Clinical Trial: Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term Outcome

Study Status: Active, not recruiting
Recruit Status: Active, not recruiting
Study Type: Observational

Official Title: Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term Outcome

Brief 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.

Detailed Summary:

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 propor
Sponsor: Sheffield Teaching Hospitals NHS Foundation Trust

Current Primary Outcome: 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


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • 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


Original Secondary Outcome:

  • 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
  • Diffussion across the lung of Carbon Monoxide(mmol/min/KPa) [ Time Frame: Upto 176 months ]
    Measure of final Diffussion across the lung of Carbon Monoxide in patients with CTEPH & IPAH reported between 01/01/2001 and of 01/09/2015
  • Emphais 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 beteween 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 MRI in patients with CTEPH and IPAH reported between 01/01/2001 and 01/09/2015


Information By: Sheffield Teaching Hospitals NHS Foundation Trust

Dates:
Date Received: September 28, 2015
Date Started: February 2015
Date Completion: October 2021
Last Updated: April 24, 2017
Last Verified: April 2017