U.S. CTEPH Registry

NCT02429284 | Completed DMC

• 1 other identifier: PH-2013-008
• Study Type: observational
• Target: 754
• Locations: 1 country
• Sites: 1

Brief Summary

The U.S. CTEPH Registry is a multicenter, observational, U.S.-based study of the clinical course and treatment of patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), WHO Group IV Classification for Pulmonary Hypertension. The mission of the Registry will be to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH through shared information, education, and collaborative investigation among pulmonary hypertension (PH) centers of excellence throughout the U.S.

Conditions

Chronic Thromboembolic Pulmonary Hypertension (CTEPH); Pulmonary Hypertension

Keywords

Chronic Thromboembolic Pulmonary Hypertension (CTEPH); Pulmonary Endarterectomy (PEA); Pulmonary Thromboendarterectomy (PTE); Pulmonary Hypertension; Balloon Pulmonary Angioplasty (BPA)

Outcome Measures

Primary Outcomes (3)

• Describe the patterns of evaluation of CTEPH Patients

  up to 5 years

• Describe the patters of risk factors and clinical presentation of CTEPH Patients

  up to 5 years

• Outcomes of medical and surgical therapy of CTEPH patients as assessed by hemodynamics, quality of life, and functional status

  up to 5 years, at 6 month intervals

Study Arms (1)

Patients newly diagnosed with CTEPH

Patients newly diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), WHO Group IV Classification for Pulmonary Hypertension.

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

All consecutive consenting patients recently diagnosed (\< 6 months) with CTEPH at participating study sites will be enrolled. Patients must meet inclusion criteria and have the diagnosis of CTEPH confirmed by the Adjudication Committee (AC) to be enrolled. Adult and pediatric patients may be enrolled and prior medical therapy will not exclude patients from enrollment.

You may qualify if:

• Patients must be a permanent resident of the United States
• Documentation of the following hemodynamic parameters by right heart catheterization
• Mean pulmonary arterial pressure (PAPm) ≥ 25 mm Hg at rest and,
• 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).
• Patients must be diagnosed with CTEPH within 6 months of being considered for study eligibility (signing of consent to participate). The 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.

You may not qualify if:

• Patients 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.
• Patients with an underlying medical disorder with an anticipated life expectancy less than 2 years.

Sponsors & Collaborators

• University of California, San Diego: lead
• E Squared Trials and Registries, Inc.: collaborator

Study Sites (1)

UCSD Health System

La Jolla, California, 92037, United States

Location

Related Publications (5)

• Kim NH, Delcroix M, Jenkins DP, Channick R, Dartevelle P, Jansa P, Lang I, Madani MM, Ogino H, Pengo V, Mayer E. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D92-9. doi: 10.1016/j.jacc.2013.10.024.

  PMID: 24355646 BACKGROUND

• McGoon MD, Benza RL, Escribano-Subias P, Jiang X, Miller DP, Peacock AJ, Pepke-Zaba J, Pulido T, Rich S, Rosenkranz S, Suissa S, Humbert M. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D51-9. doi: 10.1016/j.jacc.2013.10.023.

  PMID: 24355642 BACKGROUND

• Pepke-Zaba J, Delcroix M, Lang I, Mayer E, Jansa P, Ambroz D, Treacy C, D'Armini AM, Morsolini M, Snijder R, Bresser P, Torbicki A, Kristensen B, Lewczuk J, Simkova I, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jais X, Simonneau G. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011 Nov 1;124(18):1973-81. doi: 10.1161/CIRCULATIONAHA.110.015008. Epub 2011 Oct 3.

  PMID: 21969018 BACKGROUND

• Kerr KM, Elliott CG, Chin K, Benza RL, Channick RN, Davis RD, He F, LaCroix A, Madani MM, McLaughlin VV, Park M, Robbins IM, Tapson VF, Terry JR, Test VJ, Jain S, Auger WR. Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up. Chest. 2021 Nov;160(5):1822-1831. doi: 10.1016/j.chest.2021.05.052. Epub 2021 Jun 4.

  PMID: 34090871 DERIVED

• Kerr KM, Elliott CG, Benza RL, Channick RN, Chin KM, Davis RD, Jain S, LaCroix AZ, Madani MM, McLaughlin VV, Park MH, Tapson VF, Auger WR. The United States Chronic Thromboembolic Pulmonary Hypertension Registry: Protocol for a Prospective, Longitudinal Study. JMIR Res Protoc. 2021 May 25;10(5):e25397. doi: 10.2196/25397.

  PMID: 33848258 DERIVED

MeSH Terms

Conditions

Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Kim M Kerr, MD

  University of California, San Diego

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 4 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Clinical Professor of Medicine

Study Record Dates

• First Submitted: April 13, 2015
• First Posted: April 29, 2015
• Study Start: April 1, 2015
• Primary Completion: May 1, 2019
• Study Completion: November 1, 2019
• Last Updated: March 24, 2020

Record last verified: 2020-03

Locations

US (1)