutation in addition to a CTG-repeat expansion of 500. As a consequence of PE he developed chronic obstructive pulmonary disease and experienced recurrent pulmonary infections, which were lastly responsible for decease at age 66 y despite intensive care measures. Conclusion. The heterozygous Leiden mutation may severely affect DM1 patients to such a degree that they die from its complications. If DM1 patients present with unusual manifestations, search for causes other than a CTG-repeat expansion is indicated.
PubMed: 25918532
DOI: 10.1155/2015/271639 -
Drugs - Real World Outcomes Dec 2021In older patients with nonvalvular atrial fibrillation, oral anticoagulation is challenging, especially among very old patients. Even though positive effects of oral...
BACKGROUND
In older patients with nonvalvular atrial fibrillation, oral anticoagulation is challenging, especially among very old patients. Even though positive effects of oral anticoagulation have been described in this age group (> 85 years), there is still a high rate of inappropriate dosing.
OBJECTIVE
This study examines the quality of care for very old hospitalized patients. The aims of this study were to (1) describe the percentage of patients receiving oral anticoagulation at discharge, (2) describe the quality of drug management at discharge, regarding dosing and contraindications, and (3) provide additional data towards establishing a benchmark for the quality of care in very old patients with atrial fibrillation.
METHODS
This study is a single-center descriptive observational study. The data from 407 patients aged > 85 years who were hospitalized in 2018 with nonvalvular atrial fibrillation were collected retrospectively from the patient charts. The assessment included specific geriatric aspects, such as falls, the Charlson Comorbidity Index, and nursing categorization.
RESULTS
During hospitalization, the proportion of anticoagulated patients increased from 57.5% (n = 234) to 67.3% (n = 274). We found an increasing trend in the use of direct oral anticoagulants, with an increase from 39.8% (n = 162) to 46.2% (n = 188). Regarding the quality of drug management, 13.8% (n = 56) of the patients were not anticoagulated even in the absence of a recognizable contraindication, whereas a contraindication was ignored in only 0.8% (n = 2 out of 188). Dosing was appropriate among all patients taking rivaroxaban, edoxaban, and dabigatran. The dose of apixaban was not reduced in 23.6% (n = 21 out of 89), although this dose reduction was necessary according to the dose adjustment criteria. An underdose of a direct oral anticoagulant was found in 26.1% of the patients (n = 49 out of 188).
CONCLUSIONS
Compared to the results reported in the literature, the percentage of very old patients with atrial fibrillation receiving anticoagulants was high. During hospitalization, the proportion of patients receiving a direct oral anticoagulant increased, which was in contrast to the trend in the proportion of patients taking phenprocoumon. Our results could help to find a benchmark for anticoagulation management among hospitalized very old patients.
PubMed: 34115332
DOI: 10.1007/s40801-021-00263-6 -
RoFo : Fortschritte Auf Dem Gebiete Der... Nov 2015To investigate the incidence and possible risk factors of upper deep vein obstruction in patients both prior to first cardiac device implantation and before device...
Venous Obstruction in Asymptomatic Patients Undergoing First Implantation or Revision of a Cardiac Pacemaker or Implantable Cardioverter-Defibrillator: A Retrospective Single Center Analysis.
PURPOSE
To investigate the incidence and possible risk factors of upper deep vein obstruction in patients both prior to first cardiac device implantation and before device revision.
MATERIALS AND METHODS
Records of asymptomatic patients undergoing contrast venography prior to implantation or revision of a cardiac device from 09/2009 to 04/2012 were reviewed. Venograms were used to determine the presence of venous obstruction. Interrelations between the incidence of venous obstruction and patient- or device-related parameters were identified using Fisher's exact test and univariate logistic regression. Multivariate logistic regression was used to identify independent predictors of venous obstruction.
RESULTS
456 patients met the inclusion criteria (330 males, 126 females, 67.8 ± 12.9 years). 100 patients underwent first implantation, and 356 patients underwent device revision (mean time since implantation 82.5 ± 75.3 months). Venous obstruction was present in 11.0 % and 30.1 % before implantation and revision, respectively. Only presence of ventricular escape rhythm was significantly related to venous occlusion (p < 0.001) prior to first implantation. Prior to revision, significant predictors were male sex (p = 0.01), time since implantation (p < 0.0001), presence of escape rhythm (p = 0.02), compromised coagulation (p = 0.02), phenprocoumon (p = 0.005), and peripheral arterial disease (p = 0.01).
CONCLUSION
Although several risk factors could be identified, reliable prediction of venous obstruction was not possible. Therefore, we advocate performing venography in all patients prior to device revision or upgrade to avoid complications. In cases of first device implantation, the risks associated with venography should be weighed against the surprisingly high rate of deep upper vein obstruction.
