Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death...

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Main Authors: Naoyuki Otani, Ryo Watanabe, Takashi Tomoe, Shigeru Toyoda, Takanori Yasu, Takaaki Nakamoto
Format: Article
Language:English
Published: MDPI AG 2023-02-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/24/4/3979
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author Naoyuki Otani
Ryo Watanabe
Takashi Tomoe
Shigeru Toyoda
Takanori Yasu
Takaaki Nakamoto
author_facet Naoyuki Otani
Ryo Watanabe
Takashi Tomoe
Shigeru Toyoda
Takanori Yasu
Takaaki Nakamoto
author_sort Naoyuki Otani
collection DOAJ
description Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.
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spelling doaj.art-9a2a4f703ebf430a90e02ab3ca75da182023-11-16T21:07:39ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672023-02-01244397910.3390/ijms24043979Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary HypertensionNaoyuki Otani0Ryo Watanabe1Takashi Tomoe2Shigeru Toyoda3Takanori Yasu4Takaaki Nakamoto5Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, JapanDepartment of Cardiovascular Medicine, Dokkyo Medical University, Mibu 321-0293, JapanDepartment of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, JapanDepartment of Cardiovascular Medicine, Dokkyo Medical University, Mibu 321-0293, JapanDepartment of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, JapanDepartment of Cardiology, Dokkyo Medical University Nikkyo Medical Center, Nikko 321-1298, JapanChronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.https://www.mdpi.com/1422-0067/24/4/3979balloon pulmonary angioplastypulmonary endarterectomychronic thromboembolic pulmonary hypertension
spellingShingle Naoyuki Otani
Ryo Watanabe
Takashi Tomoe
Shigeru Toyoda
Takanori Yasu
Takaaki Nakamoto
Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
International Journal of Molecular Sciences
balloon pulmonary angioplasty
pulmonary endarterectomy
chronic thromboembolic pulmonary hypertension
title Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
title_full Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
title_fullStr Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
title_full_unstemmed Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
title_short Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension
title_sort pathophysiology and treatment of chronic thromboembolic pulmonary hypertension
topic balloon pulmonary angioplasty
pulmonary endarterectomy
chronic thromboembolic pulmonary hypertension
url https://www.mdpi.com/1422-0067/24/4/3979
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AT shigerutoyoda pathophysiologyandtreatmentofchronicthromboembolicpulmonaryhypertension
AT takanoriyasu pathophysiologyandtreatmentofchronicthromboembolicpulmonaryhypertension
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