scholarly journals From pulmonary embolism to chronic thromboembolic pulmonary hypertension: risk factors

2021 ◽  
Vol 25 (3) ◽  
pp. 11
Author(s):  
O. Ya. Vasiltseva ◽  
A. G. Edemskiy ◽  
D. S. Grankin ◽  
E. N. Kliver ◽  
A. M. Chernyavskiy
Keyword(s):  
Risk Factors ◽  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Recurrence Risk ◽  
National Guidelines ◽  
Thromboembolic Pulmonary Hypertension

<p>Chronic thromboembolic pulmonary hypertension is a long-term consequence of acute pulmonary embolism. Gradual obstruction of the pulmonary arteries and secondary changes in the pulmonary microcirculation over time cause progressive increases in pulmonary vascular resistance and pulmonary artery pressure that can result in severe right heart failure. This article provides an overview of pulmonary embolism and chronic thromboembolic pulmonary hypertension scientific literature and national guidelines. We focus on disease and recurrence risk factors and outline future directions of research to improve short- and long-term patient outcomes.</p><p>Received 17 January 2021. Revised 1 March 2021. Accepted 19 April 2021.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> The authors declare no conflicts of interests.</p><p><strong>Contribution of the authors</strong><br />Conception and study design: A.M. Chernyavskiy, A.G. Edemskiy, D.S. Grankin, E.N. Kliver<br />Drafting the article: O.Ya. Vasiltseva<br />Critical revision of the article: E.N. Kliver<br />Final approval of the version to be published: O.Ya. Vasiltseva, A.G. Edemskiy, D.S. Grankin, E.N. Kliver, A.M. Chernyavskiy</p>

scholarly journals Chronic Thromboembolic Pulmonary Hypertension Medical Management

2021 ◽  
Vol 17 (2) ◽  
pp. e28-e32
Author(s):  
Ryan Logue ◽  
Zeenat Safdar
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Of Care ◽  
Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase Stimulator

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.

scholarly journals Long-term outcomes after percutaneous transluminal pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 16 (4) ◽  
pp. 27-32 ◽  
Author(s):  
Madina B Karabasheva ◽  
Nikolai M Danilov ◽  
Olesia V Sagaidak ◽  
Yurii G Matchin ◽  
Irina E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Method ◽  
Classical Type ◽  
Chronic Obstructive ◽  
Clinical Effects ◽  
Long Term Outcomes ◽  
Thromboembolic Pulmonary Hypertension

Introduction. Chronic thromboembolic pulmonary hypertension is a precapillary form of pulmonary hypertension that develops due to thrombotic mass obstruction of the pulmonary arteries. Balloon pulmonary angioplasty (BPA) is a new, alternative treatment method for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), which demonstrated good hemodynamic and clinical effects. In this article we studied the long-term outcomes results after BPA. Material and methods. The study included 22 patients with inoperable CTEPH who were treated by BPA 6 (5; 8) interventions per patient. The results of the procedure were evaluated 2 months and 18 (12; 18) months after the last BPA. Results. A significant decrease in all important hemodynamic parameters was noted. There no difference between the date immediately after the operation and the long term period. However, the division of patients into groups with and without disease progression allow us to identify factors affecting the effectiveness of BPA (weight, the number of BPA procedures per patient, the total number of treated segmental arteries, the presence of coronary heart disease and chronic obstructive pulmonary disease, not the appointment of a concomitant pathogenetic pulmonary arterial hypertension (PAH) therapy. Conclusion. BPA is an effective method of treating patients with inoperable CTEPH, which demonstrates a good, sustainable long-term result. Patients with classical type 4 pulmonary hypertension receiving PAH-specific therapy are best responders to BPA.

Chronic Thromboembolic Pulmonary Hypertension: When to Suspect It, When to Refer for Surgery

2003 ◽  
Vol 2 (1) ◽  
pp. 4-9 ◽  
Author(s):  
Kim M. Kerr ◽  
Peter F. Fedullo ◽  
William R. Auger
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Acute Pulmonary Embolism ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The United States ◽  
Thromboembolic Disease ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Chronic Thromboembolic Disease

Chronic thromboembolic obstruction of the major pulmonary arteries is an underrecognized sequela of acute pulmonary embolism. Depending on the burden and location of thrombus, as well as on the duration of vessel obstruction, chronic thromboembolic disease may lead to pulmonary hypertension and cor pulmonale. Chronic thromboembolic disease affects an estimated 500 to 2500 patients each year in the United States, roughly 0.1 to 0.5 percent of patients who survive acute pulmonary embolism. Consequently, while this disease is uncommon, chronic thromboembolic pulmonary hypertension (CTEPH) is not rare, and should be considered in patients with unexplained dyspnea, as it is potentially correctible with pulmonary thromboendarterectomy.1

scholarly journals Chronic thromboembolic pulmonary hypertension – still evolving

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Mario Gerges ◽  
Magdi Yacoub
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Treatment Options ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Vasculature ◽  
Pulmonary Hemodynamics ◽  
Thromboembolic Pulmonary Hypertension ◽  
Multiple Treatments