Topics: Aged; Aged, 80 and over; Arm; Defibrillators, Implantable; Equipment Failure Analysis; Female; Humans; Logistic Models; Male; Middle Aged; Pacemaker, Artificial; Phlebography; Postoperative Complications; Reoperation; Retrospective Studies; Risk Factors; Statistics as Topic; Venous Thrombosis
PubMed: 26200569
DOI: 10.1055/s-0035-1553351 -
Journal of Thrombosis and Haemostasis :... Mar 2016ESSENTIALS: We developed a new algorithm to optimize vitamin K antagonist dose finding. Validation was by comparing actual dosing to algorithm predictions. Predicted and... (Comparative Study)
Comparative Study
UNLABELLED
ESSENTIALS: We developed a new algorithm to optimize vitamin K antagonist dose finding. Validation was by comparing actual dosing to algorithm predictions. Predicted and actual dosing of well performing centers were highly associated. The method is promising and should be tested in a randomized trial.
BACKGROUND
Oral vitamin K antagonists (VKAs) have a narrow therapeutic window and thus require frequent monitoring of its intensity by the international normalized ratio (INR). Improvement of VKA dosing defined as more time in therapeutic range (TTR) can reduce thrombotic disease and bleeding. Computerized decision support programs (CDSs) are used to optimize VKA dosing, but the effects are heterogeneous. CDSs significantly improve the proportion of time in the therapeutic INR range for initiation therapy but not the quality of anticoagulant management in an outpatient setting. One of the major problems of VKA dose finding is that the INR is a ratio and does not present linearity. We developed a new dose-finding algorithm, based on a novel bidirectional factor (BF). This BF is linear transformation of the nonlinear INR.
METHODS
We compared the outcomes of the new algorithm, called BF-N, with dose finding performed at three highly ranked Dutch anticoagulation centers, using both acenocoumarol and phenprocoumon.
RESULTS
The outcomes of the BF-N algorithm showed a linear correlation with VKA doses of the three centers (y = 1.001x, r(2) 0.999 for acenocoumarol and y = 0.999x, r(2) 0.999 for phenprocoumon), with a standard deviation of 3.83%. The rate of automated dosage proposals increased to 100%.
CONCLUSION
The BF-N algorithm performs well in real-life settings and increases the rate of automated dosage proposals. The algorithm can be easily built into existing CDSs. Experienced staff remains necessary for complicated situations. The new algorithm needs to be evaluated in a prospective trial.
Topics: Acenocoumarol; Administration, Oral; Algorithms; Anticoagulants; Blood Coagulation; Decision Support Techniques; Drug Dosage Calculations; Drug Monitoring; Humans; International Normalized Ratio; Linear Models; Netherlands; Nonlinear Dynamics; Observer Variation; Phenprocoumon; Predictive Value of Tests; Reproducibility of Results; Vitamin K
PubMed: 26712475
DOI: 10.1111/jth.13240 -
JACC. Case Reports Aug 2022Direct oral anticoagulant agents (DOACs) are widely used in the treatment of arterial and venous thrombi. We report the case of a 32-year-old patient who was receiving...
Direct oral anticoagulant agents (DOACs) are widely used in the treatment of arterial and venous thrombi. We report the case of a 32-year-old patient who was receiving permanent DOAC therapy. Despite adequate use, 2 large left ventricular thrombi developed. Surgical thrombectomy was performed. The patient recovered well and received anticoagulation with phenprocoumon thereafter. ( ).
PubMed: 36062055
DOI: 10.1016/j.jaccas.2022.05.033 -
International Journal of Cardiology.... Jun 2019The risk of thromboembolic events is increased in patients with non-valvular atrial fibrillation (NVAF) and renal impairment. The risk of bleeding events is increased if...
Comparative effectiveness of rivaroxaban versus a vitamin K antagonist in patients with renal impairment treated for non-valvular atrial fibrillation in Germany - A retrospective cohort study.
BACKGROUND
The risk of thromboembolic events is increased in patients with non-valvular atrial fibrillation (NVAF) and renal impairment. The risk of bleeding events is increased if these patients are treated with anticoagulants and further increased in those with active cancer.
METHODS
RELOAD, a retrospective database study, assessed the outcomes of patients with NVAF prescribed rivaroxaban versus phenprocoumon. Here, we present a subgroup analysis evaluating effectiveness and safety of rivaroxaban versus phenprocoumon in patients with NVAF and renal impairment. Analyses were additionally stratified by patients with and without evidence of cancer at baseline.
