scholarly journals Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401988262
Author(s):  
Shraddha Narechania ◽  
Rahul Renapurkar ◽  
Gustavo A. Heresi
Keyword(s):  
Pulmonary Hypertension ◽  
Imaging Techniques ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Sarcoma ◽  
Radiological Features ◽  
Thromboembolic Pulmonary Hypertension ◽  
Perfusion Defects ◽  
Perfusion Scan

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by mechanical obstruction of large pulmonary arteries secondary to one or more episodes of pulmonary embolism. Ventilation perfusion scan is the recommended initial screening test for this condition and typically shows multiple large mismatched perfusion defects. However, not all patients with an abnormal ventilation perfusion scan have CTEPH since there are other conditions that be associated with a positive ventilation perfusion scan. These conditions include in situ thrombosis, pulmonary artery sarcoma, fibrosing mediastinitis, pulmonary vasculitis and sarcoidosis, among others. Although these conditions cannot be distinguished from CTEPH using a ventilation perfusion scan, they have certain characteristic radiological features that can be demonstrated on other imaging techniques such as computed tomography scan and can help in differentiation of these conditions. In this review, we have summarized some key clinical and radiological features that can help differentiate CTEPH from the CTEPH mimics.

scholarly journals In situ Pulmonary Artery Thrombosis: A Previously Overlooked Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Yunshan Cao ◽  
Chao Geng ◽  
Yahong Li ◽  
Yan Zhang
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
De Novo ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Systemic Circulation ◽  
Artery Thrombosis ◽  
Thromboembolic Pulmonary Hypertension ◽  
Thrombotic Diseases

Pulmonary thromboembolism (PTE) is the third leading cause of death in cardiovascular diseases. PTE is believed to be caused by thrombi detached from deep veins of lower extremities. The thrombi travel with systemic circulation to the lung and block pulmonary arteries, leading to sudden disruption of hemodynamics and blood gas exchange. However, this concept has recently been challenged by accumulating evidence demonstrating that de novo thrombosis may be formed in pulmonary arteries without deep venous thrombosis. On the other hand, chronic thromboembolic pulmonary hypertension (CTEPH), a subtype of pulmonary hypertension, could have different pathogenesis than traditional PTE. Therefore, this article summarized and compared the risk factors, the common and specific pathogenic mechanisms underlying PTE, in situ pulmonary artery thrombosis, and CTEPH at molecular and cellular levels, and suggested the therapeutic strategies to these diseases, aiming to facilitate understanding of pathogenesis, differential diagnosis, and precision therapeutics of the three pulmonary artery thrombotic diseases.

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.

scholarly journals EXPRESS: Chronic thromboembolic pulmonary hypertension following pulmonary embolism with a right ventricular thrombus: A report of two cases

2021 ◽  
pp. 204589402110136
Author(s):  
Tailong Zhang ◽  
Weitao Liang ◽  
Longrong Bian ◽  
Zhong Wu
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Imaging Features ◽  
Pulmonary Endarterectomy ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right ◽  
Cardiac Masses

Right heart thrombus (RHT) accompanied by chronic thromboembolic pulmonary hypertension (CTEPH) is a rare entity. RHT may develop in the peripheral veins or in situ within the right heart chambers. The diagnosis of RHT is challenging, since its symptoms are typically non-specific and its imaging features resemble those of cardiac masses. Here, we report two cases of RHT with CTEPH that presented as right ventricular masses initially. Both patients underwent simultaneous pulmonary endarterectomy (PEA) and resection of the ventricular thrombi. Thus, when mass-like features are confirmed by imaging, RHT should be suspected in patients with CTEPH, and simultaneous RHT resection is required along with PEA.

scholarly journals The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients

Kardiologiia ◽  
2020 ◽  
Vol 60 (8) ◽  
pp. 115-123
Author(s):  
Z. S. Valieva ◽  
S. E. Gratsianskaya ◽  
T. V. Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Guanylate Cyclase ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
First Line ◽  
Pulmonary Thromboendarterectomy ◽  
First Line Therapy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Microcirculation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary type of pulmonary hypertension with chronic obstruction of large and medium branches of pulmonary arteries along with secondary alterations in pulmonary microcirculation, which cause progressive increases in pulmonary vascular resistance and pulmonary arterial pressure and ensuing severe right heart dysfunction and heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH; however, this procedure is available not for all patients. Although the surgery performed in the conditions of centers with advanced experience generally shows good results, up to 40% of patients are technically inoperable or PTE is associated with a high risk of complications. At present, riociguat, the only officially approved drug from the class of soluble guanylate cyclase stimulators, is considered as a first-line treatment for inoperable and residual forms of STEPH. Introduction of riociguat to clinical practice can be called a real breakthrough in the treatment of patients with STEPH who cannot undergo PTE or those with relapse or persistent STEPH after the surgery.

Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2800-2804
Author(s):  
Irene M. Lang ◽  
Walter Klepetko ◽  
Hiromi Matsubara
Keyword(s):  
Pulmonary Hypertension ◽  
Gold Standard ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Vascular Remodelling ◽  
Pulmonary Endarterectomy ◽  
Medical Treatments ◽  
Group 4 ◽  
Major Vessel ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions represent group 4 in the pulmonary hypertension classification. Angiosarcoma, other intravascular tumours, arteritides, congenital pulmonary arteries stenoses, and parasites (hydatidosis) remain rare differential diagnoses within group 4. CTEPH comprises an occlusive vascular remodelling process of major vessel pulmonary thromboembolism in combination with a pulmonary arteriopathy at the pre-capillary level. The gold standard treatment has been pulmonary endarterectomy that is able to restore normal pulmonary haemodynamics at rest. Because about half of the patients are felt to be unsuited for pulmonary endarterectomy, medical treatments and balloon pulmonary angioplasty have gained grounds in the management of patients with CTEPH.

scholarly journals Successful right heart remodelling and subsequent pregnancy in a patient with chronic thromboembolic pulmonary hypertension undergoing balloon pulmonary angioplasty: a case report

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Nobutaka Ikeda ◽  
Masaru Hatano ◽  
Takeshi Nagamatsu ◽  
Masato Nakamura
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Subsequent Pregnancy ◽  
Heart Catheterization ◽  
Right Heart ◽  
Promising Alternative ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

AbstractBackgroundTo date, the management of pregnancy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and the associated risk of maternal mortality have not been established. Although balloon pulmonary angioplasty (BPA) in patients with CTEPH is still an emerging procedure, this approach represents a promising alternative to pulmonary endarterectomy (PEA), especially in patients with inoperable CTEPH.Case summaryWe present a case of a 34-year-old woman with CTEPH who desired to have a child. Right heart catheterization showed a mean pulmonary artery pressure of 54 mmHg. Since the lesions were observed in the distal part of subsegmental pulmonary arteries, there was no indication for PEA. After improvement in her haemodynamic status by BPA, she became pregnant. At 40 weeks of gestation, a normal baby was delivered vaginally. Both mother and baby have made satisfactory progress.DiscussionIn cases in which the haemodynamic status is improved by effective BPA, pregnancy and childbirth may be possible, even in patients with CTEPH.

scholarly journals Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160111 ◽  
Author(s):  
David Jenkins ◽  
Michael Madani ◽  
Elie Fadel ◽  
Andrea Maria D'Armini ◽  
Eckhard Mayer
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Algorithm ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
European Respiratory Society ◽  
Complex Procedure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Definition Of ◽  
European Society Of Cardiology

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an “expert centre” for the management of this condition.

scholarly journals Chronic Thromboembolic Pulmonary Hypertension – What Have We Learned From Large Animal Models

2021 ◽  
Vol 8 ◽  
Author(s):  
Kelly Stam ◽  
Sebastian Clauss ◽  
Yannick J. H. J. Taverne ◽  
Daphne Merkus
Keyword(s):  
Pulmonary Hypertension ◽  
Animal Models ◽  
Current Knowledge ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Large Animal ◽  
Large Animal Models ◽  
Microvascular Remodeling ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right

Chronic thrombo-embolic pulmonary hypertension (CTEPH) develops in a subset of patients after acute pulmonary embolism. In CTEPH, pulmonary vascular resistance, which is initially elevated due to the obstructions in the larger pulmonary arteries, is further increased by pulmonary microvascular remodeling. The increased afterload of the right ventricle (RV) leads to RV dilation and hypertrophy. This RV remodeling predisposes to arrhythmogenesis and RV failure. Yet, mechanisms involved in pulmonary microvascular remodeling, processes underlying the RV structural and functional adaptability in CTEPH as well as determinants of the susceptibility to arrhythmias such as atrial fibrillation in the context of CTEPH remain incompletely understood. Several large animal models with critical clinical features of human CTEPH and subsequent RV remodeling have relatively recently been developed in swine, sheep, and dogs. In this review we will discuss the current knowledge on the processes underlying development and progression of CTEPH, and on how animal models can help enlarge understanding of these processes.

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.

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