New International CTEPH Database

The New International CTEPH Database is a prospective, observational multi-center disease registry run by the International CTEPH Association (ICA), which will collect data in chronic thromboembolic pulmonary hypertension (CTEPH) patients worldwide. The registry will run for approximately 5 years. Its objective is to provide an overview on epidemiology of CTEPH, mode of diagnosis and treatment approaches worldwide as well as determinants of long-term outcomes as measured by New York Heart Association (NYHA) functional class and survival.

The data collected will improve the knowledge and understanding of this condition, support the further development of diagnosis and treatment guidelines for patients with CTEPH, and contribute to improving patient care in the long-term.

Study Overview

• Status: Completed
• Conditions: Chronic Thromboembolic Pulmonary Hypertension; CTEPH

• Study Type: Observational
• Enrollment (Actual): 1019

Contacts and Locations

Study Locations

• Australia
  • Darlinghurst, Australia, NSW 2010
    • St Vincent's Hospital
  • Prahran, Australia, 3181
    • The Alfred Hospital
• Austria
  • Vienna, Austria, 1090
    • University Clinics of Vienna
• Belgium
  • Leuven, Belgium, 3000
    • University Hospitals of Leuven
• Brazil
  • Sao Paulo, Brazil, 05403900
    • University of Sao Paulo Medical School
  • São Paulo, Brazil, 04023-062
    • Hospital São Paulo, Federal University of São Paulo
• Canada
  • Ontario
    • Toronto, Ontario, Canada, ON M5G 2C4
      • Toronto General Hospital
• Denmark
  • Aarhus, Denmark, 8200
    • Aarhus University Hospital
• Egypt
  • Cairo, Egypt, 12411
    • Kasr Alaini School of Medicine
• Finland
  • Helsinki, Finland, 00250
    • Helsinki University Hospital
• Germany
  • Bad Nauheim, Germany, 61231
    • Kerckhoff-Klinik GmbH
  • Hannover, Germany, 30625
    • Medizinische Hochschule Hannover
  • Homburg, Germany, 66421
    • Universitätsklinikum des Saarlandes
• Italy
  • Pavia, Italy, 27100
    • University of Pavia
• Japan
  • Chiba, Japan, 260-8670
    • Chiba University Hospital
  • Kobe, Japan, 750-0017
    • Kobe University Graduate School of Medicine
  • Okayama, Japan, 701-1192
    • National Hospital Organization Okayama
  • Tokyo, Japan, 181-8611
    • Kyorin University Hospital
• Korea, Republic of
  • Seoul, Korea, Republic of, 135-710
    • Samsung Medical Center
• Netherlands
  • Amsterdam, Netherlands, 1081
    • VU University Medical Center
• Poland
  • Otwock, Poland, 05-400
    • Europejskim Centrum Zdrowia Otwock
  • Warsaw, Poland, 00-005
    • Medical University of Warsaw
  • Wroclaw, Poland, 51-124
    • Wojewodzki Szpital Specjalistyczny
• Russian Federation
  • Moscow, Russian Federation, 121552
    • Cardiology Research Center
  • Novosibirsk, Russian Federation, 630055
    • Novosibirsk Research Institute of Circulation Pathology Academician E.N.Meshalkin
• Slovakia
  • Bratislava, Slovakia, 83348
    • Slovak Medical University and National Institute for Cardiovascular Diseases
• Spain
  • Barcelona, Spain, 8036
    • Hospital Clinic Barcelona
  • Madrid, Spain, 28041
    • Hospital Universitarion 12 Octubre
• Switzerland
  • Zürich, Switzerland, 8005
    • Universitatsspital Zurich
• Taiwan
  • Taipei City, Taiwan, 10002
    • National Taiwan University Hospital
• Turkey
  • Istanbul, Turkey, 34899
    • Marmara University School of Medicine
• United Kingdom
  • Cambridge, United Kingdom, CB3 8RE
    • Papworth Hospital
  • Glasgow, United Kingdom, G11 6NT
    • Western Infirmary
  • London, United Kingdom, MW3 2QG
    • Royal Free London NHS Foundation Trust
  • Sheffield, United Kingdom, S10 2JF
    • Sheffield Teaching Hospitals NHS Foundation Trust
• United States
  • California
    • La Jolla, California, United States, 92037-7892
      • UC San Diego
  • Maryland
    • Baltimore, Maryland, United States, 21201
      • University of Maryland
  • Ohio
    • Cleveland, Ohio, United States, 44195
      • Cleveland Clinic

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients recently diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), meeting the registry's inclusion criteria, are eligible for inclusion into the New International CTEPH Database.

