Abstract
BackgroundChronic 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.ObjectiveThe 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.MethodsThirty 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.ResultsData 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.ConclusionsThis manuscript details the methodology and design of the first large, prospective, longitudinal registry of patients with CTEPH in the United States.Trial RegistrationClinicalTrials.gov NCT02429284; https://www.clinicaltrials.gov/ct2/show/NCT02429284International Registered Report Identifier (IRRID)DERR1-10.2196/25397
Highlights
Chronic thromboembolic pulmonary hypertension (CTEPH) is a subset of pulmonary hypertension (PH) characterized by obstruction of the pulmonary arteries with fibrotic material and vascular remodeling, which leads to increased pulmonary arterial pressure and right ventricular failure
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
Clinical presentation and the pathological changes of the pulmonary vasculature share some characteristics with pulmonary arterial hypertension (PAH), the etiology, diagnosis, and treatment of CTEPH are quite distinct from those of PAH [1]
Summary
Chronic thromboembolic pulmonary hypertension (CTEPH) is a subset of pulmonary hypertension (PH) characterized by obstruction of the pulmonary arteries with fibrotic material and vascular remodeling, which leads to increased pulmonary arterial pressure and right ventricular failure. The diagnostic and therapeutic landscape for CTEPH in the United States has changed dramatically in recent years. Digital subtraction pulmonary angiography (DSA) was long considered the gold standard for the diagnosis of CTEPH and determination of operability; technological improvements in computerized axial tomography angiography (CTA) have replaced invasive pulmonary angiography in many centers, and magnetic resonance imaging is used at some centers for both angiography (MRA) and assessment of right ventricular function. 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
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