scholarly journals The pathophysiology of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160112 ◽  
Author(s):  
Gérald Simonneau ◽  
Adam Torbicki ◽  
Peter Dorfmüller ◽  
Nick Kim
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Small Vessel ◽  
Treatment Modalities ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Substantial Impact ◽  
Vessel Disease ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis or underlying haematological or autoimmune disorders. It is now known that small-vessel abnormalities also contribute to haemodynamic compromise, functional impairment and disease progression in CTEPH. Small-vessel disease can occur in obstructed areas, possibly triggered by unresolved thrombotic material, and downstream from occlusions, possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries. The molecular processes underlying small-vessel disease are not completely understood and further research is needed in this area. The degree of small-vessel disease has a substantial impact on the severity of CTEPH and postsurgical outcomes. Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease. It requires early, reliable identification of patients with CTEPH and use of optimal treatment modalities in expert centres.

Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2550-2554
Author(s):  
Yu Taniguchi ◽  
Xavier Jaïs ◽  
Gérald Simonneau ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Flow Distribution ◽  
Small Vessel ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Vessel Disease ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive pulmonary vascular disease that is usually the consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organized thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis, and underlying haematological or autoimmune disorders, including splenectomy and antiphospholipid antibodies. Small pulmonary vessel remodelling also contributes to haemodynamic compromise, functional impairment, and disease progression in CTEPH. Small vessel disease can occur in non-obstructed areas as well as downstream from occlusions (possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries). The degree of small vessel disease has a substantial impact on the severity of CTEPH and post-surgical outcomes. Surgical, interventional, and medical treatment of CTEPH aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle, and prevent or treat small vessel disease.

scholarly journals Revisiting a Distinct Entity in Pulmonary Vascular Disease: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Medicina ◽  
2021 ◽  
Vol 57 (4) ◽  
pp. 355
Author(s):  
Munish Sharma ◽  
Deborah Jo Levine
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Progressive Increase ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Pulmonary Thromboendarterectomy ◽  
Percutaneous Balloon ◽  
Definitive Therapy ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific type of pulmonary hypertension (PH) and the major component of Group 4 pulmonary hypertension (PH). It is caused by pulmonary vasculature obstruction that leads to a progressive increase in pulmonary vascular resistance and, ultimately, to failure of the right ventricle. Pulmonary thromboendarterectomy (PEA) is the only definitive therapy, so a timely diagnosis and early referral to a specialized PEA center to determine candidacy is prudent for a favorable outcome. Percutaneous balloon pulmonary angioplasty (BPA) has a potential role in patients unsuitable for PEA. Medical therapy with riociguat is the only PH-specific medical therapy currently approved for the treatment of inoperable or persistent CTEPH. This review article aims to revisit CTEPH succinctly with a review of prevailing literature.

scholarly journals EXPRESS: Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: state-of-the-art 2020

2021 ◽  
pp. 204589402110073
Author(s):  
Michael Madani
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Small Vessel Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Treatment Modalities ◽  
Pulmonary Endarterectomy ◽  
Therapeutic Decisions ◽  
Number Of Patients ◽  
Thromboembolic Pulmonary Hypertension ◽  
Post Operative Survival

Pulmonary endarterectomy (PEA) is the treatment of choice for patients with operable CTEPH as it is potentially curative. In expert centers that conduct > 50 PEA procedures per year, peri- and post-surgical mortality rates are very low and long-term outcomes are excellent, with 3-year post-operative survival of > 80%. Therapeutic decisions in CTEPH are based largely on the location of the arterial obstruction, with PEA for obstructions in main, lobar, and segmental vessels, and balloon pulmonary angioplasty (BPA) and medical therapy for small-vessel disease. Medical therapy is also an option for patients with persistent/recurrent pulmonary hypertension after PEA or BPA. With increasing surgical experience and improvements in instruments and procedures, an increasing number of patients are now considered operable who would previously have been inoperable, including some patients with subsegmental disease. At our University, around 200 PEA procedures are performed every year and several advances have been developed, including resection of more distal disease, availability of PEA to patients previously considered to be at too high risk for surgery, improved management of post-PEA complications, and minimally invasive PEA. PEA can be combined with other treatment modalities, including BPA, medical therapy for persistent/recurrent pulmonary hypertension after PEA, and medical therapy or BPA as bridging therapy before surgery. Data on these combinations are, however, limited. Combination treatment should therefore be considered on an individual patient basis. In the future, however, multimodal therapy with PEA, BPA, and/or medical therapy is likely to be an important treatment option for many patients.

