A Registry for United States Patients with Chronic Thromboembolic Pulmonary Hypertension


About this study

The purpose of this registry will be to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of United States patients with chronic thromboembolic pulmonary hypertension through shared information, education, and collaborative investigation among pulmonary hypertension centers throughout the U.S.

Participation eligibility

Participant eligibility includes age, gender, type and stage of disease, and previous treatments or health concerns. Guidelines differ from study to study, and identify who can or cannot participate. There is no guarantee that every individual who qualifies and wants to participate in a trial will be enrolled. Contact the study team to discuss study eligibility and potential participation.

Inclusion Criteria

  • Must be a permanent resident of the United States
  • Has documentation of the following hemodynamic parameters by right heart catheterization
    • Mean pulmonary arterial pressure (PAPm) ≥ 25 mm Hg at rest
    • Pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg (or > 15 mmHg if justified)
  • Radiologic confirmation that chronic thromboembolic disease is the cause of the pulmonary hypertension by
    • One or more mismatched perfusion defect(s) by lung ventilation/perfusion scan and
    • Confirmation of chronic thromboembolic disease by evidence of bands/webs, vessel narrowing or occlusion seen on CT pulmonary angiogram (CTA), conventional angiography or MR angiography (MRA)
  • Must be diagnosed with chronic thromboembolic pulmonary hypertension within 6 months of being considered for study eligibility (signing of consent to participate)
    • Date of diagnosis will be defined as when both hemodynamic criteria have been met and chronic thromboembolic disease is confirmed to be the cause of the pulmonary hypertension by an abnormal V/Q scan and the presence of chronic thromboembolic disease on CTA, MRA or pulmonary angiography
    • Hemodynamic and radiologic criteria can be met at separate time points
    • The most recently met criteria time point will be defined as the date of diagnosis.
  • Prior to enrollment all subjects must have the diagnosis of CTEPH confirmed by the Adjudication Committee


Exclusion Criteria

  • Unwilling or unable to provide written consent for participation in the study
    • Appropriate surrogate consent will be obtained for pediatric patients as defined by each investigational site's IRB
  • An underlying medical disorder with an anticipated life expectancy less than 2 years
  • Do not meet inclusion criteria including
    • Have not had documentation of hemodynamic criteria by right heart catheterization as outlined in the inclusion criteria
    • Do not have radiologic confirmation of chronic thromboembolic disease as outlined in the inclusion criteria
    • Meet the criteria for inclusion into WHO Groups I, II, III, or V

Participating Mayo Clinic locations

Study statuses change often. Please contact the study team for the most up-to-date information regarding possible participation.

Mayo Clinic Location Status

Rochester, Minn.

Mayo Clinic principal investigator

Michael Krowka, M.D.

Closed for enrollment

More information


  • Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have permitted comparisons between populations in different eras and environments. Inclusion of patients with all subtypes of PAH has also allowed comparisons of these subpopulations. We describe herein the basic methodology by which PAH registries have been conducted, review key insights provided by registries, summarize issues related to interpretation and comparison of the results, and discuss the utility of data to predict survival outcomes. Potential sources of bias, particularly related to the inclusion of incident and/or prevalent patients and missing data, are addressed. A fundamental observation of current registries is that survival in the modern treatment era has improved compared with that observed previously and that outcomes among PAH subpopulations vary substantially. Continuing systematic clinical surveillance of PAH will be important as treatment evolves and as understanding of mechanisms advance. Considerations for future directions of registry studies include enrollment of a broader population of patients with pulmonary hypertension of all clinical types and severity and continued globalization and collaboration of registry databases. Read More on PubMed
  • Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France. Read More on PubMed
  • Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. Read More on PubMed

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