Clinical Trials Logo

Clinical Trial Summary

This study will help to provide more up to date prognostic data on the natural history of untreated proximal CTEPH which will be helpful in discussions regarding surgical versus medical treatment and by exploring the similarities and differences between distal CTEPH and IPAH the investigators hope to get insights into disease mechanisms in patients with distal CTEPH.


Clinical Trial Description

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 Design


Related Conditions & MeSH terms


NCT number NCT02565030
Study type Observational
Source Sheffield Teaching Hospitals NHS Foundation Trust
Contact
Status Completed
Phase
Start date February 2015
Completion date October 2021

See also
  Status Clinical Trial Phase
Active, not recruiting NCT06092424 - High Altitude (HA) Residents With Pulmonary Vascular Diseseases (PVD), Pulmonary Artery Pressure (PAP) Assessed at HA (2840m) vs Sea Level (LA) N/A
Active, not recruiting NCT06003244 - High Altitude (HA) Residents With Pulmonary Vascular Diseases (PVD), 6 Minute Walk Distance (6MWD) Assessed at 2840m (HA) With and Without Supplemental Oxygen Therapy (SOT) N/A
Active, not recruiting NCT06072417 - HA Residents With PVD, SDB Assessed at HA (2840m) vs LA (Sea Level) N/A
Terminated NCT01953965 - Look at Way the Heart Functions in People With Pulmonary Hypertension (PH) Who Have Near Normal Right Ventricle (RV) Function and People With Pulmonary Hypertension Who Have Impaired RV Function. Using Imaging Studies PET Scan and Cardiac MRI. Phase 2
Recruiting NCT02061787 - the Application of Cardiopulmonary Exercise Testing in Assessment Outcome of Patients With Pulmonary Hypertension
Not yet recruiting NCT03102294 - Inspiratory Muscle Training in Chronic Thromboembolic Pulmonary Hypertension N/A
Recruiting NCT04071327 - Pulmonary Hypertension Association Registry
Completed NCT00313222 - Bosentan Effects in Inoperable Forms of Chronic Thromboembolic Pulmonary Hypertension Phase 3
Recruiting NCT05311072 - Change-a Multi-center Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Database in China
Recruiting NCT05340023 - Proteomic Pattern Associated With the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension
Enrolling by invitation NCT05568927 - Validation of SEARCH, a Novel Hierarchical Algorithm to Define Long-term Outcomes After Pulmonary Embolism
Completed NCT03786367 - Dyspnea in Chronic Thromboembolic Pulmonary Hypertension
Recruiting NCT04081012 - N-acetyl Cysteine in Post-reperfusion Pulmonary Injury in Chronic Thromboembolic Pulmonary Hypertension. N/A
Not yet recruiting NCT06384534 - Exercise Performance on Ambient Air vs. Low-Flow Oxygen Therapy in Pulmonary Vascular Disease (PVD) N/A
Withdrawn NCT05693779 - Exercise Therapy After Pulmonary Thromboendarterectomy or Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension N/A
Not yet recruiting NCT02426203 - 3D Echocardiographic Assessment of RV Function in Patients Undergoing Pulmonary Endarterectomy N/A
Completed NCT02094001 - Pilot Study to Evaluate Right Ventricular Function With Riociguat in CTEPH Phase 2
Enrolling by invitation NCT03388476 - Endtidal Carbon Dioxide for Earlier Detection of Pulmonary Hypertension
Recruiting NCT04206852 - Safety and Efficacy of Balloon Pulmonary Angioplasty in China
Completed NCT02111980 - RF Surgical Sponge-Detecting System on the Function of Pacemakers and Implantable Cardioverter Defibrillators N/A