scholarly journals Surgical Treatment of Chronic Thromboembolic Pulmonary Hypertension

2007 ◽  
Vol 6 (2) ◽  
pp. 83-91
Author(s):  
Michael M. Madani ◽  
Stuart W. Jamieson
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Management ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Thromboembolic Disease ◽  
Definitive Treatment ◽  
Pulmonary Vasculature ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Long Term Prognosis

Pulmonary hypertension as the result of chronic pulmonary thromboembolic disease is a serious condition with poor long-term prognosis. The condition is one of the more common cardiovascular diseases affecting Americans, yet it is severely underdiagnosed. Pulmonary thromboendarterectomy is the definitive treatment for chronic pulmonary hyper-tension as the result of thromboembolic disease; however. it is an uncommon procedure, primarily because of lack of recognition on the part of the clinicians. Patients affected by chronic thromboembolic pulmonary hypertension (CTEPH) may present with a variety of debilitating cardiopulmonary symptoms. However, once diagnosed, there is no curative role for medical management, and surgery remains the only option. Palliation therapy with medical management in order to delay surgery carries the risk of prolonging the disease and irreversibly damaging unaffected pulmonary vasculature.

4975Efficacy of pulmonary hypertension-targeted drugs for severe chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Inami ◽  
M Kataoka ◽  
J Itoh ◽  
K Takeuchi ◽  
H Kikuchi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Targeted Drugs ◽  
Significant Difference ◽  
Balloon Pulmonary Angioplasty ◽  
Severe Group ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
High Pulmonary Vascular Resistance

Abstract Background High pulmonary vascular resistance (PVR) is a risk factor for reperfusion pulmonary edema by balloon pulmonary angioplasty (BPA). It has not been clarified whether improvement of hemodynamics by pulmonary hypertension (PH)-targeted drugs before BPA session is effective clinically. Severe chronic Thromboembolic pulmonary hypertension (CTEPH) patients have small vessel disease like pathological findings of idiopathic pulmonary arterial hypertension. Objectives The purpose of this study was to investigate the efficacy of PH-targeted drugs for patients with CTEPH before BPA. Methods This study included consecutive 147 CTEPH patients treated with PH-targeted drugs before BPA. All patients were divided into 2 groups such as severe group (PVR >12.5 wood units) and non-severe group (PVR <12.5 wood units). PVR were compared between baseline and after treated with PH-targeted drugs. All data were expressed by median [25thpercentile-75thpercentile]. Results The median age and male were 66 [53–74] years old and 42. Numbers of patients treated with phosphodiesterase V inhibitors, endothelin receptor antagonists and riociguat were 84 (57%), 92 (63%), and 43 (29%), respectively. PVR significantly decreased after treated with PH-targeted drugs in two groups (severe group: 16.3 [13.7–20.0] to 10.0 [7.2–13.3] wu, P<0.0001; non-severe group: 6.8 [4.9–8.9] to 5.7 [3.5] to 7.8, P<0.001). Changes of PVR in severe group were significantly larger than in non-severe group (−6.9 [−8.5 to −3.2] vs. −0.7 [−2.4 to 0.7], P<0.0001). There was no significant difference in efficacy of BPA and numbers of dilated vessel between two groups (severe vs. non-severe group after BPA; PVR: 2.7 [1.9–3.5] vs. 2.1 [1.7–3.1] wu, P>0.05; numbers of dilated vessel: 18 [14–21] vs. 16 [10–21], P>0.05) Conclusions PH-targeted drugs may improve hemodynamics for severe CTEPH more effectively.

scholarly journals Chronic thromboembolic pulmonary hypertension: a distinct disease entity

