Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up
Kim M Kerr, C Greg Elliott, Kelly Chin, Raymond L Benza, Richard N Channick, R Duane Davis, Feng He, Andrea LaCroix, Michael M Madani, Vallerie V McLaughlin, Myung Park, Ivan M Robbins, Victor F Tapson, Jeffrey R Terry, Victor J Test, Sonia Jain, William R Auger, Kim M Kerr, C Greg Elliott, Kelly Chin, Raymond L Benza, Richard N Channick, R Duane Davis, Feng He, Andrea LaCroix, Michael M Madani, Vallerie V McLaughlin, Myung Park, Ivan M Robbins, Victor F Tapson, Jeffrey R Terry, Victor J Test, Sonia Jain, William R Auger
Abstract
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.
Keywords: CTEPH; chronic thromboembolic pulmonary hypertension; pulmonary hypertension; registry; venous thromboembolism.
Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.
Figures
References
- Kim N.H., Delcroix M., Jais X., et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(1):1801915.
- Rich S., Dantzker D.R., Ayres S.M., et al. Primary pulmonary hypertension: a national prospective registry. Ann Int Med. 1987;107(2):216–223.
- Badesch D.B., Raskob G.E., Elliott C.G., et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest. 2010;137(6):376–387.
- Humbert M., Sitbon O., Chaouat A., et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023–1030.
- Ling Y., Johnson M.K., Kiely D.G., et al. Changing demographics, epidemiology and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–796.
- McGoon M.D., Benza R.L., Escribano-Subias P., et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013;62(suppl 25):D51–D59.
- Pepke-Zaba J., Delcroix M., Lang I., et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973–1981.
- Delcroix M., Lang M., Pepke-Zaba, et al. Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation. 2016;133(9):859–871.
- Guerin L., Couturaud F., Parent F., et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Thrombosis Haemost. 2014;112(3):598–605.
- Klock F.A., Barco S., Konstantinides S.V., et al. Determinants of diagnostic delay in chronic thromboembolic pulmonary hypertension: results from the European CTEPH Registry. Eur Respir J. 2018;52(6):1801687.
- Bonderman D., Wilkens H., Wakounig S., et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):325–331.
- Lang I.M., Simonneau G., Pepke-Zaba J.W., et al. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension; a case-control study. Thromb Haemost. 2013;110(1):83–91.
Source: PubMed