TY - JOUR
T1 - The United States Chronic Thromboembolic Pulmonary Hypertension Registry
T2 - Protocol for a prospective, longitudinal study
AU - Kerr, Kim M.
AU - Elliott, C. Greg
AU - Benza, Raymond L.
AU - Channick, Richard N.
AU - Chin, Kelly M.
AU - Davis, R. Duane
AU - Jain, Sonia
AU - LaCroix, Andrea Z.
AU - Madani, Michael M.
AU - McLaughlin, Vallerie V.
AU - Park, Myung H.
AU - Tapson, Victor F.
AU - Auger, William R.
N1 - Funding Information:
KK received a research grant paid to the institution from Bayer HealthCare and is a consultant to Actelion. CGE sits on the steering committee for UCSD (via grant from Bayer). KC is a consultant and sits on the steering/adjudication committees of Actelion, UCSD (via a grant from Bayer), United Therapeutics, Altavant, and Gossamer Bio. KC receives research support (paid to the institution) from Actelion, Ironwood, National Institutes of Health, and SoniVie, and receives financial/material support from the American Heart Association. RB is on the advisory board of Bayer. RC receives research support (paid to institution) from Bayer and Actelion; is a consultant for Actelion, Bayer, United Therapeutics, and Gossamer; and is part of the Speakers Bureau for Actelion and Bayer. MM is a consultant for Bayer, Actelion, and Wexler Surgical. VM receives research support (paid to institution) from Acceleron, Actelion, Gilead, Sonovie, Reata, and United Therapeutics, and performs scientific consulting for Acceleron, Actelion, Altavant, Caremark, LLC, CiVi Biopharma Inc, Gossamer Bio, Liquidia, and United Therapeutics. MP is a consultant for Bayer, Actelion, Abbott, and AstraZeneca; is part of the Speakers Bureau for Bayer; and received travel support from Bayer and Actelion. VFT receives research support (paid to institution) by National Institutes of Health, Actelion, Bayer, BMS/Pfizer, United Therapeutics, EKOS/BTG, Inari, Penumbra, and Johnson & Johnson; is on the advisory board/offers consulting to Actelion, BMS/Pfizer, United Therapeutics, EKOS/BTG, National Institutes of Health, Bayer, Johnson & Johnson, and Thrombolex; and has received speaking honoraria from Janssen and EKOS/BTG. WA is a consultant for Bayer, Cereno Scientific, and Actelion. The other authors have no conflicts of interest to declare.
Funding Information:
The Steering Committee would like to thank Jeff Terry, MBA, CCRP, for his patience and tireless leadership as Manager of the US-CTEPH-R. We also would like to thank Kathy Feldkircher, PhD, and Abby Poms, RRT, CCRP, of E-Squared Trials and Registries. Their extensive experience, knowledge, enthusiasm, and effort contributed to the development and success of this registry. This registry was funded by an Investigator Initiated Study Research Grant from Bayer Healthcare Pharmaceuticals awarded to The Regents of the University of California, UCSD. The sponsor had no role in the design of the study, the collection and analysis of the data, or the preparation of the manuscript.
Publisher Copyright:
©Kim M Kerr, C Greg Elliott, Raymond L Benza, Richard N Channick, Kelly M Chin, R Duane Davis, Sonia Jain, Andrea Z LaCroix, Michael M Madani, Vallerie V McLaughlin, Myung H Park, Victor F Tapson, William R Auger.
PY - 2021/5
Y1 - 2021/5
N2 - Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common, and diagnostic and treatment guidelines are often not followed. Data gathered from international registries have improved our understanding of CTEPH, but these data may not be applicable to the US population owing to differences in demographics and medical practice patterns. Objective: The US CTEPH Registry (US-CTEPH-R) was developed to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as the short- and long-term outcomes of surgical and nonsurgical therapies in the modern treatment era. Methods: Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of patients newly diagnosed with CTEPH. Enrollment criteria included a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multidisciplinary adjudication committee. Following enrollment, subjects were followed biannually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, and all other testing was at the discretion of the treating clinician. Details regarding surgical therapy, balloon pulmonary angioplasty, and medical therapy were collected at enrollment and at follow-up, as well as information related to health care utilization and survival. Results: Data from this registry will improve understanding of the demographics, risk factors, and treatment patterns of patients with CTEPH, and the longitudinal impact of therapies on quality of life, health care utilization, and survival. Conclusions: This manuscript details the methodology and design of the first large, prospective, longitudinal registry of patients with CTEPH in the United States.
AB - Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common, and diagnostic and treatment guidelines are often not followed. Data gathered from international registries have improved our understanding of CTEPH, but these data may not be applicable to the US population owing to differences in demographics and medical practice patterns. Objective: The US CTEPH Registry (US-CTEPH-R) was developed to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as the short- and long-term outcomes of surgical and nonsurgical therapies in the modern treatment era. Methods: Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of patients newly diagnosed with CTEPH. Enrollment criteria included a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multidisciplinary adjudication committee. Following enrollment, subjects were followed biannually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, and all other testing was at the discretion of the treating clinician. Details regarding surgical therapy, balloon pulmonary angioplasty, and medical therapy were collected at enrollment and at follow-up, as well as information related to health care utilization and survival. Results: Data from this registry will improve understanding of the demographics, risk factors, and treatment patterns of patients with CTEPH, and the longitudinal impact of therapies on quality of life, health care utilization, and survival. Conclusions: This manuscript details the methodology and design of the first large, prospective, longitudinal registry of patients with CTEPH in the United States.
KW - CTEPH
KW - Nonsurgical
KW - Pulmonary embolism
KW - Pulmonary hypertension
KW - Registry
KW - Surgical
KW - Therapy
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=85106896069&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85106896069&partnerID=8YFLogxK
U2 - 10.2196/25397
DO - 10.2196/25397
M3 - Review article
C2 - 33848258
AN - SCOPUS:85106896069
SN - 1929-0748
VL - 10
JO - JMIR Research Protocols
JF - JMIR Research Protocols
IS - 5
M1 - e25397
ER -