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 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.

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 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.

Medical Therapy for Chronic Thromboembolic Pulmonary Hypertension

2014 ◽  
Vol 12 (4) ◽  
pp. 193-198 ◽  
Author(s):  
Josanna Rodriguez-Lopez ◽  
Richard N. Channick
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Soluble Guanylate Cyclase ◽  
Significant Proportion ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Thromboendarterectomy ◽  
Specialized Center ◽  
Thromboembolic Pulmonary Hypertension ◽  
Recent Phase

Chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary hypertension (PH) secondary to chronic emboli, obstructing the pulmonary arteries. This results in increased pulmonary vascular resistance and right ventricular failure. CTEPH is the only form of PH that is potentially curable, through a surgical procedure that removes the chronic emboli: pulmonary thromboendarterectomy (PTE). The first step in managing patients diagnosed with CTEPH is to determine if they are operable. The use of medical therapy should never delay referral for surgery, which should be done at a specialized center with expertise in CTEPH. Since a significant proportion of CTEPH patients are not surgical candidates, or are among the 10% to 15% of patients that have persistent or recurrent PH after surgery, there is a need for effective medical therapy. The use of several pulmonary arterial hypertension (PAH)-targeted agents have been studied, mostly in small uncontrolled trials. A recent Phase 3 clinical trial found riociguat, a stimulator of soluble guanylate cyclase (sGC), to be effective for inoperable CTEPH.

Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

2014 ◽  
Vol 12 (4) ◽  
pp. 186-192 ◽  
Author(s):  
David Poch ◽  
Victor Pretorius
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Wedge Pressure ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

Sign in / Sign up

Export Citation Format

Share Document