2015 ◽  
Vol 24 (136) ◽  
pp. 246-252 ◽  
Author(s):  
Irene Lang
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Vasculature ◽  
Distinct Disease ◽  
Thromboembolic Pulmonary Hypertension ◽  
Perfusion Scan ◽  
Pulmonary Arterial

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension (PH). One disease hypothesis is that CTEPH results from the non-resolution of venous thromboembolism. CTEPH is characterised by the presence of obstructive fibrotic thromboembolic material in the major pulmonary vessels, with concomitant microvascular arteriopathy, resulting in progressive PH. The clinical presentation of CTEPH is similar to pulmonary arterial hypertension with nonspecific symptoms, but it is distinguished from pulmonary arterial hypertension by the presence of mismatched segmental defects on the ventilation/perfusion scan. The exact prevalence and incidence of CTEPH are unknown, but are thought to have been underestimated in the past. CTEPH is unique among the subgroups of PH in that it is potentially curable with pulmonary endarterectomy, a surgical intervention intended to remove the occlusive material from the pulmonary vasculature. However, in some patients the obstructions are technically inaccessible or the risk/benefit ratios are unfavourable, making the condition inoperable. It is thought that the involvement of the smaller, more distal vessels is a target for medical treatment. Untreated, CTEPH may result in right heart failure and death. The pathophysiological mechanisms which cause CTEPH are complex and have not yet been fully elucidated.

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 Methods of Pharmacological Treatment of Chronic Thromboembolic Pulmonary Hypertension Current Approaches to the Patients Management

Kardiologiia ◽  
2018 ◽  
Vol 58 (11) ◽  
pp. 82-93
Author(s):  
E. A. Ushkalova ◽  
S. K. Zyryanov ◽  
K. E. Zatolochina
Keyword(s):  
Pulmonary Hypertension ◽  
Guanylate Cyclase ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Vasculature ◽  
The European Union ◽  
Pulmonary Thromboendarterectomy ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Thromboembolic Pulmonary Hypertension ◽  
Phosphodiesterase Type

In this paper we have discussed epidemiology, pathogenesis, and approaches to treatment of chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a unique potentially curable form of pulmonary hypertension. The gold standard of CTEPH treatment is pulmonary thromboendarterectomy. However, about 40% of patients with CTEPH are inoperable due to distal surgically inaccessible lesions of the pulmonary vasculature, severe hemodynamic impairments, or other contraindications. In addition, nearly half of patients have persistent or recurrent pulmonary hypertension following surgery. Current guidelines support the use of pharmacotherapy in these patients. In the article we have presented results of main clinical studies of targeted drugs therapy (endothelin receptor antagonists, prostanoids, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators) of patients with CTEPH. The only drug that has demonstrated robust clinical efficacy in terms of improvment hemodynamic parameters, exercise capacity and patients’ quality of life is the stimulator of the soluble guanylate cyclase riociguat. The efficacy and safety of riociguat have been investigated in short-term and long-term studies with follow-up up to 6 years. Results of these studies have constituted the basis forits approval by the regulatory authorities of more than 50 countries for the treatment of inoperable CTEPH and persistent or recurrent CTEPH after pulmonary thromboendarterectomy. In the European Union, USA and many other countries, riociguat is the only pharmacological agent approved for these indications.

scholarly journals Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Alexandra Arvanitaki ◽  
George Giannakoulas ◽  
Eva Triantafyllidou ◽  
Christos Feloukidis ◽  
Afroditi K. Boutou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Structural Changes ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Multiple Comparisons ◽  
Capillary Density ◽  
Pulmonary Vascular Disease ◽  
Healthy Controls ◽  
Continuous Variables ◽  
Thromboembolic Pulmonary Hypertension ◽  
Bonferroni Test

Abstract Background Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity. Methods Α prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test). Results Both IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p < 0.001) and increased loop width (15.7 ± 3.9 μm and 15.8 ± 1.9 μm vs. 11.5 ± 2.3 μm, p < 0.001). More than half of patients with IPAH presented microhaemorrhages on capillary nailfold, while increased shape abnormalities in capillary morphology and more capillary thrombi per linear mm were detected in patients with CTEPH compared to patients with IPAH and healthy controls. All PH patients presented a non-specific NVC pattern compared to controls (p < 0.001). Conclusion The findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

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