Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism

F A Klok, M Delcroix, H J Bogaard

Research output: Contribution to journalReview articleAcademicpeer-review

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long delay. Once the diagnosis is confirmed, the treatment of choice is pulmonary endarterectomy which effectively lowers the pulmonary vascular resistance and normalizes resting pulmonary artery pressures, leading to recovery of the right ventricle. When pulmonary endarterectomy is not technically feasible, balloon pulmonary angioplasty may be a potential acceptable alternative. Also, medical treatment may help to improve patient's symptoms and hemodynamics. Current studies are focusing on strategies for earlier CTEPH diagnosis after acute PE, as well as the most optimal treatment of inoperable patients. This review will focus on the epidemiology, risk factors, diagnosis and treatment of CTEPH from the perspective of the PE patient.

Original languageEnglish
Pages (from-to)1040-1051
Number of pages12
JournalJournal of Thrombosis and Haemostasis
Volume16
Issue number6
DOIs
Publication statusPublished - Jun 2018

Cite this

@article{dc1a4b2d6ce44663869bef86b9b51bdc,
title = "Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism",
abstract = "Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50{\%} of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long delay. Once the diagnosis is confirmed, the treatment of choice is pulmonary endarterectomy which effectively lowers the pulmonary vascular resistance and normalizes resting pulmonary artery pressures, leading to recovery of the right ventricle. When pulmonary endarterectomy is not technically feasible, balloon pulmonary angioplasty may be a potential acceptable alternative. Also, medical treatment may help to improve patient's symptoms and hemodynamics. Current studies are focusing on strategies for earlier CTEPH diagnosis after acute PE, as well as the most optimal treatment of inoperable patients. This review will focus on the epidemiology, risk factors, diagnosis and treatment of CTEPH from the perspective of the PE patient.",
author = "Klok, {F A} and M Delcroix and Bogaard, {H J}",
note = "{\circledC} 2018 International Society on Thrombosis and Haemostasis.",
year = "2018",
month = "6",
doi = "10.1111/jth.14016",
language = "English",
volume = "16",
pages = "1040--1051",
journal = "Journal of Thrombosis and Haemostasis",
issn = "1538-7933",
publisher = "Wiley-Blackwell",
number = "6",

}

Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism. / Klok, F A; Delcroix, M; Bogaard, H J.

In: Journal of Thrombosis and Haemostasis, Vol. 16, No. 6, 06.2018, p. 1040-1051.

Research output: Contribution to journalReview articleAcademicpeer-review

TY - JOUR

T1 - Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism

AU - Klok, F A

AU - Delcroix, M

AU - Bogaard, H J

N1 - © 2018 International Society on Thrombosis and Haemostasis.

PY - 2018/6

Y1 - 2018/6

N2 - Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long delay. Once the diagnosis is confirmed, the treatment of choice is pulmonary endarterectomy which effectively lowers the pulmonary vascular resistance and normalizes resting pulmonary artery pressures, leading to recovery of the right ventricle. When pulmonary endarterectomy is not technically feasible, balloon pulmonary angioplasty may be a potential acceptable alternative. Also, medical treatment may help to improve patient's symptoms and hemodynamics. Current studies are focusing on strategies for earlier CTEPH diagnosis after acute PE, as well as the most optimal treatment of inoperable patients. This review will focus on the epidemiology, risk factors, diagnosis and treatment of CTEPH from the perspective of the PE patient.

AB - Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long delay. Once the diagnosis is confirmed, the treatment of choice is pulmonary endarterectomy which effectively lowers the pulmonary vascular resistance and normalizes resting pulmonary artery pressures, leading to recovery of the right ventricle. When pulmonary endarterectomy is not technically feasible, balloon pulmonary angioplasty may be a potential acceptable alternative. Also, medical treatment may help to improve patient's symptoms and hemodynamics. Current studies are focusing on strategies for earlier CTEPH diagnosis after acute PE, as well as the most optimal treatment of inoperable patients. This review will focus on the epidemiology, risk factors, diagnosis and treatment of CTEPH from the perspective of the PE patient.

U2 - 10.1111/jth.14016

DO - 10.1111/jth.14016

M3 - Review article

VL - 16

SP - 1040

EP - 1051

JO - Journal of Thrombosis and Haemostasis

JF - Journal of Thrombosis and Haemostasis

SN - 1538-7933

IS - 6

ER -