The United States Chronic Thromboembolic Pulmonary Hypertension Registry: Protocol for a prospective, longitudinal study

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

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations


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.

Original languageEnglish (US)
Article numbere25397
JournalJMIR Research Protocols
Issue number5
StatePublished - May 2021


  • Nonsurgical
  • Pulmonary embolism
  • Pulmonary hypertension
  • Registry
  • Surgical
  • Therapy
  • Treatment

ASJC Scopus subject areas

  • Medicine(all)


Dive into the research topics of 'The United States Chronic Thromboembolic Pulmonary Hypertension Registry: Protocol for a prospective, longitudinal study'. Together they form a unique fingerprint.

Cite this