RESULTS
When using the 'one tablet per day' definition of estimating drug exposure time, the incidence of the primary endpoint of ischaemic stroke was significantly lower in patients (without evidence of cancer at baseline) receiving rivaroxaban 15 mg or 20 mg once daily versus those receiving phenprocoumon (2.40 vs 3.51 events per 100 patient-years, respectively; hazard ratio [HR] = 0.72, 95% confidence interval [CI] 0.55-0.94, = 0.015); with the incidence of the primary safety outcome of intracranial haemorrhage being numerically lower (0.57 vs 0.89 events per 100 patient-years, respectively; HR = 0.66, 95% CI 0.38-1.14, = 0.14). Similar results were observed when using the 'empirical defined daily dose' definition to estimate drug exposure time and when including patients with evidence of cancer.
CONCLUSION
The prescription of rivaroxaban in patients with NVAF and renal impairment was associated with a lower incidence of ischaemic stroke and intracranial haemorrhage versus phenprocoumon in patients without evidence of cancer.
PubMed: 31111087
DOI: 10.1016/j.ijcha.2019.100367 -
The American Journal of Case Reports Sep 2022BACKGROUND Despite use of optimal oral anticoagulation regimens, left atrial appendage (LAA) thrombus may develop and persist in patients with atrial fibrillation (AF)....
Percutaneous Left Atrial Appendage Closure with the LAmbre Device Protected by a Cerebral Protection System in a 76-Year-Old Man with Persistent Left Atrial Appendage Thrombus.
BACKGROUND Despite use of optimal oral anticoagulation regimens, left atrial appendage (LAA) thrombus may develop and persist in patients with atrial fibrillation (AF). The therapeutic options in this population are limited. Percutaneous LAA closure (LAAC) is performed to reduce thrombus formation and the risk of thromboembolism. However, this approach is prohibited in patients with LAA thrombus. We report the case of a 76-year-old man with AF and persistent LAA thrombus who underwent percutaneous LAAC with the LAmbre device protected by the SENTINEL Cerebral Protection System (CPS). CASE REPORT A 76-year-old man with history of persistent AF treated with pulmonary veins and LAA isolation developed LAA thrombus resistant to different anticoagulation therapies, including apixaban and Phenprocoumon, with a target international normalized ratio (INR) of 3-3,5. Repeated follow-up transesophageal echocardiography showed a persistent LAA thrombus despite optimal INR values. Thus, we performed a percutaneous LAAC using a double-umbrella-designed appendage occluder and a CPS to minimize the risk of intraprocedural cerebral embolization. The procedure was guided by transesophageal echocardiography and fluoroscopy. No signs of systemic thromboembolism were noted, and the CPS filters showed no evidence of thrombotic material. CONCLUSIONS This report shows that the use of a CPS during percutaneous LAAC may reduce the risk of procedural cerebral embolization and could be used as an alternative therapy for patients with contraindication or lack of effectiveness of oral anticoagulation.
Topics: Aged; Anticoagulants; Atrial Appendage; Atrial Fibrillation; Cardiac Catheterization; Heart Diseases; Humans; Male; Thromboembolism; Thrombosis; Treatment Outcome
PubMed: 36101478
DOI: 10.12659/AJCR.937063 -
Medicine Nov 2017Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome....
RATIONALE
Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome. The clinical findings of this entity are aneurysms of the aorta or large peripheral arteries which usually present in an acute setting due to rupture of an aneurysm.
PATIENT CONCERNS
We present a case of a 43-year-old Caucasian male with histologically proven cystic medial degeneration of the lower limb vessels mimicking peripheral artery occlusive disease. Despite antiplatelet and anticoagulant treatment, the patient suffered multiple vascular stenosis and occlusions.
DIAGNOSES
Multiple arterial stenoses and thromboses leading to peripheral artery occlusive disease caused by cystic medial degeneration Erdheim-Gsell.
INTERVENTIONS
Multiple surgical and endovascular interventions including bypass graft and intra-arterial thrombolysis as well as oral antiplatelet and anticoagulant therapy.
OUTCOME
Despite dual antiplatelet therapy, anticoagulant therapy with rivaroxaban and multiple surgical and endovascular interventions, the patient developed recurrent arterial thromboses. The patient did not suffer further thrombotic events since clopidogrel and phenprocoumon were administered.
LESSONS
Clinical presentation of cystic medial degeneration Erdheim-Gsell mimicking peripheral artery occlusive disease is very unusual. Due to the fragile vessel wall, patients with cystic medial degeneration might have a higher risk to develop arterial thromboses, even under antiplatelet therapy or anticoagulant treatment.