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension (PH). The disease is still underdiagnosed, and the true prevalence is unknown. CTEPH is characterized by intraluminal non-resolving thrombus organization and fibrous stenosis, or complete obliteration of pulmonary arteries, promoted by progressive remodeling of the pulmonary vasculature. One consequence of this is an increase in pulmonary vascular resistance and pressure, resulting in PH and progressive right heart failure, leading to death if left untreated.Endovascular disobliteration by pulmonary endarterectomy (PEA) is the preferred treatment for CTEPH patients. PEA surgery is the only technique that can potentially cure CTEPH disease, especially in patients with fresh or organized thrombi of the proximal branches of pulmonary arteries. However, not all patients are eligible for PEA surgery. Recent research has provided evidence suggesting balloon pulmonary angioplasty (BPA) and targeted medical therapy as additional promising available treatments options for inoperable CTEPH and recurrent/persistent PH after PEA surgery.Studies on BPA have shown it to improve pulmonary hemodynamics, symptoms, exercise capacity and RV function in inoperable CTEPH. Subsequently, BPA has developed into an essential component of the modern era of CTEPH treatment. Large randomized controlled trials have demonstrated varying significant improvements with targeted medical therapy in technically inoperable CTEPH patients. Thus, treatment of CTEPH requires a comprehensive multidisciplinary assessment, including an experienced PEA surgeon, PH specialist, BPA interventionist and CTEPH-trained radiologist at expert centers. In this comprehensive review, we address the latest developments in the fast-evolving field of CTEPH. These include advancements in imaging modalities and developments in operative and interventional techniques, which have widened the range of patients who may benefit from these procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed. As the treatment options for CTEPH improve, hybrid management involving multiple treatments in the same patient may become a viable option in the near future.

scholarly journals Chronic Thromboembolic Pulmonary Hypertension

2018 ◽  
Vol 35 (02) ◽  
pp. 136-142 ◽  
Author(s):  
G. Pretorius ◽  
Stuart Jamieson
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Scar Tissue ◽  
Right Heart ◽  
Fibrotic Scar ◽  
Thromboembolic Pulmonary Hypertension

AbstractChronic thromboembolic pulmonary hypertension occurs when acute thromboemboli fail to dissolve completely. The resulting fibrotic scar tissue within the pulmonary arteries is obstructive and eventually leads to right heart failure. Medical therapy for this condition is supportive, but surgery with pulmonary artery endarterectomy is curative, and carries a low mortality at experienced centers.

scholarly journals Overview of Riociguat and Its Role in the Treatment of Pulmonary Hypertension

2020 ◽  
pp. 089719002096129
Author(s):  
Marianne Kenny ◽  
Megan M. Clarke ◽  
Kristen T. Pogue
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Term Survival ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Pulmonary hypertension (PH), which includes pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), is a progressive condition with significant morbidity and mortality due to right heart failure if left untreated. Riociguat is a soluble guanylate cyclase (sGC) stimulator and is the only treatment approved for both PAH and CTEPH. The objectives of this review are to describe the epidemiology and pathophysiology of PAH and CTEPH; synthesize the pharmacology, efficacy, safety, and utilization of riociguat; and discuss the role of the pharmacist in managing patients with these conditions. Data presented in this review is supported by peer reviewed literature, using PubMed and key words including pulmonary hypertension, pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and riociguat. The review draws on key studies and review articles that discuss the pathophysiology of PAH and CTEPH, as well as articles discussing the safety and efficacy of riociguat. The overall goal in the treatment of PAH and CTEPH is to improve long-term survival. Treatment planning depends on the type of PH, treatment goals, comorbidities, and risk profiles. Pharmacists serve a valuable role as part of the multidisciplinary team in the care of patients with PH, many of whom may have comorbidities that contribute to high costs and resource utilization. Riociguat is a first-in-class medication and the only approved treatment for both PAH and CTEPH. In clinical trials, riociguat has demonstrated favorable efficacy and tolerability. Riociguat is a valuable addition to the armamentarium of options for treating patients with PH.

scholarly journals Molecular Research in Chronic Thromboembolic Pulmonary Hypertension

2019 ◽  
Vol 20 (3) ◽  
pp. 784 ◽  
Author(s):  
Isabelle Opitz ◽  
Michaela Kirschner
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Current Knowledge ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pi3k Signaling ◽  
Molecular Alterations ◽  
Complex Processes ◽  
Thromboembolic Pulmonary Hypertension ◽  
Differential Gene

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a debilitating disease, for which the underlying pathophysiological mechanisms have yet to be fully elucidated. Occurrence of a pulmonary embolism (PE) is a major risk factor for the development of CTEPH, with non-resolution of the thrombus being considered the main cause of CTEPH. Polymorphisms in the α-chain of fibrinogen have been linked to resistance to fibrinolysis in CTEPH patients, and could be responsible for development and disease progression. However, it is likely that additional genetic predisposition, as well as genetic and molecular alterations occurring as a consequence of tissue remodeling in the pulmonary arteries following a persistent PE, also play an important role in CTEPH. This review summarises the current knowledge regarding genetic differences between CTEPH patients and controls (with or without pulmonary hypertension). Mutations in BMPR2, differential gene and microRNA expression, and the transcription factor FoxO1 have been suggested to be involved in the processes underlying the development of CTEPH. While these studies provide the first indications regarding important dysregulated pathways in CTEPH (e.g., TGF-β and PI3K signaling), additional in-depth investigations are required to fully understand the complex processes leading to CTEPH.

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