Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term Outcome
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|ClinicalTrials.gov Identifier: NCT02565030|
Recruitment Status : Active, not recruiting
First Posted : October 1, 2015
Last Update Posted : June 14, 2018
|Condition or disease|
|Chronic Thromboembolic Pulmonary Hypertension Idiopathic Pulmonary Arterial Hypertension|
Chronic thrombo-embolic pulmonary hypertension (CTEPH) is thought to represent the consequence of failure of thrombus resolution after the establishment of thrombosis within the elastic pulmonary arteries. Thrombotic material becomes fibrosed, with occlusive vascular remodeling and the development of a secondary small vessel arteriopathy, termed the two-compartment model. This results in an increase in pressure and vascular resistance in the pulmonary vasculature and leads eventually to right heart failure.
It has been demonstrated that 2 year survival for CTEPH patients with mean pulmonary arterial pressure(mPAP) >50 mm Hg was only 20%, however, preliminary data from Sheffield Teaching Hospitals suggest that survival is significantly better than this. Until recently surgery was the only treatment for CTEPH in selected patients. Studies have shown the 10 year survival in patients with CTEPH after PEA surgery was 74%. More recently, for patients with inoperable CTEPH, drug therapy has been shown to improve exercise capacity. Despite recent publications the natural history and mechanisms underlying CTEPH are poorly understood.
The main area of research focus in this study will be improving understanding of the natural history of chronic thromboembolic pulmonary hypertension. The purpose of this research protocol is to conduct a retrospective review of patients with CTEPH diagnosed at the Sheffield Centre between 2001 and 2014 to understand more about the natural history of disease including prognostic indicators. The Sheffield Service has one of the largest cohorts of CTEPH in the world (>650 diagnosed cases since 2001). Approximately half of these patients undergo curative surgery with pulmonary endarterectomy. However, a significant proportion of patients with operable disease decline surgery and a significant proportion of patients have disease that is too distal for surgery, so called distal CTEPH. There is a relative paucity of data on i) the natural history of this disease, ii) an understanding of the relative contribution of obstruction and distal vasculopathy to elevations in pulmonary vascular resistance iii) the effects of distal CTEPH on right ventricular function and iv) how distal CTEPH differs from idiopathic pulmonary arterial hypertension (IPAH).
The second part of this study will focus on understanding disease mechanisms utilising MR imaging techniques and using the Sheffield PH Biobank resource to understand differences in the IPAH and distal CTEPH cohorts. The Sheffield PH Biobank has samples and detailed phenotypic data on over 400 treatment naïve patients with all forms of pulmonary hypertension with serial sampling and long term follow up providing a unique resource to make detailed comparisons across all forms of pulmonary hypertension.
|Study Type :||Observational|
|Estimated Enrollment :||1200 participants|
|Official Title:||Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term Outcome|
|Actual Study Start Date :||February 2015|
|Estimated Primary Completion Date :||October 2021|
|Estimated Study Completion Date :||October 2021|
CTEPH surgical disease, operated
Patients with proximal CTEPH who have undergone Pulmonary Endarterectomy (PEA) surgery
CTEPH surgical disease, not operated
Patients with proximal CTEPH with operable distribution of disease& have not undergone PEA surgery due to the following reasons:
CTEPH non surgical
Patients with distal CTEPH with inoperable distribution of disease inaccessable to surgery.
Patients with IPAH as per European Society of Cardiology(ESC) criteria
- Death [ Time Frame: Upto 176 months ]All patients with CTEPH or IPAH who have been documented as having died between 01/01/2001 and 01/09/2015
- Mean Pulmonary Arterial Pressure(mm of Hg) [ Time Frame: Upto 176 months ]Measure of the final Mean Pulmonary Arterial Pressure with Right Heart Catheter studies in patients with CTEPH and IPAH reported between 01/01/2001 and 01/09/2015
- Diffusion across the lung of Carbon Monoxide(mmol/min/KPa) [ Time Frame: Upto 176 months ]Measure of final Diffusion across the lung of Carbon Monoxide in patients with CTEPH & IPAH reported between 01/01/2001 and of 01/09/2015
- Emphasis 10 Quality Of Life Questionaire Score(0-50) [ Time Frame: Upto 176 months ]Measure of the Emphasis 10 Quality of Life Questionaire Score in patients with CTEPH and IPAH in their final clinic visit reported between 01/01/2001 and 01/09//2015
- Incremental Shuttle Walk Distance( in metres) [ Time Frame: Upto 176 months ]The measure of the final Incremental Shuttle Walk Distance of patients with CTEPH and IPAH reported between 01/01/2015 and 01/09/2015
- World Health Organization Functional Class(I-IV) [ Time Frame: Upto 176 months ]Measure of the final World Health Organization Functional Class in patients with CTEPH and IPAH reported between 01/01/2001 and 01/09/2015
- Right Ventricular Ejection Fraction(%) [ Time Frame: Upto 176 months ]Measure of the final Right Ventricular Ejection Fraction with Cardiac Magnetic Resonance Imaging(MRI) in patients with CTEPH and IPAH reported between 01/01/2001 and 01/09/2015
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02565030
|Sheffield Teaching Hospitals NHS Foundation Trust|
|Sheffield, South Yorkshire, United Kingdom, S10 2JF|
|Principal Investigator:||Syed Quadery, MBBS||Sheffield Teaching Hospitals NHS Foundation Trust|
|Study Chair:||David Kiely, MD||Sheffield Teaching Hospitals NHS Foundation Trust|
|Study Director:||Wild Jim, PhD||University Of Sheffield, School of Medicine|
|Study Director:||Andy Swift, MD||University Of Sheffield , School Of Medicine|