Topics: Adult; Aortic Aneurysm, Thoracic; Arterial Occlusive Diseases; Cysts; Humans; Male; Thrombosis
PubMed: 29381979
DOI: 10.1097/MD.0000000000008782 -
PloS One 2015Vitamin-K antagonists (VKAs) present an effective anticoagulant treatment in deep venous thrombosis (DVT). However, the use of VKAs is limited because of the risk of... (Observational Study)
Observational Study
BACKGROUND
Vitamin-K antagonists (VKAs) present an effective anticoagulant treatment in deep venous thrombosis (DVT). However, the use of VKAs is limited because of the risk of bleeding and the necessity of frequent and long-term laboratory monitoring. Therefore, new oral anticoagulant drugs (NOACs) such as dabigatran, with lower rates of (major) intracranial bleeding compared to VKAs and not requiring monitoring, may be considered.
OBJECTIVES
To estimate resource utilization and costs of patients treated with the VKAs acenocoumarol and phenprocoumon, for the indication DVT. Furthermore, a formal cost-effectiveness analysis of dabigatran compared to VKAs for DVT treatment was performed, using these estimates.
METHODS
A retrospective observational study design in the thrombotic service of a teaching hospital (Deventer, The Netherlands) was applied to estimate real-world resource utilization and costs of VKA monitoring. A pooled analysis of data from RE-COVER and RE-COVER II on DVT was used to reflect the probabilities for events in the cost-effectiveness model. Dutch costs, utilities and specific data on coagulation monitoring levels were incorporated in the model. Next to the base case analysis, univariate probabilistic sensitivity and scenario analyses were performed.
RESULTS
Real-world resource utilization in the thrombotic service of patients treated with VKA for the indication of DVT consisted of 12.3 measurements of the international normalized ratio (INR), with corresponding INR monitoring costs of €138 for a standardized treatment period of 180 days. In the base case, dabigatran treatment compared to VKAs in a cohort of 1,000 DVT patients resulted in savings of €18,900 (95% uncertainty interval (UI) -95,832, 151,162) and 41 (95% UI -18, 97) quality-adjusted life-years (QALYs) gained calculated from societal perspective. The probability that dabigatran is cost-effective at a conservative willingness-to pay threshold of €20,000 per QALY was 99%. Sensitivity and scenario analyses also indicated cost savings or cost-effectiveness below this same threshold.
CONCLUSIONS
Total INR monitoring costs per patient were estimated at minimally €138. Inserting these real-world data into a cost-effectiveness analysis for patients diagnosed with DVT, dabigatran appeared to be a cost-saving alternative to VKAs in the Netherlands in the base case. Cost savings or favorable cost-effectiveness were robust in sensitivity and scenario analyses. Our results warrant confirmation in other settings and locations.
Topics: Anticoagulants; Antithrombins; Cost Savings; Cost-Benefit Analysis; Dabigatran; Decision Support Techniques; Health Resources; Humans; Netherlands; Pulmonary Embolism; Quality-Adjusted Life Years; Retrospective Studies; Venous Thrombosis; Vitamin K
PubMed: 26241880
DOI: 10.1371/journal.pone.0135054 -
International Journal of Molecular... Sep 2021Patients with atrial fibrillation and previous ischemic stroke (IS) are at increased risk of cerebrovascular events despite anticoagulation. In these patients, treatment...
Patients with atrial fibrillation and previous ischemic stroke (IS) are at increased risk of cerebrovascular events despite anticoagulation. In these patients, treatment with non-vitamin K oral anticoagulants (NOAC) such as edoxaban reduced the probability and severity of further IS without increasing the risk of major bleeding. However, the detailed protective mechanism of edoxaban has not yet been investigated in a model of ischemia/reperfusion injury. Therefore, in the current study we aimed to assess in a clinically relevant setting whether treatment with edoxaban attenuates stroke severity, and whether edoxaban has an impact on the local cerebral inflammatory response and blood-brain barrier (BBB) function after experimental IS in mice. Focal cerebral ischemia was induced by transient middle cerebral artery occlusion in male mice receiving edoxaban, phenprocoumon or vehicle. Infarct volumes, functional outcome and the occurrence of intracerebral hemorrhage were assessed. BBB damage and the extent of local inflammatory response were determined. Treatment with edoxaban significantly reduced infarct volumes and improved neurological outcome and BBB function on day 1 and attenuated brain tissue inflammation. In summary, our study provides evidence that edoxaban might exert its protective effect in human IS by modulating different key steps of IS pathophysiology, but further studies are warranted.
Topics: Animals; Blood-Brain Barrier; Brain Ischemia; Disease Models, Animal; Humans; Infarction, Middle Cerebral Artery; Inflammation; Mice; Pyridines; Severity of Illness Index; Stroke; Thiazoles
PubMed: 34576055
DOI: 10.3390/ijms22189893