Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry

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 according to 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 ¼ 566), Operable patients who did not undergo surgery (n ¼ 88), and those who were Inoperable (n ¼ 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% inhospital 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.

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for chronic thromboembolic pulmonary hypertension patients after acute pulmonary embolism: Update

“…In an international registry, among patients undergoing PEA, the mortality associated with PH was 3.5% at 32-month follow-up [18]. In contrast, the annual all-cause mortality rate in the US registry was 5.6% [19], while in a single-center study by Polish authors, peri-operative mortality was 9.1% [20].…”

“…It should only be performed in experienced centers that specialize in this technique. In Poland, 30%-32% of patients with CTEPH are qualified for PEA [3,16,17] similar to Japan (23.5%) and in contrast to Western European countries (72.1%) and the US (87.2%) [18,19]. The incidence of persistent CTEPH after PEA is about 50% in international registers, and 46% in Poland [2,3].…”

What’s new in pulmonary hypertension clinical research: lessons from the best abstracts at the 2020 American Thoracic Society International Conference

“…Residual post-operative pulmonary hypertension (mPAP ≥ 38 mm Hg and PVR > 5.6 WU) has been established as a predictor of long-term mortality risk 143 . Given the aforementioned limitations, the study presented by Dr. Kerr at the ATS virtual minisymposium is a welcome addition to the body of literature in CTEPH 7,11 . It is not only the largest and most modern CTEPH registry, but also the first to analyze the outcomes of patients who were deemed operable but did not undergo PTE.…”

“…Table 1 provides a list of the abstracts and presenters featured during the session [1][2][3][4][5][6][7] . Notably, some of these studies have already made it to publication in peer-reviewed journals [8][9][10][11] .…”

Interventional Imaging Roadmap to Successful Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

“…3,4 Registry data suggest that up to 40% of all patients with CTEPH do not have surgically accessible disease. 5 To address this subgroup of patients, balloon pulmonary angioplasty (BPA) is a percutaneous procedure performed over multiple sessions to revascularize pulmonary artery branches. Increasing experience suggests that BPA is a promising therapeutic option in patients with CTEPH who are not candidates for PEA.…”