scholarly journals Diagnosis of chronic thromboembolic pulmonary hypertension: A Canadian Thoracic Society clinical practice guideline update

2019 ◽  
Vol 3 (4) ◽  
pp. 177-198 ◽  
Author(s):  
Doug Helmersen ◽  
Steeve Provencher ◽  
Andrew M. Hirsch ◽  
Anne Van Dam ◽  
Carole Dennie ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Clinical Practice Guideline ◽  
Practice Guideline ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Thromboembolic Pulmonary Hypertension

scholarly journals Diagnostic Evaluation and Management of Chronic Thromboembolic Pulmonary Hypertension: A Clinical Practice Guideline

2010 ◽  
Vol 17 (6) ◽  
pp. 301-334 ◽  
Author(s):  
Sanjay Mehta ◽  
Doug Helmersen ◽  
Steeve Provencher ◽  
Naushad Hirani ◽  
Fraser D Rubens ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Clinical Practice Guideline ◽  
Computed Tomography Angiography ◽  
Practice Guideline ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Impact

BACKGROUND: Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.METHODS: A representative interdisciplinary panel of medical experts undertook a formal clinical practice guideline development process. A total of 20 key clinical issues were defined according to the patient population, intervention, comparator, outcome (PICO) approach. The panel performed an evidence-based, systematic, literature review, assessed and graded the relevant evidence, and made 26 recommendations.RESULTS: Asymptomatic patients postpulmonary embolism should not be screened for CTEPH. In patients with pulmonary hypertension, the possibility of CTEPH should be routinely evaluated with initial ventilation/ perfusion lung scanning, not computed tomography angiography. Pulmonary endarterectomy surgery is the treatment of choice in patients with surgically accessible CTEPH, and may also be effective in CTEPH patients with disease in more ‘distal’ pulmonary arteries. The anatomical extent of CTEPH for surgical pulmonary endarterectomy is best assessed by contrast pulmonary angiography, although positive computed tomography angiography may be acceptable. Novel medications indicated for the treatment of pulmonary hypertension may be effective for selected CTEPH patients.CONCLUSIONS: The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.

A survey on pulmonary thromboendarterectomy in the Asia-Pacific region

2017 ◽  
Vol 25 (5) ◽  
pp. 345-349
Author(s):  
Mohammad Bashar Izzat ◽  
Farouk Almohammad ◽  
Ahmad F Raslan
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Practice Guidelines ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Asia Pacific ◽  
Pacific Region ◽  
Asia Pacific Region ◽  
Pulmonary Thromboendarterectomy ◽  
Healthcare Facilities ◽  
Thromboembolic Pulmonary Hypertension

Background Surgical pulmonary thromboendarterectomy has been established as the treatment of choice for chronic thromboembolic pulmonary hypertension. We conducted a survey among Asia-Pacific cardiothoracic surgeons to examine their current practice demographics and reflect their views on the future prospects for pulmonary thromboendarterectomy surgery. Methods All cardiothoracic surgeons who were registered on the CTSNet.org website and based in the Asia-Pacific region were invited to participate in an online survey. The electronic questionnaire was completed by 172 (6.3%) surgeons. Responses were recorded anonymously and tabularized as absolute figures and fractions. Results Pulmonary thromboendarterectomy surgery has been performed by few Asia-Pacific surgeons and in small numbers of patients, but survival rates and functional outcomes were satisfactory in most experiences. Failures were thought to have resulted primarily from the difficulty in selecting suitable candidates for surgery. The need for greater clinical experience was clear, yet this might be hampered by the limited recognition of pulmonary thromboendarterectomy surgery as a potential cure for chronic thromboembolic pulmonary hypertension. Most surgeons considered that this procedure should be restricted to designated expert centers, and acknowledged the need to organize dedicated regional meetings where clinical practice guidelines can be established and updated as appropriate for regional healthcare facilities. Conclusions Although much effort is needed before pulmonary thromboendarterectomy surgery becomes widely adopted in the Asia-Pacific region, many surgeons and centers are already seeking its implementation. Designating expert centers, bringing together a regional working group on pulmonary thromboendarterectomy, and establishing clinical practice guidelines as appropriate for regional healthcare facilities were recommended.

scholarly journals Diagnosis of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism

2020 ◽  
Vol 55 (6) ◽  
pp. 2000189 ◽  
Author(s):  
Fredrikus A. Klok ◽  
Francis Couturaud ◽  
Marion Delcroix ◽  
Marc Humbert
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Acute Pulmonary Embolism ◽  
Functional Limitations ◽  
Diagnostic Delay ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Clear Guideline

Chronic thromboembolic pulmonary hypertension (CTEPH) is the most severe long-term complication of acute pulmonary embolism (PE). Untreated CTEPH is fatal, but, if diagnosed in time, successful surgical (pulmonary endarterectomy), medical (pulmonary hypertension drugs) and/or interventional (balloon pulmonary angioplasty) therapies have been shown to improve clinical outcomes, especially in case of successful pulmonary endarterectomy. Early diagnosis has however been demonstrated to be challenging. Poor awareness of the disease by patients and physicians, high prevalence of the post-PE syndrome (i.e. persistent dyspnoea, functional limitations and/or decreased quality of life following an acute PE diagnosis), lack of clear guideline recommendations as well as inefficient application of diagnostic tests in clinical practice lead to a reported staggering diagnostic delay >1 year. Hence, there is a great need to improve current clinical practice and diagnose CTEPH earlier. In this review, we will focus on the clinical presentation of and risk factors for CTEPH, and provide best practices for PE follow-up programmes from expert centres, based on a clinical case.

