Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2)

2016 ◽  
Vol 88 (10) ◽  
pp. 63-73 ◽  
Author(s):  
I E Chazova ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Randomized Clinical Trials ◽  
Treatment Options ◽  
Specific Treatment ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Postoperative Management ◽  
Diagnosis And Treatment ◽  
Long Term Results ◽  
Management Algorithm ◽  
Thromboembolic Pulmonary Hypertension

The paper gives current approaches to treating chronic thromboembolic pulmonary hypertension (CTEPH) from the document «Federal Guidelines for the Diagnosis and Treatment of CTEPH» approved at the Third Russian Congress on Pulmonary Hypertension on December 11, 2015. The guidelines had been elaborated to optimize the treatment of patients with CTEPH on the basis of an analysis of the data of the present-day registries and multicenter randomized clinical trials, national and international guidelines and consensus documents, and documents published in recent years. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The paper presents indications for and contraindications to pulmonary thromboendartectomy; preparation for surgery; operating room facilities; the specific features of postoperative management and possible complications; and long-term results. In terms of therapy, in addition to non-pharmacological measures, the authors discuss maintenance and specific treatment options for CTEPH, balloon pulmonary angioplasty, and lung/heart-lung transplantation. In conclusion, they propose a management algorithm in patients with CTEPH and requirements for its problem to the center of experts.

scholarly journals The role of the heart team in the diagnosis and treatment of chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 27 (5) ◽  
pp. 9-21
Author(s):  
L. V. Kulyk ◽  
Yu. M. Sirenko ◽  
G. D. Radchenko ◽  
L. I. Vasylyeva ◽  
I. O. Zhyvylo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Surgical Treatment ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Diagnosis And Treatment ◽  
Long Term Results ◽  
High Tech ◽  
Heart Team ◽  
Thromboembolic Pulmonary Hypertension ◽  
Team Concept

The aim – to present to the medical community the functioning algorithm of the heart team concept in the diagnosis and surgical treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in Ukraine. The concept of the CTEPH team implies a multi-disciplinary approach to the diagnosis and, consequently, surgical treatment of CTEPH with the participation of a radiologist, a pulmonologist, a cardiologist, a hematologist, a neurologist, and a cardiac surgeon. Considering the low incidence of the disease, the need for high-tech diagnostic tools, including CT angiography and angiopulmonography, as well as challenges of evaluating the operability of patients and the technical complexity of operations, patients with CTEPH are get together in the so-called reference centers. Within the framework of the CTEPH team concept, specific protocols and «road maps» have been developed for both diagnosis and treatment of the disease. In order to eliminate subjectivity in determining the operability of a patient with CTEPH, a special algorithm has been developed, which is applied to a clinical case for illustration. Monitoring patients with pulmonary embolism who are qualifed for a newly coined definition – postembolic pulmonary syndrome, has become a new task of the reference centers. The new syndrome is suggested to include CTEPH, as well as a similar, but not identical pathological condition, named chronic thromboembolic lung disease. The treatment of choice for CTEPH is pulmonary thrombendarterectomy. Mandatory elements of the surgical protocol include the creation of a «dry» operating field by means of a temporary circulatory arrest under deep hypothermia of 18 °C. Operations for distal lesions of the pulmonary arteries have become a recent achievement. The success of the operation depends on the anatomical type of the lesions, the degree of distal arteriopathy, the extent of the intervention, and the comorbid factors. Immediate and long-term results of the operation are evaluated as good and very good. The long-term survival of patients after surgery is significantly higher than of those who were administered medical treatment.Conclusions. Patients with suspected CTEPH should be referred to a reference expert center for diagnosis verification and operability determining. The standard diagnosis of CTEPH is angiopulmonography with simultaneous measurement of pressure in the right heart; the standard treatment is pulmonary thrombendarterectomy.

Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1)

2016 ◽  
Vol 88 (9) ◽  
pp. 90-101 ◽  
Author(s):  
I E Chazova ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Randomized Clinical Trials ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Diagnosis And Treatment ◽  
Diagnostic Process ◽  
Heart Catheterization ◽  
Main Task ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of ≥25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of ≤15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months. Up to now, our country has had no guidelines for the diagnosis and treatment of this rare severe disease that, when appropriately untreated, has an extremely poor prognosis. The main task in the preparation of this document was to generalize and analyze the data of current registries, multicenter randomized clinical trials, national and international guidelines, and consensus documents recently published on this problem in order to optimize a diagnostic process and treatment in this category of patients. Part 1 gives a definition of CTEPH, its place in the clinical classification, epidemiology and prognosis, risk factors, pathogenesis and morphology, diagnostic approaches and determination of operability in patients, and specific features of differential diagnosis.

scholarly journals Brazilian Thoracic Society recommendations for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 46 (4) ◽  
pp. e20200204-e20200204
Author(s):  
Caio Julio Cesar dos Santos Fernandes ◽  
Jaquelina Sonoe Ota-Arakaki ◽  
Frederico Thadeu Assis Figueiredo Campos ◽  
Ricardo de Amorim Correa ◽  
Marcelo Basso Gazzana ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Diagnosis And Treatment ◽  
Thromboembolic Pulmonary Hypertension

scholarly journals Anesthetic management of pulmonary endarterectomy in a patient with suprasystemic pulmonary pressure: a case report

