scholarly journals Direct oral anticoagulants in thrombotic antiphospholipid syndrome associated with chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 18 (3) ◽  
pp. 755-756
Author(s):  
Mateo Porres‐Aguilar ◽  
Susan R. Kahn ◽  
Andrew M. Hirsch
Author(s):  
Mateo Porres-Aguilar ◽  
Marius M. Hoeper ◽  
Belinda N. Rivera-Lebron ◽  
Gustavo A. Heresi ◽  
Debabrata Mukherjee ◽  
...  

2020 ◽  
Vol 40 (03) ◽  
pp. 348-355
Author(s):  
Laurent Bertoletti ◽  
Valentine Mismetti ◽  
George Giannakoulas

AbstractSince the earliest works on the understanding of different forms of pulmonary hypertension, thrombosis has been involved in the pathophysiology of the disease, both in pulmonary arterial hypertension (PAH) and in chronic thromboembolic pulmonary hypertension (CTEPH). Autopsy and then pathophysiological data paved the way for the use of anticoagulants as a treatment for PAH and CTEPH. In PAH their role has diminished with the advent of specific targeted therapies, but they are still prescribed in more than half of PAH patients, because of concomitant venous thromboembolism or atrial fibrillation. In CTEPH long-term anticoagulant therapy is the cornerstone of the management. The recent development of direct oral anticoagulants (DOACs) raises the question of the best anticoagulation strategy, both in patients with PAH and in patients with CTEPH. In this review, we present an overview of the history of anticoagulants in the management of patients suffering from PAH or CTEPH, an update of the available data on the underlying rationale of their use in these subjects, an alert on the potential risks of using DOACs in these poorly explored situations, and the setting up of dedicated trials to evaluate the best anticoagulant treatment strategies in patients suffering from PAH or CTEPH.


2018 ◽  
Vol 24 (1) ◽  
pp. 1-8
Author(s):  
Eglė Palevičiūtė ◽  
Radvilė Malickaitė ◽  
Mindaugas Matačiūnas ◽  
Virginija Šileikienė ◽  
Lina Kryžauskaitė ◽  
...  

Abstract Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary antiphospholipid syndrome. Pulmonary endarterectomy - the surgical removal of the organized thromboembolic material from the proximal pulmonary arteries - is the procedure of choice and potentially a curative option for patients with chronic thromboembolic pulmonary hypertension, including patients with antiphospholipid syndrome. We report an exceptionally severe and complicated case with favourable outcome. Case presentation: We present a case of a successful high risk pulmonary endarterectomy in a 29-year-old female with primary antiphospholipid syndrome and end-stage chronic thromboembolic pulmonary hypertension. Despite highly complicated perioperative course an impressive improvement in symptoms and functional status was achieved. Conclusion: We hope that this complicated but successfully managed case of a combination of two rare diseases will arouse earlier suspicion and timely diagnosis for such patients and will encourage physicians to promptly refer the suitable patients to a pulmonary endarterectomy team. It is important to remember that severe thrombocytopenia may occur in patients with antiphospholipid syndrome


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Hosokawa ◽  
K Abe ◽  
H Tsutsui

Abstract Background/Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) requires a lifelong anticoagulation therapy. However, the efficacy or safety of non-vitamin K antagonist oral anticoagulants (NOACs) have not been established in CTEPH. Purpose We aimed to evaluate the effects of NOACs on the changes in pulmonary vascular resistance (PVR) and their adverse events such as bleeding and clinical worsening in CTEPH. Methods We retrospectively compared the changes in PVR among CTEPH patients taking vitamin-K antagonists (VKA) (n=38) and those taking NOACs (n=46) to evaluate the effectiveness of NOACs for the prophylaxis of thrombotic disease progression in CTEPH. Also, we extracted incidence of clinically relevant bleeding and clinical worsening in CTEPH as an exploratory outcome measures. Clinical worsening in CTEPH was defined as composite outcome of death from any cause, lung transplantation and worsening pulmonary hypertension that resulted in hospitalization, addition of specific drug for pulmonary hypertension, or rescue pulmonary endarterectomy or balloon pulmonary angioplasty. Clinically relevant bleeding was defined as composite major bleeding and/or clinically relevant non-major bleeding, which is defined as bleeding associated with the need for medical intervention, contact with a physician, interruption of anticoagulation therapy, discomfort or impairment of activities of daily life. Results The changes in PVR (VKA group; −0.17±0.82 woods unit/year, NOACs group; −0.44±0.89 woods unit/year, p=0.32) were comparable between VKA and NOACs groups. The incidence of clinical worsening in CTEPH (VKA group; 1.8% per patient-year, NOACs group; 0% per patient-year) and clinically relevant bleeding (VKA group; 2.8% per patient-year, NOACs group; 2.8% per patient-year) was comparable between the VKA and NOACs groups. D-dimer levels (VKA group; 0.58±0.22 μg/ml, NOACs group; 0.67±0.47 μg/ml, p=0.41) were also comparable between the groups. Conclusions Our study revealed that NOACs had a similar effectiveness in the prevention of thrombotic disease progression in CTEPH as compared with VKA. The result suggest that NOACs might have similar efficacy and safety as VKA in daily clinical practice. Acknowledgement/Funding Japan Agency for Medical Research and Development


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