scholarly journals Safety and effectiveness of riociguat for chronic thromboembolic pulmonary hypertension in real-world clinical practice: interim data from post-marketing surveillance in Japan

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093898
Author(s):  
Nobuhiro Tanabe ◽  
Takeshi Ogo ◽  
Masaru Hatano ◽  
Ayaka Kigawa ◽  
Toshiyuki Sunaya ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Adverse Drug Reactions ◽  
Real World ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
World Health ◽  
Drug Reactions ◽  
Thromboembolic Pulmonary Hypertension ◽  
Health Organization

This multicenter, prospective, non-interventional study (ClinicalTrials.gov: NCT02117791) evaluated the safety and effectiveness of riociguat for chronic thromboembolic pulmonary hypertension in Japanese clinical practice, registering all patients with chronic thromboembolic pulmonary hypertension treated with riociguat following its launch in Japan in April 2014. Safety was assessed by analyzing the adverse drug reactions. Effectiveness measurements included the assessment of change in World Health Organization functional class, six-minute walk test, and hemodynamics. Overall, 1031 patients were included in the safety analysis with 811 (78.7%) patients in World Health Organization functional class II/III. The mean treatment duration was 591.4 days (median 441.0 days). Adverse drug reactions were reported in 19.5% of patients, the most common being hypotension (5.9%), headache (3.0%), dizziness (1.9%), and gastroesophageal reflux disease (1.5%). Serious adverse drug reactions were reported in 2.1% of patients. Estimated survival was 97.0% at one year, 95.8% at two years, and 94.4% at three years. The effectiveness analysis ( n = 1027) showed significant increases from baseline in six-minute walking distance, and significant reductions from baseline in mean pulmonary arterial pressure and pulmonary vascular resistance. These interim results of riociguat in Japanese patients with chronic thromboembolic pulmonary hypertension demonstrated a safety profile that was generally consistent with those of pivotal clinical studies. The study is ongoing, and will continue to provide insights into the safety and effectiveness of riociguat in real-world practice.

scholarly journals 2020 Clinical practice guidelines for Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension

2021 ◽  
Vol 26 (12) ◽  
pp. 4683
Author(s):  
S. N. Avdeev ◽  
O. L. Barbarash ◽  
A. E. Bautin ◽  
A. V. Volkov ◽  
T. N. Veselova ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Russian Society ◽  
Thromboembolic Pulmonary Hypertension ◽  
National Congress

Russian Society of Cardiology (RSC)With the participation: Association of Cardiovascular Surgeons of Russia, Russian Respiratory Society, Federation of Anesthesiologists and Resuscitators, Association of Rheumatologists of Russia, National Congress of Radiation Diagnosticians.

scholarly journals Population pharmacokinetics of riociguat and its metabolite in patients with chronic thromboembolic pulmonary hypertension from routine clinical practice

2020 ◽  
Vol 10 (1) ◽  
pp. 204589401989803
Author(s):  
Danica Michaličková ◽  
Pavel Jansa ◽  
Miroslava Bursová ◽  
Tomáš Hložek ◽  
Radomír Čabala ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Pulmonary Hypertension ◽  
Clinical Practice ◽  
Creatinine Clearance ◽  
Randomized Clinical Trials ◽  
Total Bilirubin ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Routine Clinical Practice ◽  
Population Pharmacokinetic ◽  
Thromboembolic Pulmonary Hypertension

Pharmacokinetic data for riociguat in patients with chronic thromboembolic pulmonary hypertension (CTEPH) have previously been reported from randomized clinical trials, which may not fully reflect the population encountered in routine practice. The aim of the current study was to characterize the pharmacokinetic of riociguat and its metabolite M1 in the patients from routine clinical practice. A population pharmacokinetic model was developed in NONMEM 7.3, based on riociguat and its metabolite plasma concentrations from 49 patients with CTEPH. One sample with riociguat and M1 concentrations was available from each patient obtained at different time points after last dose. Age, bodyweight, sex, smoking status, concomitant medications, kidney and liver function markers were tested as potential covariates of pharmacokinetic of riociguat and its metabolite. Riociguat and M1 disposition was best described with one-compartment models. Apparent volume of distribution (Vd/F) for riociguat and M1 were assumed to be the same. Total bilirubin and creatinine clearance were the most predictive covariates for apparent riociguat metabolic clearance to M1 (CLf,M1/F) and for apparent riociguat clearance through remaining pathways (CLe,r/F), respectively. CLf,M1/F, CLe,r/F, Vd/F of riociguat and M1, and clearance of M1 (CLe,M1/F) for a typical individual with 70 mL/min creatinine clearance and 0.69 mg/dL total bilirubin were 0.665 L/h (relative standard error = 17%)), 0.66 (18%) L/h, 3.63 (15%) L and 1.47 (19%) L/h, respectively. Upon visual identification of six outlying individuals, an absorption lag-time of 2.95 (6%) h was estimated for these patients. In conclusion, the only clinical characteristics related to riociguat exposure in patients with CTEPH from routine clinical practice are total bilirubin and creatinine clearance. This confirms the findings of the previous population pharmacokinetic studies based on data from randomized clinical trials.

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