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

Graphical abstract
Graphical abstract
Figure 1
Figure 1
Disposition of subjects. Flow diagram for study participants. Subjects were assessed and deemed Operable or Inoperable. Operable subjects were further divided into those who underwent pulmonary thromboendarterectomy (Operated) and those who did not (Operable/No Surgery). mPAP = mean pulmonary artery pressure; PVR = pulmonary vascular resistance.
Figure 2
Figure 2
WHO functional class at enrollment and 1-year follow-up. WHO functional class is similar between the three cohorts at enrollment, but at 1-year follow-up Operated patients have a statistically better WHO functional class compared with both Inoperable and Operable/No Surgery patients (P < .001). WHO = World Health Organization.
Figure 3
Figure 3
Medical therapy at enrollment and 1-year follow-up. At enrollment, there is no difference in the percentage of patients in each cohort using oxygen or diuretics. More Inoperable patients are on PH-targeted therapy than Operated patients at enrollment (P < .001). At 1-year follow-up, significantly more Inoperable patients are on diuretics and/or oxygen than Operated patients (P <.001) and more Inoperable and Operable/No Surgery patients are being treated with PH-targeted therapy compared with operated patients (P < .001). PH = pulmonary hypertension.

References

    1. Kim N.H., Delcroix M., Jais X., et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(1):1801915.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. 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.
    1. Bonderman D., Wilkens H., Wakounig S., et al. Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J. 2009;33(2):325–331.
    1. 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

スポンサーと協力者

医学的状態

薬物療法

3
購読する