Only newly diagnosed, i.e. incident patients are eligible for inclusion. A patient is considered an incident patient if a diagnostic heart catheterization was performed within 1 year prior to the initial inclusion visit (for the first patient included) at the site participating in the New International CTEPH Database.

Description

Inclusion Criteria:

• Patients must be newly diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) (incident patients)
• Patients must have been treated with anti-coagulation for at least 3 months before diagnosis of CTEPH
• Patients must be willing to provide informed consent

• Patients must meet the following criteria for CTEPH:

  • Mean pulmonary artery pressure (PAP) ≥ 25 mmHg at rest
  • Abnormal VQ scan, pulmonary angiogram, computed tomography (CT) pulmonary angiogram, or magnetic resonance (MR) pulmonary angiogram confirming chronic thromboembolic disease as recommended by standard guidelines

Exclusion Criteria:

• None specified

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Death	Min. 3 years
Lung transplantation	Min. 3 years
Change in New York Heart Association (NYHA) functional class	Min 3. years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Age at diagnosis		Min. 3 years
Gender		Min. 3 years
Ethnicity		Min. 3 years
Disease severity	As measured by New York Heart Association (NYHA) functional class	Min. 3 years
History of deep vein thrombosis (DVT)		Min. 3 years
History of acute pulmonary embolism		Min. 3 years
Hemodynamic parameters		Min. 3 years
Patient on oxygen (yes/no)		Min. 3 years
Medical treatment	e.g. mono- versus combination therapy	Min. 3 years
Pulmonary endarterectomy (PEA)	e.g. operated versus non-operated	Min. 3 years
Balloon pulmonary angioplasty (BPA)	e.g. BPA versus pulmonary endarterectomy (PEA)	Min. 3 years

Collaborators and Investigators

• Sponsor: International CTEPH Association

Publications and helpful links

General Publications

• Guth S, D'Armini AM, Delcroix M, Nakayama K, Fadel E, Hoole SP, Jenkins DP, Kiely DG, Kim NH, Lang IM, Madani MM, Matsubara H, Ogawa A, Ota-Arakaki JS, Quarck R, Sadushi-Kolici R, Simonneau G, Wiedenroth CB, Yildizeli B, Mayer E, Pepke-Zaba J. Current strategies for managing chronic thromboembolic pulmonary hypertension: results of the worldwide prospective CTEPH Registry. ERJ Open Res. 2021 Aug 16;7(3):00850-2020. doi: 10.1183/23120541.00850-2020. eCollection 2021 Jul.

Helpful Links

• Description of the registry on the website of the International CTEPH Association
• Abstract on long-term outcomes presented at ERS 2021

Study record dates

Study Major Dates

• Study Start: February 1, 2015
• Primary Completion (Actual): January 1, 2020
• Study Completion (Actual): January 1, 2020

Study Registration Dates

• First Submitted: January 12, 2016
• First Submitted That Met QC Criteria: January 12, 2016
• First Posted (Estimate): January 14, 2016

Study Record Updates

• Last Update Posted (Actual): August 8, 2022
• Last Update Submitted That Met QC Criteria: August 4, 2022
• Last Verified: August 1, 2022

More Information

Terms related to this study

• Keywords: Chronic thromboembolic pulmonary hypertension; PEA; CTEPH; Pulmonary endarterectomy; Balloon pulmonary angioplasty; BPA
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Hypertension, Pulmonary
• Other Study ID Numbers: CTEPH Registry