TY - JOUR
T1 - Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry
T2 - Enrollment Characteristics and 1-Year Follow-up
AU - Kerr, Kim M.
AU - Elliott, C. Greg
AU - Chin, Kelly
AU - Benza, Raymond L.
AU - Channick, Richard N.
AU - Davis, R. Duane
AU - He, Feng
AU - LaCroix, Andrea
AU - Madani, Michael M.
AU - McLaughlin, Vallerie V.
AU - Park, Myung
AU - Robbins, Ivan M.
AU - Tapson, Victor F.
AU - Terry, Jeffrey R.
AU - Test, Victor J.
AU - Jain, Sonia
AU - Auger, William R.
N1 - Publisher Copyright:
© 2021 The Authors
PY - 2021/11
Y1 - 2021/11
N2 - 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.
AB - 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.
KW - CTEPH
KW - chronic thromboembolic pulmonary hypertension
KW - pulmonary hypertension
KW - registry
KW - venous thromboembolism
UR - http://www.scopus.com/inward/record.url?scp=85116045079&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85116045079&partnerID=8YFLogxK
U2 - 10.1016/j.chest.2021.05.052
DO - 10.1016/j.chest.2021.05.052
M3 - Article
C2 - 34090871
AN - SCOPUS:85116045079
SN - 0012-3692
VL - 160
SP - 1822
EP - 1831
JO - CHEST
JF - CHEST
IS - 5
ER -