Chest
Volume 160, Issue 5, November 2021, Pages 1822-1831
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Pulmonary Vascular: Original Research
Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up

Preliminary data have been presented in abstract form at the following: CHEST Annual Meeting, 2015 (October 24-28, 2015; Montreal, QC, Canada) and 2016 (October 22-26, 2016; Los Angeles, CA); the American Thoracic Society International Conference, 2016 (May 13-18, 2016; San Francisco, CA) and 2020 (virtual); and the 2017 International Society of Heart and Lung Transplant (April 5-8, 2017; San Diego, CA).
https://doi.org/10.1016/j.chest.2021.05.052Get rights and content
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Background

The United States Chronic Thromboembolic Pulmonary Hypertension Registry (US-CTEPH-R) was designed to characterize the demographic characteristics, evaluation, clinical course, and outcomes of surgical and nonsurgical therapies for patients with chronic thromboembolic pulmonary hypertension.

Research Question

What are the differences in baseline characteristics and 1-year outcomes between operated and nonoperated subjects?

Study Design and Methods

This study describes a multicenter, prospective, longitudinal, observational registry of patients newly diagnosed (< 6 months) with CTEPH. Inclusion criteria required a mean pulmonary artery pressure ≥ 25 mm Hg documented by right heart catheterization and radiologic confirmation of CTEPH. Between 2015 and 2018, a total of 750 patients were enrolled and followed up biannually until 2019.

Results

Most patients with CTEPH (87.9%) reported a history of acute pulmonary embolism. CTEPH diagnosis delays were frequent (median, 10 months), and most patients reported World Health Organization functional class 3 status at enrollment with a median mean pulmonary artery pressure of 44 mm Hg. The registry cohort was subdivided into Operable patients undergoing pulmonary thromboendarterectomy (PTE) surgery (n = 566), Operable patients who did not undergo surgery (n = 88), and those who were Inoperable (n = 96). Inoperable patients were older than Operated patients; less likely to be obese; have a DVT history, non-type O blood group, or thrombophilia; and more likely to have COPD or a history of cancer. PTE resulted in a median pulmonary vascular resistance decline from 6.9 to 2.6 Wood units (P < .001) with a 3.9% in-hospital mortality. At 1-year follow-up, Operated patients were less likely treated with oxygen, diuretics, or pulmonary hypertension-targeted therapy compared with Inoperable patients. A larger percentage of Operated patients were World Health Organization functional class 1 or 2 at 1 year (82.9%) compared with the Inoperable (48.2%) and Operable/No Surgery (56%) groups (P < .001).

Interpretation

Differences exist in the clinical characteristics between patients who exhibited operable CTEPH and those who were inoperable, with the most favorable 1-year outcomes in those who underwent PTE surgery.

Clinical Trial Registration

ClinicalTrials.gov; No.: NCT02429284; URL: www.clinicaltrials.gov.

Key Words

chronic thromboembolic pulmonary hypertension
CTEPH
pulmonary hypertension
registry
venous thromboembolism

Abbreviations

6MWD
6-min walk distance
BPA
balloon pulmonary angioplasty
CTEPH
chronic thromboembolic pulmonary hypertension
DSA
digital subtraction angiography
mPAP
mean pulmonary artery pressure
PAH
pulmonary arterial hypertension
PE
pulmonary embolism
PH
pulmonary hypertension
PTE
pulmonary thromboendarterectomy
UCSD
University of California San Diego
US-CTEPH-R
United States Chronic Thromboembolic Pulmonary Hypertension Registry
WHO
World Health Organization

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FUNDING/SUPPORT: This registry was funded by an Investigator Sponsored Study Research Grant from Bayer Healthcare Pharmaceuticals awarded to The Regents of the University of California, UC San Diego.