2020 ◽  
Vol 8 (6) ◽  
pp. 2323
Author(s):  
Pravinkumar H. Patel ◽  
Harvesp Panthakey ◽  
Sandip Katkade ◽  
Hemant Mehta
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Edema ◽  
Chronic Hypoxia ◽  
Anesthetic Management ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Postoperative Management ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Pressure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Indian Male

Pulmonary endarterectomy (PEA) is a potentially curative procedure for chronic thromboembolic pulmonary hypertension. A 34-year Indian male presented with supra-systemic pulmonary pressure was taken for PEA after achievable optimization. A successful PEA was performed with the help of total cardiac arrest. Patient developed reperfusion pulmonary edema post operatively, which was managed medically. The problems encountered by anesthesiologists in this case were preoperative pulmonary hypertension with chronic hypoxia and postoperative management of reperfusion pulmonary edema.

scholarly journals Clinical Updates on the Diagnosis and Management of Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
Vol 49 (5) ◽  
pp. 320-330
Author(s):  
Wen Ruan ◽  
Jonathan JL Yap ◽  
Kevin KH Quah ◽  
Foong Koon Cheah ◽  
Ghee Chee Phuah ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Residual Disease ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Pulmonary Endarterectomy ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Cardinal Symptom

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options. Methods: A PubMed search on articles relevant to pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed. Results: CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catherisation is important in the final diagnosis of CTEPH. Conclusion: Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options. Keywords: Balloon pulmonary angioplasty, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism, Pulmonary endarterectomy, Pulmonary hypertension

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 Adherence to disease-specific drug treatment among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 6 (4) ◽  
pp. 00299-2020
Author(s):  
Barbro Kjellström ◽  
Anna Sandqvist ◽  
Clara Hjalmarsson ◽  
Magnus Nisell ◽  
Per Näsman ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Specific Treatment ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Oral Disease ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Disease Specific ◽  
Time Since Diagnosis

BackgroundPulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong treatment. The aim of the present study was to investigate adherence to disease-specific treatment in patients with PAH or CTEPH.MethodsThe study comprised an adult population diagnosed with PAH (n=384) or CTEPH (n=187) alive in 2016–2017. The study utilised three registries: the Swedish PAH registry, the National Board of Health and Welfare, and Statistics Sweden. Withdrawals from pharmacies of disease-specific oral treatments were studied. Adherence was assessed as: 1) Number of days covered defined as the difference between the total number of daily dosages dispensed and the total number of days covered; and 2) Manual assessment by two persons that independently reviewed each patient's prescription fill history to detect anomalies or patterns of deteriorating or improving adherence over time.ResultsThe mean age was 61±16 years, 61% were female and mean time since diagnosis was 4.6 years. Adherence was 62% using the Number of days covered method and 66% by the Manual assessment method. Drug-specific adherence varied from 91% for riociguat to 60% for sildenafil. Good adherence was associated with shorter time since diagnosis in patients with PAH and with lower number of concomitant other chronic treatments in patients with CTEPH. Age, sex, socioeconomic status or number of pulmonary hypertension (PH) treatments were not associated with adherence.ConclusionAdherence to oral disease-specific treatment was 60–66% and associated with time since diagnosis and number of concomitant chronic treatments. Sex, age or socioeconomic factors did not affect adherence.

scholarly journals The changing landscape of chronic thromboembolic pulmonary hypertension management

2017 ◽  
Vol 26 (146) ◽  
pp. 170105 ◽  
Author(s):  
Michael Madani ◽  
Takeshi Ogo ◽  
Gérald Simonneau
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Of Care ◽  
Surgical Removal ◽  
Pulmonary Endarterectomy ◽  
Current Standard ◽  
Beneficial Impact ◽  
Background Therapy ◽  
Thromboembolic Pulmonary Hypertension

For patients with chronic thromboembolic pulmonary hypertension (CTEPH), the current standard of care involves surgical removal of fibro-thrombotic obstructions by pulmonary endarterectomy. While this approach has excellent outcomes, significant proportions of patients are not eligible for surgery or suffer from persistent/recurrent pulmonary hypertension after the procedure. The availability of balloon pulmonary angioplasty and the approval of the first medical therapy for use in CTEPH have significantly improved the outlook for patients ineligible for pulmonary endarterectomy. In this comprehensive review, we discuss the latest developments in the rapidly evolving field of CTEPH. These include improvements in imaging modalities and advances in surgical and interventional techniques, which have broadened the range of patients who may benefit from such procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed, particularly the encouraging data from the recent MERIT-1 trial, which demonstrated the beneficial impact of using macitentan to treat patients with inoperable CTEPH, including those on background therapy. As the treatment options for CTEPH improve, hybrid management involving more than one intervention in the same patient may become a viable option in the near future.

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