Chronic Thromboembolic Pulmonary Hypertension

Publication Date: December 1, 2018
Last Updated: March 14, 2022
In addition to chronic anticoagulation therapy, each patient with chronic thromboembolic disease (CTEPH) should receive treatment assessment starting with evaluation for pulmonary endarterectomy (PEA), which is the guideline recommended treatment.
6731
For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.
6731
CTEPH treatment guidelines should not be applied to chronic thromboembolic disease (CTED).
6731
PEA should be offered to all eligible patients with CTEPH.
6731
Balloon pulmonary angioplasty (BPA) should be reserved for expert centres, where it should be considered for symptomatic CTEPH patients ineligible for PEA due to distal chronic thromboembolism or persistent/recurrent PH after surgery.
6731
BPA complications should be defined and uniformly reported.
6731
An expert CTEPH centre should be able to assess and deliver all established treatment modalities with outcomes similar to or exceeding those published.
6731
Patients with operable CTEPH should receive PEA as the treatment of choice.
6731
Patients with persistent/recurrent symptomatic PH following PEA should receive medical therapy and be considered for BPA or re-do endarterectomy in cases of significant re-occlusion.
6731

Chronic thromboembolic pulmonary hypertension (CTEPH): revised treatment algorithm.

Recommendation Grading

Overview

Title

Chronic Thromboembolic Pulmonary Hypertension

Authoring Organization

Publication Month/Year

December 1, 2018

Last Updated Month/Year

June 12, 2023

Supplemental Implementation Tools

Document Type

Other

External Publication Status

Published

Country of Publication

European

Inclusion Criteria

Female, Male, Adult, Older adult

Health Care Settings

Ambulatory, Emergency care, Hospital, Operating and recovery room

Intended Users

Social worker, paramedic emt, athletics coaching, nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Diagnosis, Prevention, Management, Treatment

Diseases/Conditions (MeSH)

D006976 - Hypertension, Pulmonary, D013923 - Thromboembolism

Keywords

pulmonary hypertension, pulmonary embolism, Chronic thromboembolic, pulmonary endarterectomy, pulmonary angioplasty

Supplemental Methodology Resources

Data Supplement