United States Chronic Thromboembolic Pulmonary Hypertension Registry: Protocol Design.

BACKGROUND: 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 has improved our understanding of CTEPH, but this data may not be applicable to the U.S. population due to differences in demographics and medical practice patterns. OBJECTIVE: Objective: The U.S. 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 short and long-term outcomes of surgical and non-surgical therapies in the modern treatment era. METHODS: Methods: Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of newly diagnosed CTEPH patients. Enrollment criteria included a mean pulmonary artery pressure > 25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multi-disciplinary adjudication committee. Following enrollment, subjects were followed bi-annually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, 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: Results: Data from this registry will improve the understanding of the demographics, risk factors, and treatment patterns of CTEPH patients and the longitudinal impact of therapies on quality of life, healthcare utilization, and survival. CONCLUSIONS: Conclusions: This manuscript details the methodology and design of the first large, prospective, longitudinal, registry of CTEPH patients in the U.S. CLINICALTRIAL: ClinicalTrials.gov ID: NCT02429284.